Diseases of the Male Genitalia: Nonmalignant and malignant
Diseases of the Male Genitalia: Non malignant
Authored by Milton W Datta, MD, Assistant Professor, Department
of Pathology, Medical College of Wisconsin
Coauthored by Hon Pak, MD, Chief of Dermatologic Surgery and Outpatient
Clinic, Assistant Professor, Department of Dermatology, Wilford
Hall Air Force Medical Center, Uniformed Services University; Scott
M Acker, MD, Assistant Professor, Departments of Pathology and Dermatology,
Medical College of Wisconsin; Peter Langenstroer, MD, Assistant
Professor, Department of Surgery, Division of Urology, Medical College
Milton W Datta, MD, is a member of the following medical societies:
College of American Pathologists
Edited by Martha K Terris, MD, FACS, Chief, Section of Urology,
Veterans Palo Alto Health Care System; Assistant Professor, Department
of Urology, Stanford University Medical Center; Francisco Talavera,
PharmD, PhD, Senior Pharmacy Editor, eMedicine; Laurence Klotz,
MD, Professor, Department of Surgery, University of Toronto School
of Medicine, Canada; J Stuart Wolf, Jr, MD, Director of Michigan
Center for Minimally Invasive Urology, Associate Professor, Department
of Urology, University of Michigan Medical Center; and Stephen W
Leslie, MD, FACS, Founder and Medical Director of the Lorain Kidney
Stone Research Center, Assistant Clinical Professor, Department
of Urology, Medical College of Ohio
Definition of terms and anatomy
Nonmalignant lesions account for the majority of dermatologic disorders
seen by urologists. They usually are not biopsied, but rather, they
are treated empirically and are not commonly referred to dermatologists
or pathologists. Throughout this article, the discussion focuses
on diseases that are either specific to the male genitalia or make
their initial appearance in this location. Disorders suggestive
of systemic conditions or diseases are not discussed in this article.
While rare in the United States and minimally emphasized in medical
schools, a resurgence in some infectious diseases is occurring due
to increased world emigration and the spread of resistant forms.
Topics in this article are divided into groups based on their initial
clinical presentation and most common appearance. The underlying
discussion focuses on diagnosis, treatment, and clinical outcome.
Attempts are made to include the most recent and relevant treatment
options, emphasizing the use of pharmaceuticals and their impact.
US Centers for Disease Control and Prevention (CDC) recommendations
are stressed, and promising experimental treatments are noted. While
presenting a comprehensive review of each disorder is not the goal
of this article, a thorough and understandable knowledge base for
the treatment of patients is provided, along with references that
lead to more detailed and comprehensive studies of the field.
In the field of dermatology, use of specific terms to describe clinical
findings allows communication of essential information and may suggest
a differential diagnosis. To the uninitiated, these terms can lead
to confusion, frustration, and a review of other career options.
Therefore, this article begins with definitions of terms used to
describe the most common dermatologic appearances.
Macules: These are small defined areas of color change that are
not palpable (ie, flat or sometimes collapsed, but not bumpy). Macules
tend to be smaller than 1 cm.
Patch: This is a larger (>1 cm) area of color change that is
nonpalpable (ie, level with the skin). In essence, a patch is a
Papules: These are small lesions that are palpable (ie, feels like
a bump). They tend to be smaller than 1 cm and can be any color
Plaque: This is a large (>1 cm) raised area with palpable borders.
Basically, it is a large papule with extensions along the edges.
Note that the height is not increased. A plaque is large, raised,
and flat. Edges can be palpated, and borders are well delineated.
Like papules, they can be any color or texture. Often, this represents
a confluence of papules.
Nodule: This is a large (>1.5 cm) papule. Unlike plaques, an
increase in the lesion's height is noted.
Vesicles: These are fluid-filled bumps, usually smaller than 1 cm
in diameter. The common term is blister. A distinct characteristic
is that when it is incised and the fluid is removed, the vesicle
collapses. It can be considered a fluid-filled papule.
Pustules: These are vesicles filled with neutrophils.
Dermatologic anatomy of the male genitalia
The male external genitalia are comprised of the penis and scrotum.
The penis is divided into the more distal glans and the shaft or
body, which is anchored by its root in the perineal pouch. The prepuce
(foreskin) covers the glans. It is a thin loose covering of keratinizing
skin with associated underlying eccrine (sweat) and sebaceous glands
and a highly vascular stroma without underlying adipose tissue.
The prepuce is composed of the following 5 layers: (1) external
keratinizing epidermis, (2) underlying dermis with eccrine and sebaceous
glands, (3) fine strands of dartos muscle, (4) lamina propria, and
(5) internal squamous mucosa.
The glans is an extension of the highly vascular corpus spongiosum
and is covered by squamous epithelium that is keratinized in the
circumcised male. The scrotum extends the keratinized squamous epithelium
with underlying dartos muscle and associated external spermatic
fascia. Here the dermis contains hair follicles in association with
eccrine, apocrine, and sebaceous glands. Scattered fat cells are
present, although well-formed subcutaneous adipose tissue usually
The majority of the dermatologic disorders are confined to the epidermis,
underlying dermis, and associated adnexal structures. These areas
include the keratinized epithelium, the underlying dermis with its
rich vasculature, associated smooth muscle, and associated hair
follicles and sweat and sebaceous glands.
Trichomycosis (see Trichomycosis Pubis) is an infection of the hair
shaft that is found in areas bearing sweat glands, in particular
the inguinal area. Treatment centers on the use of topical antibiotics
and drying agents.
Corynebacterium tenuis, which prefers the moist microenvironment
of the inguinal regions, is the causative organism associated with
most cases. Although up to 33% of adults have colonization by this
bacterium in the inguinal or axillary regions, factors such as hyperhidrosis
initiate more extensive growth and clinical manifestations.
Patients typically present with yellow/black or red nodules on the
hair shafts in the inguinal region. Nodules are often on the scrotum
and occasionally on the base of the shaft of the penis. These lesions
can be associated with erythema and itching. Superinfection with
other dermatophytes has been noted.
Clinical separation from other organisms often is not performed,
and treatment focuses on empirical use of drying agents and topical
Treatment involves alleviating the hyperhidrosis, focusing on the
use of drying agents. Topical clindamycin is helpful in refractory
or extensive cases.
The main complication appears in immunocompromised hosts who can
develop more extensive septicemia secondary to colonization of catheters
and surgical sites. Treatment in the immune-competent host yields
few long-term adverse effects.
Folliculitis (see Folliculitis) is an infection of the hair shaft
that occurs in the pubic area and clears with appropriate antibiotic
treatment, as in other regions of the body.
This lesion often occurs from external trauma (shaving, rubbing),
irritation from hyperhidrosis and moisture, or topical irritants.
This environment leads to bacterial colonization and overgrowth,
leading to subsequent clinical presentation. Organisms identified
include Staphylococcus species but also can involve Pseudomonas
and Candida species.
Patients often present with small red papules or pustules centered
on hair follicles. These often are present at the base of the penile
shaft or on the scrotum. Often, additional symptomatology is not
The differential diagnosis is broad and includes infections with
other organisms that could result in folliculitis under various
circumstances. Because treatment often is empirical and cultures
or biopsy are not performed routinely, clinical examination to exclude
viral (Tzanck prep), fungal (potassium hydroxide [KOH] prep), or
neoplastic (biopsy) causes are the most important. Folliculitis
also can be part of the spectrum of disease presentation in Behçet
disease (see Bechet Disease) and, thus, also should be considered
in a patient with other systemic symptoms including oral and genital
aphthae, uveitis, joint disease, and gastrointestinal disease.
Treatment is directed at the offending organism. It includes improved
hygiene, drying agents, and topical and oral antimicrobials or antivirals
as necessary. Conservative therapy is reserved for sterile folliculitis.
Outcome usually is satisfactory. Failures result from misclassification
of the causative organism or the presence of a mixed culture of
Defined as the inflammation of the foreskin and the glans in uncircumcised
males, balanoposthitis (see Balanoposthitis) may have any of multiple
bacterial and fungal origins. Complex infections also have been
well documented. These often are due to a poorly retractile foreskin
and poor hygiene that leads to colonization and overgrowth. Treatment
centers on improved hygiene and circumcision, if needed.
Often identified in association with poorly retractile foreskins
in young boys (<5 y), balanoposthitis is associated with bacterial
infection secondary to poor hygiene. Anaerobic organisms have been
associated with the diagnosis, although in most cases, the patient
is treated empirically and a specific organismal etiology is not
obtained. Candidal infection appears to be the most common cause
of disease. The condition in older adult men often has other etiologies,
including intertrigo; irritant dermatitides; and candidal, viral,
or fungal infections.
In adults, a good clinical history focusing on topical irritants
and home treatments, coupled with KOH and Tzanck preps, leads to
the correct diagnosis. Biopsy seldom is needed, although a lack
of response to treatment warrants a biopsy to rule out premalignant
or malignant lesions.
The differential diagnosis includes intertrigo (see Intertrigo);
irritant dermatitides; and candidal (see Candidiasis, Cutaneous),
viral, or fungal infections. As emphasized above, the use of clinical
diagnosis and KOH and scrape preps often leads to the correct diagnosis.
In addition, ulcerating lesions of the penis may result from bites
with subsequent infection by oral flora. These lesions can be diagnosed
based on clinical history and culture of the ulcer. Rare causes
include amebiasis (see Amebiasis), usually in homosexual men, which
should be suspected in patients with poor response to antibiotics.
Examination for trophozoites is confirmatory.
Topical antibiotics and antifungals or low-potency steroid creams
often lead to clearing of the lesion. Treatment includes proper
hygiene with washing and drying of the prepuce. Circumcision usually
is effective if medical therapy fails.
The outcome often is favorable. Treatment failures often lead to
further clinical examination and further treatments tailored to
the particular offending agent. Failure of response in the setting
of appropriate treatment suggests malignancy. This necessitates
a biopsy to rule out both primary and secondary malignancies involving
the penis. One case report describes the presentation of acute promyelocytic
leukemia as an ulcerating balanoposthitis.
Plasma cell balanitis
First described in 1952 by Zoon, plasma cell balanitis (Zoon balanitis,
Balanitis Circumscripta Plasmacellularis) is characterized by the
histologic appearance of an infiltrate rich in plasma cells. Although
asymptomatic, these lesions may represent a variant of lichen planus.
Histologically, these lesions do not share features with lichen
planus. Treatment often involves circumcision, although topical
steroids and retinoids have been used with some success, based on
the possible origin in lichen planus.
Although the etiology is unclear, the possibility that this lesion
is a plasma cell-rich variant of lichen planus has gained some attention,
mainly due to the potential for response to anti-inflammatory treatments.
In one study, immunoglobulin E (IgE) and immunoglobulin G (IgG)
were determined to be major immunoglobulin classes in plasma cellular
infiltrate; thus, the disorder may have more in common with a hypersensitivity
reaction than an infectious process. Other classes of immunoglobulins
were produced in lesser quantities. Immunoperoxidase studies revealed
low immunoglobulin M (IgM) levels and a mixed kappa-to-lambda ratio,
which favored a polyclonal B-cell stimulation. Another premise explains
the disorder as a disturbed preputial ecology that results in the
nonspecific balanitis. Progression to a malignant B-cell proliferation
has not been reported, although a report of carcinoma of the penis
postdating Zoon balanitis has been published. No association with
human papillomavirus (HPV) and Zoon balanitis has been identified.
Although the mean age at presentation is 53 years, patients aged
18-88 years are described in 2 series and presentation in children
as young as 12 years has been described. Usually, plasma cell balanitis
occurs in men, although a similar lesion has been described as plasma
cell vulvitis in women. Typically, men are uncircumcised in most
series. In one analysis of diseases in uncircumcised men, Zoon balanitis
was present in uncircumcised males only.
Occasionally, discharge may be the presenting complaint. The clinical
course typically is chronic, and delays in presentation average
12 months due to embarrassment or lack of access to health care.
Delays in seeking treatment have been as long as 17 years. Usually,
patients present with a single, large (>2 cm), bright red-to-brown,
sharply circumscribed, moist patch on the glans or inner prepuce.
Rarely, multiple patches occur that can erode and ulcerate. Clinical
involvement typically (85%) is on both the glans and prepuce or
prepuce only. Presentation on the glans alone is less common.
Histologic evaluation reveals a bandlike plasma cell infiltrate
of the upper dermis, with dilated capillaries and associated extravasated
red cells and hemosiderin deposition. The overlying epidermis is
thin, with marked spongiosus and without cytological atypia. The
spongiosus can be so marked that it accentuates the individual keratinocytes
to give them a characteristic diamond-shaped appearance. The cellular
population is composed of plasma cells, with associated macrophages,
mast cells, and eosinophils. These findings are confirmed by electron
Often, the clinical resemblance to erythroplasia of Queyrat leads
to biopsy of the lesions. See Erythroplasia of Queyrat (Bowen Disease
of the Glans Penis). Subsequent histologic features allow separation
from these entities. Occasionally, balanitis xerotica obliterans
(see Lichen Sclerosus et Atrophicus) also may show dense plasmacytic
infiltrates. Candidal balanitis also should be considered in the
differential diagnosis. Although previously described as a separate
entity, chronic pseudoerythroplastic balanitis now appears to be
a different stage in the course of plasma cell balanitis.
Previously, treatment focused on surgical excision or laser ablation,
although the use of Retin-A has recently met with some success.
The use of topical and intralesional corticosteroids has met with
mixed results, with some groups advocating use and other groups
finding them (particularly topical forms) ineffective. Experimental
use of cyclosporin A has been attempted, with preliminary results
that are hopeful.
Although outcome with the use of newer treatments has yielded promising
results, surgical removal of the foreskin has been advocated as
standard case management. Circumcision was successful in 27 patients,
with no recurrence after 3 years of follow-up. Most authors report
a nearly 100% curative effect with adequate circumcision.
Candidal balanitis (see Candidiasis, Cutaneous) is a relatively
common disorder, most often present in uncircumcised men with poor
hygiene. It has been associated with vulvovaginitis in the sexual
partner, and treatment of both partners with antifungals usually
yields an adequate result.
While common in uncircumcised men, the main association appears
to be with poor hygiene and the entrapment of smegma under the prepuce.
Additional factors that contribute to infection include surface
maceration, diabetes mellitus, and immunosuppression. Candidal balanitis
often is associated with vaginal infection in the sexual partner,
thus leading to a co-infection. While many species have been isolated,
Candida albicans is by far the most common.
Clinical presentation is bright red patches/papules with associated
small pustules/erosions (satellite lesions) that most commonly involve
the foreskin and glans. Patients also often describe itching and
burning sensations after intercourse. Diagnosis using a KOH scrape
reveals yeastlike fungus with budding and pseudohyphal forms. Biopsies
often reveal a prominent neutrophilic infiltrate in the epidermis
and associated chronic inflammation in the dermis. The diagnostic
pseudohyphae usually are present colonizing the stratum corneum.
The differential diagnosis includes dermatophytes and can be ruled
out based on the clinical presentation of satellite lesions (uncommon
in tinea) and the morphologic appearance on the KOH scrape.
Treatment focuses on the cause of the papules and pustules, with
antifungals used for presumed candidal infections. Topical azole
creams or nystatin have been used successfully in the treatment
of candidal balanitis. A positive culture alone is not sufficient
for diagnosis, and other organisms also must be ruled out because
Candida species may colonize other forms of balanitis.
Treatment often is effective if the partner also is treated, removing
possible reservoirs for future reinfection.
Dermatophyte infections include fungal organisms that colonize the
keratinized epidermal layer and establish an associated inflammatory
host reaction. When present in the groin/male genitalia, they are
referred to as tinea cruris or "jock itch" (see Tinea
Cruris). Clinical presentation is characterized by the annular scaly
patch common to presentations in most other locations on the body.
These lesions can be diagnosed by KOH scrape preps and treated with
These infections often are present in communal showers at gym facilities
and spread to the genital areas from the feet via towel drying.
The fungi colonize the superficial layers of the epidermis and include
the species Trichophyton rubrum, Trichophyton mentagrophytes, and
Patients usually present with red-brown circinate lesions with raised
borders. These often involve the thighs, the groin, and, less commonly,
the scrotum. Secondary excoriation and lichenification are common,
and continued excoriation can lead to secondary bacterial infection.
Involvement of the scrotum is associated more often with candidal
infection (see Candidiasis, Cutaneous), and is suggested in this
setting. The finding of satellite lesions is more in keeping with
a candidal infection than dermatophyte.
Topical antifungal creams are effective, along with drying agents.
Also, towel drying should be performed from top to bottom, ending
with the feet.
Treatment with antifungals usually is sufficient, although recurrence
is possible. Recurrent disease may cause postinflammatory hyperpigmentation.
The first causative agent of a human disease was identified in 1687.
It was scabies (see Scabies). The mite Sarcoptes scabiei causes
these highly contagious pruritic lesions. While this contagion can
be sexually transmitted, poor hygiene and living conditions also
can lead to infection. Treatment is effective, but it must involve
the entire living group and include removal of contaminated bedding
While highly contagious, prolonged contact is necessary to cause
transmission. This most often is achieved through contact with contaminated
clothes or bedding. Scabies mites create burrows in the epidermis
up to 1 cm in length and lay eggs within these nests. These eggs
often elicit an IgE-mediated cellular immune response and the associated
pruritus. In fact, patients often have elevated serum IgE levels.
Larvae emerge from their burrows in 4 days and mature in 10-14 days.
Patients often present with severely pruritic scaly red patches
that may be papular or nodular (more common on the male genitalia).
Associated excoriation of the lesions is common due to severe itching
that is worse at night or when the patient is warm. Diagnosis is
made by teasing out samples of the mite with a needle or by scraping
and identifying mite body parts or eggs from skin prep treated with
KOH. While not often performed, biopsies reveal the female mite
in the keratinizing horny layer, with eggs in the burrow staining
positive with periodic acid-Schiff (PAS) stain. Epidermal spongiosus,
associated dermal eosinophilia, and a prominent superficial and
deep dermal inflammatory reaction also are present.
Nodular and treated forms (scabies incognito) may make the diagnosis
more difficult. The use of topical corticosteroids also may mask
a more typical presentation. Secondary bacterial or viral herpes
simplex virus (see Herpes Simplex) superinfection may result in
presentation as vesicles and lead to misdiagnosis.
Lindane 1% lotion or cream is recommended, with application and
subsequent washing 8 hours later. This is not recommended for pregnant
women or children younger than 2 years. In these patients, permethrin
5% cream is used. Antipruritic agents also are valuable to reduce
the amount of excoriation and discomfort. Treatment also must cover
additional family members and partners to remove the reservoir and
eliminate reinfection. Often, itching continues for as long as a
week after treatment, most likely due to the immune reaction to
residual scabies material. If no response is observed, perform a
second search for mites before a second round of treatment is given.
Clothes and bedding should be washed in hot water and dried in a
hot dryer before subsequent use.
The first treatment usually is 90% effective. A particular crusted
variant (hyperkeratotic or Norwegian scabies) exists, characterized
by a more keratotic psoriasiform lesion that is seen in patients
with HIV or other immunocompromised states. This lesion is highly
contagious, even to individuals who are not immunocompromised, due
to the high total mite burden and may lead to localized epidemic
outbreaks. Bacterial superinfection, usually by staphylococcal or
streptococcal species, may lead to localized and systemic findings
and even acute glomerulonephritis.
AGENTS AFFECTING THE MALE GENITALIA
While uncommon in the United States as a cause of genital ulcers,
lymphogranuloma venereum (see Lymphogranuloma Venereum) accounts
for up to 10% of genital ulcers in areas of India and Africa. Only
113 US cases were reported to the CDC in 1997. After a patient with
an often-unidentified primary papule, inguinal lymphadenitis, and
bubo formation presents for clinical care, treatment involves the
use of antibiotics to clear the infection and prevent tertiary sequelae.
Chlamydia trachomatis, an obligate intracellular organism that infects
macrophages, is the causative agent. Serotypes L1-3 have been associated
with infection. Characterization of the rate of transmission or
the reservoir of C trachomatis has not been defined clearly, although
asymptomatic women are believed to be carriers of infection. Incubation
periods of 3-30 days are reported. The patient often does not notice
the initial presenting small papule or ulcer. The second stage has
its onset 2-6 weeks later, with lymphadenitis of the inguinal nodes
and associated lymphangitis.
The condition occurs in men aged 15-40 years, with presentation
as an acute ulcerating disease more common in men than in women.
The initial presentation (primary stage) occurs 1-4 weeks after
exposure as a transient firm painless papule or ulcer that may not
be noticed by either the patient or physician. Male patients tend
to present during the second stage, with painful inguinal adenopathy
that usually is unilateral. Subsequent rupture and bubo formation
may be accompanied by fever, chills, headache, nausea, and arthralgias.
Diagnosis is hampered by the difficulty in culturing the organism.
The best results are obtained using aspirates from an involved inguinal
lymph node and cell-typing the culture after growth. Culture requires
growth in cycloheximide treated McCoy or HeLa cells. Even under
these conditions, yields of only 50% are reported. Serologic tests
also are available and produce a strong reaction by compliment fixation
tests in the second stage of disease. These tests have difficulty
in separating the various serotypes for Chlamydia species, including
those involved in conjunctivitis. In the appropriate clinical setting,
an antibody titer of 1:64 or greater, or a 4-fold increase in titer,
supports the diagnosis. Antibody titers do not correlate well with
the clinical severity of the disease.
The differential diagnosis includes granuloma inguinale and syphilis
(see Granuloma Inguinale (Donovanosis) and Syphilis), both of which
can be separated based on the clinical and smear findings.
The recommended treatment is doxycycline at 100 mg PO bid or erythromycin
at 500 mg qid. Treatments should be continued for 3 weeks and may
need to be combined with aspiration of the lymph nodes. Incision
and drainage may lead to nonhealing fistula formation.
Untreated cases can lead to the formation of sinus tracts and third-stage
disease, also referred to as genital anorectal syndrome. Rectal
involvement is more prevalent in homosexual males and is associated
with proctocolitis, perirectal abscesses, and lymphatic hyperplasia
and lymphatic hemorrhoids. Scarring and local tissue destruction
is the rule, with stricture, scarring, and lymphedema.
Although recently decreasing in incidence, with 3.2 cases per 100,000
population in 1998, specific reservoirs of infection still are present,
in particular in populations with HIV and in urban users of crack
cocaine. Infection is initiated by sexual contact that transmits
Treponema pallidum and establishes a primary infection (see Syphilis).
Antibiotic treatment is effective, but if left untreated, secondary
syphilis may result.
The causative organism is the spirochete T pallidum. Infection occurs
mainly through contact with individuals in the primary and latent
stages of the disease (within the first couple of years) and is
acquired through direct sexual contact, with risk rates of 10-60%.
Infection is through the skin and mucous membranes, and it leads
to the chancre after a 2- to 4-week incubation period. Primary lesions
last approximately 2 weeks, with healing occurring over 6 weeks.
Presentation usually is with a painless penile ulcerated lesion
(chancre). A small red papule breaks down to form an indurated ulcer
with a nonpurulent base. The lesions are located most commonly on
the glans, foreskin, or scrotum, although up to 5% may present at
extragenital locations. Anorectal lesions also are common in infected
homosexual males and may present clinically as anal fissures. Nontender
inguinal adenopathy also may be present.
Diagnosis is established with scrapings from the ulcer base examined
by darkfield microscopy to detect the characteristic corkscrew motility
of the organism or with fluorescence microscopy with antibodies
to T pallidum. Serologic tests, including nontreponemal antibody
titers via microhemagglutination assays (venereal disease research
laboratory [VDRL], rapid plasma reagin [RPR]), can be used for screening
and correlate with disease activity. Tests can decrease titers after
1 year. Increases by 4-fold indicate treatment failure or reinfection.
A positive diagnosis can be obtained 7 days after chancre formation.
False-positive results have been associated with collagen vascular
disease, liver disease, HIV, and herpes infections. Thus, a positive
test should be clarified using a second, more specific test such
as the fluorescent treponemal antibody adsorption test (FTA-ABS).
The differential diagnosis includes chancroid (see Chancroid) and
lymphogranuloma venereum (see Lymphogranuloma Venereum), both of
which are more confusing when presenting in atypical fashion or
in individuals positive for HIV. Careful clinical examination and
the preparation of smears for analysis lead to the correct diagnosis.
The possibility of superimposed infections also should be considered
when the presentation is atypical.
Treatment with penicillin G (2.4 million U in single dose) still
is the preferred and recommended treatment. Often, a second dose
is administered a week later, a treatment that can yield a serologic
cure. Doxycycline (100 mg bid for 2 wk) or tetracycline is an alternative
in patients sensitive to penicillin. Patients should be aware that
treatment might result in a transient acute febrile reaction with
accompanying headache and myalgias (Jarisch-Herxheimer reaction)
that usually occurs within 24 hours after initiation of treatment,
especially in early cases. Treatment failures occur in all groups,
thus serology must be checked at 3, 6, and possibly 12 months to
ensure a decrease in antibody titers and a lack of reinfection.
If left untreated, half the patients progress directly to secondary
syphilis, with a variety of clinical signs and symptoms due to the
hematogenous spread of the organism. Generalized constitutional
symptoms, including headache, fever, and sore throat, are associated
with mucocutaneous signs including a maculopapular rash that favors
the extremities and mucous patches with central erosion. Eventually,
the untreated course leads through a latent period (during which
the patient continues to be infectious) to tertiary syphilis with
multiorgan involvement. If the VDRL/RPR has not been reduced, a
CNS fluid examination should be performed to rule out CNS involvement
as a potential persistent reservoir. In addition, the CDC recommends
that patients also be encouraged to check their HIV status. The
clinical course of syphilis in patients infected with HIV often
is atypical and can be accelerated.
Granuloma inguinale (donovanosis) is a sexually transmitted disease
that is extremely rare in the United States, with only 8 cases reported
to the CDC in 1997. See Granuloma Inguinale (Donovanosis). Occurring
predominantly in tropical areas, including southeast Asia, India,
the Caribbean, and aboriginal Australia, possible outbreaks may
be related to travel from endemic regions. The papules are caused
by Calymmatobacterium granulomatis and are readily treated with
antibiotics when recognized. A prominent problem is the delay by
patients in seeking treatment, leading to the need for aggressive
treatment once they present with locally aggressive or systemic
C granulomatis, a gram-negative pleomorphic obligate intracellular
bacillus, is responsible for the disease and is present within histiocytes
in the ulcerated lesions. The actual rate of infectivity is low,
ranging from 0.4-0.5%, and extended incubation periods of 1-12 weeks
are common. The disease is characterized by multiple subcutaneous
papules that coalesce to form large ulcers where the infectious
bacillus can be found within histiocytes. Autoinfection is common
with the formation of so-called "kissing" lesions. While
not as common as in chancroid, inguinal lymphadenitis can occur,
with occasional erosion and bubo formation.
Typically occurring in patients aged 20-40 years, early presentation
is characterized by single-to-multiple papules that form red nontender
firm ulcers with bleeding granulation tissue bases. This ulcer is
the clinical hallmark of the disease, and without treatment, progressive
local tissue destruction occurs. Lymphadenopathy results more from
bacterial superinfection than from actual donovanosis and, thus,
is uncommon. While the classic presentation is recognizable to the
vigilant, granuloma inguinale can take on a variety of phenotypes,
with more severely ulcerative lesions, large dry vegetative masses,
and painful foul-smelling necrotic variants. When an atypical presentation
occurs, the presence of a superimposed infection also must be considered.
The histologic appearance of the prototypical ulcer reveals acantholytic
epidermal borders with pseudoepitheliomatous hyperplasia. The ulcer
bed contains prominent granulation tissue and vascular ectasia.
Microabscess formation is present, and the abscesses contain large
histiocytes with Donovan bodies (cytoplasmic vacuoles containing
dark particulate material composed of bipolar staining bacilli).
The identification of these Donovan bodies still is considered the
simplest standard for diagnosis. Diagnosis can be made by taking
tissue from the ulcer base and immediately crushing it between slides
and staining with Giemsa or Wright stain. The bacilli, which appear
as safety pin-shaped organisms, are readily identifiable within
macrophages. Reliable culture is not available, with the last documented
successful culture in 1962. Recent attempts at growth using coculture
with monocytes have been reported. Work on an indirect immunofluorescence
test also has been described.
While the classical presentation is rather pathognomonic, variant
presentations may confuse the diagnosis. The verrucous form may
simulate condyloma (see Condyloma Acuminatum) or carcinoma, and
the necrotic variant may lead to consideration of chancroid (see
Chancroid). Smear cytology with stains should demonstrate the correct
First-line treatment is with doxycycline (100 mg bid) or trimethoprim-sulfamethazine
(TMP-SMZ) (double-strength bid) with treatment for at least 3 weeks
or until all lesions have cleared. The CDC recommends ciprofloxacin
(750 mg bid) or erythromycin (500 mg qid) as second-line treatments
for refractory disease. Tetracycline resistance has led to this
antibiotic no longer being recommended for treatment.
Treatment usually is effective, but relapses do occur within 6-18
months. Monitoring of patients is recommended. Patients with granuloma
inguinale are prominent reservoirs for HIV. While granuloma inguinale
does not tend to produce generalized symptoms, systemic dissemination,
if left untreated, can lead to death. Lymphatic obstruction can
lead to lymphedema and scrotal elephantiasis. Scarring and fibrosis
also are well-documented sequelae of untreated locally aggressive
disease and may require surgical intervention for repair.
This sexually transmitted disease (see Chancroid) is found primarily
in Africa, the Caribbean basin, and southwest Asia and is rare in
the United States, where incidence is decreasing except among black
and Hispanic heterosexual men. Of the outbreaks in the United States,
85% are in New York, California, Texas, and South Carolina and are
associated with prostitution and crack cocaine use. It is the most
common cause of genital ulcers in the developing world.
The causative organism is Haemophilus ducreyi, a gram-negative bacterium.
It adheres to the surface of epithelial cells and produces cytotoxins
that are associated with cellular damage and ulcer formation.
Presentation occurs 3 days to 2 weeks after exposure. Tender erythematous
papules develop into painful acute ulcers 0.3-2 cm in size. They
have a gray-yellow foul exudate; the base is friable, and it bleeds
easily. In half the cases, chancroid is associated with painful
lymphadenopathy. Approximately 25% of these patients experience
progression of the lymphadenitis to a suppurative bubo that ulcerates.
Histologic features include the identification of 3 zones, a surface
fibrinoid exudate with neutrophils and debris, an intermediate zone
of vascular proliferation and thrombosis, and a deep zone of lymphoplasmacytic
Bacteria can be identified but often are easier to see in smears.
Diagnosis by morphologic features alone is only 30-50% accurate.
Gram stain of the ulcer base, revealing gram-negative coccobacilli
in chains and clusters (described as the railroad-track/school-of-fish
pattern) is helpful, although the polymicrobial flora of the ulcer
bases makes diagnosis based on this difficult. Cultures are definitive,
although the growth of the organism is problematic and must be performed
using a mixed biplate culture method. Success in different laboratories
varies from 0-80%.
Atypical presentations are common, often because patients have more
than one disease. Because of the difficulty in diagnosis, the CDC
recommends establishing a provisional diagnosis based on one or
more painful genital ulcers, clinical presentation with lymphadenopathy
suggestive of chancroid-negative T pallidum, and herpes simplex
virus (HSV) laboratory results.
Genital herpes and syphilis can mimic chancroid (see Syphilis).
They often are found coincidentally due to the high rate of multiple
infections in susceptible individuals. These diagnoses can be eliminated
based on laboratory results and clinical impression. In particular,
suppurative inguinal adenopathy is pathognomonic of chancroid.
Treat with antibiotics. Differences in local sensitivity have been
noted, and H ducreyi can acquire both gram-negative and gram-positive
resistance factors. Current antibiotic treatments include a choice
of the following: erythromycin base at 500 mg qid for 7 days, ceftriaxone
at 250 mg IM single dose, or azithromycin at 1 g PO as a single
dose. Ceftriaxone was effective in the United States but has been
associated with treatment failures in Kenya. Fleroxacin has been
effective in Kenya. TMP-SMZ has been ineffective. Due to the nature
of the disease, sexual partners also should be treated. Vaccines
for chancroid currently are in development.
Antibiotic regimens should improve symptoms in 3 days; ulcers heal
and adenopathy decreases in 7 days. The at-risk population also
is at risk for HIV-1. Patients with HIV do not respond as well to
the outlined antibiotic treatments.
DISORDERS OF THE MALE GENITALIA
Often present in the genital and perianal skin, lichen sclerosus
et atrophicus is referred to as balanitis xerotica obliterans when
it involves the glans or prepuce, especially in the later stages
of the disease (see Balanitis Xerotica Obliterans). This benign
disorder of unknown, possibly autoimmune, etiology is observed in
uncircumcised males and is an established cause of phimosis and
meatal stenosis. Typically following an insidious course, it is
associated with scarring and fissure formation. Treatment with corticosteroid
immunosuppressive regimens improves the outcome dramatically.
Although it often develops in older men, one study reported the
disorder in as many as 14% of prepubertal boys at circumcision.
It is considered a form of inflammatory sclerosing dermatitis, with
atrophy similar to lichen sclerosus of the vulva. Although balanitis
xerotica obliterans has been associated with phimosis (14% of cases
in one series) and observed with penile squamous cell carcinoma,
most cases do not show such a relationship.
In preliminary descriptions, the cause was uncertain. However, recent
evidence suggests an autoimmune origin. Autoimmune antibodies, including
antismooth muscle or antiparietal cell antibodies, have been noted
in these patients. Other associated autoimmune disorders include
vitiligo, alopecia areata, hyperthyroid and hypothyroid disorders,
pernicious anemia, and diabetes mellitus. The association with specific
human leukocyte antigen (HLA) types, including HLA-A29, B44, B40,
and Aw31, is significant. Researchers suggest dysregulated T-cell-mediated
dermal immune response leads to destruction of the dermal collagen
and basement membrane and exposure of keratinocytes to the dermis.
Balanitis xerotica obliterans occurs in young males (up to 14%)
and older men (40%) who present with phimosis and undergo circumcision
and usually involves the glans, prepuce, or urethral meatus, and,
occasionally, the shaft. The clinical presentation is that of a
well-defined, flattened, pale gray-to-white/ivory patch that looks
like wrinkled cigarette paper or roughened lichenoid scale with
prominent margins, usually involving the ureteral meatus. Often,
the advanced lesion is firm due to the underlying dermal fibrosis.
Unlike the pruritus associated with vulvar lesions, male lesions
often are asymptomatic, although sometimes they itch or burn. While
diagnosis often is based on clinical grounds, histology reveals
characteristics of lichen sclerosus et atrophicus, including prominent
orthokeratosis and hyperkeratosis in a setting of marked atrophy.
Areas of hyperplasia and atrophy may alternate.
Cytologic atypia is not present within the epithelium. Basal cell
vacuolization and clefting may occur, along with occasional bullae
formation. Also, no associated orthokeratotic plugging of hair follicles
exists because of a lack of follicles in commonly affected areas.
Initially, the dermis is markedly edematous, with superficial homogeneous
pale pink homogenization. It becomes progressively collagenous and
hyalinized in advanced cases and may be associated with a mild lymphoplasmacytic
The differential diagnosis includes vitiligo (see Vitiligo), due
to the presence of postinflammatory pigmentation changes and dermatophyte
reactions with associated pruritus. Most of these can be eliminated
based on clinical history, KOH scrape preparation, or biopsy if
Often, circumcision is the treatment for foreskin lesions. Topical
agents, such as hormonal testosterone creams, petrolatum, and antifungals,
are largely ineffective. While high-dose topical steroids have been
used with success in women (in some cases even reversing the course
of disease), they have not been studied in detail in men. The promising
results in women suggest the need to examine these treatments in
men. Based on limited studies in men, clobetasol propionate 0.05%
cream bid for 3-4 months may be a promising treatment option. However,
long-term steroid treatment may cause local atrophy or lead to reactivation
of latent infections such as HPV. Other treatment modalities involving
surgery, laser therapy, and retinoids produce limited success.
Association with squamous cell carcinoma has been reported, although
a direct relationship of the 2 disorders is not well established.
Overall a relatively uncommon skin disorder of unclear, possibly
autoimmune, etiology, lichen planus involves the destruction of
epithelial cells by the patient's T lymphocytes (see Lichen Planus).
This slowly progressing lesion may respond to topical steroids and
fade, leaving postinflammatory hyperpigmentation. While usually
occurring in women, lichen planus may involve the male genitalia.
The glans is a common site.
Usually, onset of genital lichen planus is in the fourth to sixth
decades. Its course is slowly progressing and insidious. The exact
etiology is unknown. However, a possible delayed hypersensitivity
reaction to an unknown epidermal antigen is the current theory.
The cellular immune response to basal epithelial cells causes initial
destruction of the basal cell layer by T cells. Familial cases and
cases associated with liver cell abnormalities, including chronic
active hepatitis and primary biliary cirrhosis, have been reported.
HLA-DR1 is associated with lichen planus. A more severe form associated
with erosive gingivitis is argued to be similar to vulvovaginal
syndrome in females.
The patient presents with several dome-shaped, markedly pruritic,
pink-purple papules/plaques, which can have delicate white lines
(Wickham striae), similar to those described in the oral cavity,
crossing the surfaces. Often excoriated, these lesions commonly
can present as multiple violaceous papules, except on the glans,
where it can adopt an annular configuration. Lichen planus can ulcerate
on surfaces subjected to trauma or on mucosal surfaces. The histologic
appearance is that of acanthosis, hyperkeratosis, wedge-shaped hypergranulosis,
and a dense bandlike inflammatory infiltrate that obliterates the
dermal-epidermal junction, with basal cell vacuolization and Civatte/cytoid
body formation. On mucosa, parakeratin with the characteristic inflammatory
infiltrate may be present.
The differential diagnosis includes psoriasis, which usually presents
with less pruritus. Often, psoriasis involves other portions of
the body, including the limbs, where it shows the more characteristic
silver scale. The scaling of psoriasis usually is not present in
genital lesions. Tinea cruris (see Tinea Cruris) tends to present
with a raised border, a more cleared central area, and a peripheral
scaling reaction. Candidiasis (see Candidiasis, Cutaneous) presents
more acutely with erythema, edema, and satellite lesions. Balanitis
xerotica obliterans (see Balanitis Xerotica Obliterans) shows atrophy
and plugged hair follicles.
Topical steroids have been used to treat of lichen planus. Courses
of 4-6 weeks are necessary if postinflammatory hyperpigmentation
is present for extended periods after treatment. Symptomatic relief
with use of topical steroids (and even IV prednisone) has been reported.
Whether they actually affect the clinical course is still in doubt.
In some steroid-resistant cases without response to steroids (steroid
resistance), treatment with cyclosporin A (3 mg/kg/d) is reported
to lead to partial regression after 2 weeks, allowing for surgical
intervention with circumcision.
Characteristically, lichen planus resolves spontaneously and does
not require treatment. Regardless of the method of treatment, most
lesions heal in 6-18 months. These lesions can be associated with
lichen planus at other sites, in particular, mucocutaneous involvement.
Occasionally, lichen planus can result in phimosis, which necessitates
subsequent circumcision. Squamous cell carcinoma has been reported
to arise in lichen planus of the penis. In one case, the lesion
was treated by Mohs surgery, and the patient was reported free of
tumor 2 years postoperatively.
Named in 1817 by Bateman, molluscum contagiosum is a highly contagious
viral infection that can present in the inguinal region (see Molluscum
Contagiosum). It is characterized by umbilicated papules and has
a distinct histologic appearance. Treatment usually involves curettage
or topical remedies, and outcome is self-limited.
The causative agent is a DNA-containing poxvirus that infects the
epithelium and leads to cellular proliferation and production of
viral particles. These particles are then released by cellular rupture
and lead to subsequent infection of neighboring cells. After an
incubation period of 2-7 weeks, multiple papules surface and last
for 2-6 months. Autoinfection is common, and treatment is focused
on eradicating the virus and breaking the viral cycle.
In adults, molluscum contagiosum is transmitted sexually. It also
can be acquired naively by children, usually presenting on the face,
extremities, and upper trunk. Adult patients present with multiple,
discrete, dome-shaped, 3- to 6-mm papules on the inner thigh, penis,
and scrotum. The hallmark of central umbilication appears in only
25% of the lesions. These papules can have a pedunculated gross
appearance, and milky-white material can be extruded from the central
The characteristic histologic appearance of the lesions is a cup-shaped
or cystlike invagination of epidermis with acanthosis. Epidermal
cells from the stratum malpighii to the more superficial layers
have characteristic viral cytoplasmic inclusions, (ie, molluscum
bodies [Henderson-Patterson bodies]). These bodies initially are
eosinophilic but become more basophilic as they progress to the
surface of the epidermis and fill with poxvirus particles. The stratum
corneum ruptures and releases the molluscum bodies at the surface,
creating the characteristic milky-white material that can be extruded
from the gross lesions.
Clinical differentials include candidal infections when lesions
are surrounded by an erythematous halo and folliculitis, which molluscum
can mimic in atypical presentations (see Candidiasis, Cutaneous).
Larger outbreaks with coalescent lesions, usually in patients who
are immunosuppressed, may mimic furuncles or plaques. Disseminated
histoplasmosis and cryptococcosis can mimic molluscum infections
in individuals with HIV (see Histoplasmosis and Cryptococcosis).
Molluscum can involve cysts and is included in the differential
diagnosis of cystic sebaceous glands. Both can be diagnosed based
on histologic examination.
Treatment is curettage, cryotherapy, or laser vaporization. Application
of podophyllin or silver nitrate to ablate the lesions has been
used with mild success.
While most lesions regress in 6-12 months, treatment prevents autoinfection
and lowers the transmission risk. Involution occurs without scarring,
and, on average, the full cycle, including reinoculation, runs a
2-year course. In patients who are immunosuppressed (including patients
with HIV), multiple lesions can present as giant papular lesions
that fail to regress, requiring more aggressive ablation. Treatment
in these patients is centered on control of the spread rather than
eradication of the virus.
This viral vesicular eruption, due to HSV, is the most common cause
of genital ulcers in developed countries (see Herpes Simplex). Most
cases are HSV-2 (US seroprevalence of 22%). Higher seroprevalence
rates are reported in inner cities and developing countries. Also,
HSV-1 is emerging as a significant cause of genital herpes. In 5-30%
of initial cases, HSV-1 is involved. Because the virus can establish
a latent infection, recurrent clinical manifestations are common,
but their impact can be lessened with antiviral treatment.
HSV is caused by the sexual transmission of a double-stranded DNA
herpesvirus that initiates a surface epithelial infection that subsequently
involves the sensory nerve ganglia. The virus then establishes a
latent state in these nerve cells that can be reactivated through
multiple mechanisms including stress, sunlight, skin trauma, and
cold or heat. They are mediated by prostaglandins, in particular
prostaglandin E2 (PGE2) and prostaglandin F2 (PGF2). Recurrence
is more common in men than in women and occurs more often in whites
than in blacks.
Transmission occurs through viral shedding from active lesions and
asymptomatic individuals; symptomatic lesions are more efficient
in transmitting the virus. Most transmissions (up to 70%) occur
during periods of asymptomatic shedding. While most cases are associated
with HSV-2, HSV-1 is responsible for a larger share of sexually
transmitted viral infections. Of note, transmission of HSV-1 is
less effective than HSV-2 and is associated with lower rates of
clinical recurrence and transmission.
Most patients are clinically asymptomatic; fewer than 10% report
a history of infection. Initial symptoms may occur 2-4 weeks after
infection, or they may delay for years after the initial exposure.
Symptoms are systemic (fever, malaise, headache) and are more common
in women than in men. Localized itching, pain, and dysuria can occur.
Genital lesions include multiple vesicles with red bases that form
pustules, rupture, and crust over.
A Tzanck smear can identify multinucleated epithelial cells with
intranuclear inclusions and can establish a diagnosis in the right
clinical setting; however, it is neither sensitive nor specific.
Histologic appearance is of marked acantholysis with vesicles that
contain proteinaceous fluid and degenerating cells, often with multinucleated
forms. Characteristic intranuclear viral inclusions of HSV are noted.
Culture of the virus can differentiate the 2 types, although sensitivity
is only 50%, and the cultures must be established within the first
3 days of symptoms. Commercially available serologic tests using
fluorescent antibodies cannot distinguish between HSV-1 and HSV-2.
The differential diagnosis is limited for herpes but includes chancroid
and granuloma inguinale, both of which can be differentiated based
on the different incubation periods, presence or absence of lymphadenopathy,
and laboratory serologic tests. See Granuloma Inguinale (Donovanosis)
Elimination of the virus is not possible. Treatment centers on symptom
control and infectivity. Initial clinical episodes should be treated
with acyclovir (400 mg tid), famciclovir (250 mg tid), or valacyclovir
(1 g bid) for 7-10 days or until ulcer healing is complete. These
drugs shorten the duration of the lesions duration and reduce viral
shedding but do not change the severity or frequency of recurrences.
If antiviral treatment is initiated within 24 hours of recurrences,
it can lessen the impact of the developing lesions in as many as
25% of patients.
Continuous treatment with acyclovir has been shown to reduce asymptomatic
shedding and clinical outbreaks. As many as 80% of patients are
symptom-free at 5 years. The decision to place a patient on continuous
antiviral therapy is subjective and depends on the severity of recurrences
and the psychosocial factors of the patient. CDC recommendations
for continuous treatment are acyclovir at 400 g bid, famciclovir
at 250 mg bid, or valacyclovir at 500-1000 mg qid, depending on
the number of recurrences (> or <10 per y).
Clinical manifestations are most severe in the first episode and
can last up to 15 days. For 80-90% of patients who do not receive
continuous antiviral treatment, recurrent episodes occur in the
first year, with subsequent recurrences averaging 4 per year for
HSV-2 and 1 per year for HSV-1. HSV is recurrent in 50-65% of patients,
usually with gradually decreasing frequency. Disseminated herpes
infections can occur in individuals who are immunosuppressed and
those with complications associated with HSV with hepatitis and
Condyloma acuminatum is the most common warty lesion of the penis,
estimated to be present in as many as 5% of adults aged 20-40 years
(see Condyloma Acuminatum). This sexually transmitted viral disease
has a characteristic histology, extensive pathobiology, and established
treatment options, but no known cure.
These epidermal lesions of disordered keratinocyte proliferation
are caused by members of HPV, most commonly types 6 and 11 (90%
of lesions), although in condyloma with evidence of epithelial dysplasia,
types 16, 18, 31, 33, and 35 are noted. These DNA viruses spread
through sexual intercourse and infect the keratinocytes, leading
to the disordered proliferation of cells and subsequent formation
of the condylomatous lesion. Basic biology has elucidated specific
viral proteins involved in the disruption of the proliferative regulatory
machinery of the keratinocyte, leading to cellular proliferation.
In particular, viral proteins E6 and E7 lead to the sequestration,
degradation, and disruption of 2 key cellular proteins, the retinoblastoma
protein (RB) and p53, leading to a loss of cell-cycle control and
the continued multiplication of cells.
Condylomata are associated with sexual transmission. In fact, the
association between men with penile condylomata and sexual partners
who have cervical lesions is high (50-85% of males whose sexual
partners have HPV lesions have penile lesions).
Most often, condylomata are located at the corona of the glans or
adjacent to the meatus, but they can occur on the scrotum and perineum.
Frequently, they present as papillary cauliflowerlike lesions and
can measure up to a few centimeters across. Also, flat lesions can
occur. Detection using 5% acetic acid can reveal these and subclinical
lesions as white areas (flat condylomata). Of note, the sensitivity
and specificity of acetic acid detection of condylomata in males
is not well established, and treatment based on the eradication
of acetowhite areas is not a proven and recommended guideline.
Histology reveals proliferation of the squamous epithelium with
acanthosis and papillomatosis. Orderly maturation, minimal cytologic
atypia, and mitoses confined to the basal cell layer (key features
lost in dysplasia and squamous cell carcinoma) are noted within
the specimen. Hyperkeratosis, parakeratosis, and koilocytosis are
Differential diagnoses (all that can be ruled out based on clinical
history and histologic evaluation) include condyloma latum, seborrheic
keratoses, nevi, and pearly penile papules. See Seborrheic Keratosis
and Pearly Penile Papules.
The goal of treatment is to remove warts and treat symptomatic problems.
No treatment eradicates HPV infection. Treatments focus on the destruction
or removal of the lesion and include cryotherapy, laser ablation,
and surgical excision with histologic evaluation. Treatment with
podophyllin has been successful, but it can cause cytologic atypia
that may be misdiagnosed as carcinoma on subsequent biopsy. Also,
podophyllin is not recommended for urethral lesions, where 5% 5-fluorouracil
or thiotepa is preferred. Topical administration of imiquimod, an
immune response modifier, has shown clinical utility.
While condylomata may regress spontaneously, approximately half
persist and thus necessitate treatment with podophyllin, cryotherapy,
or laser ablation. Because it is sexually transmitted, sexual partners
are at higher risk for cervical dysplasia and should be screened
for HPV infection. Because it is sexually transmitted, sexual abuse
must be suspected if children are diagnosed with condylomata.
MISCELLANEOUS BENIGN LESIONS OF THE MALE GENITAL TRACT
Present in 8-20% of males, pearly penile papules (hirsutoid papillomas)
are benign asymptomatic papules that may represent embryological
remnants (see Pearly Penile Papules). Often, they are the cause
of much consternation for the patient and are misdiagnosed by the
Similar lesions have been noted in cats, dogs, and chimpanzees.
These small papules are best described as embryological remnants.
Pearly penile papules usually present in adolescence as multiple-grouped
lesions along the corona and sulcus. Histologically, dilated ectatic
vessels present in a fibrous stroma, and areas of epithelial thickening
over this fibrovascular lesion have been described. Many authors
believe these lesions are angiofibromas due to their histologic
similarity. They tend to be fixed, do not regress, and can recur
after incomplete excision.
These tend to present as 1- to 3-mm diameter, yellow-white, domelike
papules arranged in a row on the corona of the glans in adolescent
males. Presentation may occur in males in their second to fifth
decades. Rare cases in children as young as 11 years have been reported.
While asymptomatic, they are a source of great concern, and they
often are presented as a sexually transmitted disease.
The differential diagnosis includes molluscum contagiosum and condyloma
acuminatum. These diagnoses can be ruled out based on clinical history
and histologic findings (see Molluscum Contagiosum and Condyloma
These lesions require no treatment, although the patient may request
excision for cosmetic reasons. They are of no clinical significance.
In general, penile cysts are not common but have been well documented.
The largest series presented (160 cases) was from Japan. Due to
their asymptomatic nature, penile cysts may not come to clinical
attention and may be more common.
These cysts often are divided into 2 main categories, median raphe
cysts and epidermal inclusion cysts. Follicular cysts (epidermal
inclusion cysts) are the most common cystic lesions of the penis
and occur on the shaft. Median raphe cysts have various names, including
mucoid cysts, urethral cysts, and apocrine cystadenomas. All are
benign and can be treated with simple excision.
Follicular cysts (epidermal inclusion cysts) are believed to arise
from hair follicles along the penile shaft due to blockage of the
follicular infundibulum. Median raphe cysts arise from the urethral
and paraurethral epithelium. Follicular cysts tend to be located
at the base of the shaft in association with hair follicles and
are not present at the urethral meatus. Median raphe cysts originate
from the external paraurethral ducts (reflected by their site of
origin) and present in association with the external meatus, urethra,
or along the median raphe. Most median raphe cysts are lined by
a stratified columnar epithelium and contain clear mucinous fluid.
Electron microscopy reveals an intact underlying myoepithelium and
secretory features, including microvilli and secretory granules
characteristic of duct-lining epithelium as found in the paraurethral
Histologically, apocrine metaplasia is common. In some cases, squamous
metaplastic epithelium leads to misdiagnosis of an epidermal inclusion
cyst. These cysts are believed to arise from the paraurethral ducts
that formed from the lacunae of Morgagni/branched paraurethral glands
of Littré. Some have a direct connection to the urethra,
and some lack communication. The cysts are present all along the
median raphe, from the urethral meatus to the anus.
Most patients are asymptomatic and present with a painless mass.
Often, patients have this lesion for quite some time. Forty patients
in one study presented more than 20 years after onset, while in
another set of 3 patients, the length of time to presentation was
3-26 years. Cysts range in size from 0.2-2 cm in diameter. Most
present along the median raphe of the penis shaft.
The differential diagnosis includes follicular cysts and median
raphe cysts. These differ in clinical location (base of shaft vs
along the median raphe) and histology (squamous infundibular epithelium
Usually, treatment involves complete excision, including communicating
Excision removes the cyst and subsequent risk of infection or rupture,
usually from intercourse. Take care when excision involves cysts
adjacent to the urethral meatus to prevent damage to the urethra.
Documented recurrence in up to 2% of cases usually is due to incomplete
excision of median raphe cysts that communicate with additional
Hypertrophic keloidal scars, although rare, can occur on the penis,
most commonly after surgery such as circumcision (see Keloid and
Hypertrophic Scar). This exuberant response to injury can be controlled
with steroid injections and surgical removal.
The postulated mechanism involves increased fibroblastic activity
and collagen deposition that occludes local vessels and leads to
ischemia, tissue damage, and additional fibroblastic repair reaction
resulting in a positive feedback loop. A component of inflammatory
response also may be crucial in the development of this disorder.
Keloids occur in patients aged 8-56 years and are associated with
previous surgery for injury to the penis. While circumcision most
often is the previous procedure, patients have been described with
keloid formation secondary to hidradenitis suppurativa. Often, patients
seek clinical care because of embarrassment and discomfort associated
with sexual activity. Clinical examination reveals a large firm
mass present at the site of tissue injury. Biopsy reveals abundant
deposition of collagen in the dermis, with associated plump fibroblasts,
thrombosed vessels, and inflammatory cells. Characteristic markedly
thickened collagen bundles look like wax dripping.
The differential diagnosis includes both benign and malignant tumors
of the penis. Usually, these can be eliminated from the differential
based on histologic analysis, rate of growth, and clinical presentation.
In particular, penile neurofibromas have been described in association
with neurofibromatosis, and reports of both benign and malignant
schwannomas of the penis have been recorded.
Treatment includes intralesional steroid injections (triamcinolone
acetate once a mo for 7 mo) and surgical excision. Recurrence still
While recurrence is possible, treatment often is satisfactory and
allows return to sexual function.
Diseases of the Male Genitalia: Malignant
malignant dermatologic lesions of the male genitalia are squamous
in origin and associated with the human papillomaviruses (HPV).
Rarely, tumors are derived from the cutaneous adnexa, melanocytes,
or the urethral urothelium. Cutaneous metastases to the penis are
strikingly uncommon and, when present, usually are in the setting
of widely disseminated disease in an immediately antemortem patient.
An area of ongoing debate is the clinical definition of dysplasia
or in situ carcinoma of the penis and scrotum. Initial descriptions
were based on the variety of clinical presentations and associations
with other tumors, which led to the description of 3 preneoplastic
disorders: Bowen disease, erythroplasia of Queyrat, and bowenoid
papulosis. The underlying pathologic similarity of these disease
entities has been reinforced with the isolation of similar HPV subtypes
in each disorder. As more cases are gathered, initial differences
in the rate of progression to invasive carcinoma and the association
with underlying internal malignancies are becoming less significant.
In other words, what initially appeared to be 3 separate clinical
entities may in fact be just the clinical spectrum of a single disease,
all initiated by the same causative agent (HPV) and modified by
associated immune and host defense mechanisms.
Variations in the clinical presentation of invasive carcinoma also
lead to dissimilar predictions of outcome. Squamous cell carcinoma
of the penis may present as specific associated variants (ie, exophytic/fungating
type, verrucous type, invasive/ingrowing type). Similar differences
are difficult to identify in melanoma or urothelial carcinomas of
the male genitalia because of the limited number of cases to evaluate.
Initially described by Tarnowsky in 1891, erythroplasia of Queyrat
(see Erythroplasia of Queyrat (Bowen Disease of the Glans Penis)
was separated from Bowen disease based on clinical appearance and
lack of association with internal malignancy. Today, the overlap
between these 2 entities is more evident, and separation of the
2 remains controversial. Histologic appearance and rates of progression
to squamous cell carcinoma are similar. The presence of an association
with an internal malignancy may have more to do with the age of
the patients and has been called into question for both diseases.
Usually, erythroplasia of Queyrat occurs in men aged 46-60 years,
although it can occur at any age. The histologic features suggest
a preinvasive squamous epithelial lesion that has demonstrated a
definite risk (up to 10%) for progression to invasive malignancy.
HPV has been isolated from this disease, including high-risk serotypes.
Patients present mainly with bright red, minimally elevated, glistening,
velvety plaques. Often, erythroplasia of Queyrat involves the glans
or prepuce. Occasionally, presentation can mimic a cutaneous horn.
In half the patients, erythroplasia of Queyrat is a solitary lesion
and often is present for months to years before clinical attention
is sought. The histologic examination is similar to bowenoid papulosis
and Bowen disease, with features of carcinoma in situ, including
the following: full thickness alteration of the squamous epithelium,
a loss of polarity, cytologic atypia, mitoses above the basal layer,
dyskeratosis, and atypical mitoses. The underlying dermis has a
marked chronic inflammatory infiltrate and vascular proliferation,
but it does not show evidence of invasion of tumor into the underlying
Differential diagnosis includes Zoon balanitis (see Balanitis Circumscripta
Plasmacellularis) or any other nonhealing lesion that does not respond
to medical treatment. Because of the risk of malignancy, these nonresponding
lesions require biopsy to rule out this and other premalignant or
malignant processes. Other lesions to be considered include drug
eruption (see Drug Eruptions), psoriasis, or lichen planus (see
Treatment of erythroplasia of Queyrat often involves removal or
destruction of the lesion. Methods employed include cryotherapy,
laser ablation, and surgical excision. Topical vesicants have been
used for local control. Some authors recommend a surgical excision
with a 0.5-cm margin around the lesion. This has the advantage of
allowing pathologic examination of the specimen to rule out the
presence of invasive tumor. Also, Mohs surgery has been used in
the treatment of erythroplasia of Queyrat.
The argument has been made that circumcision protects against this
disease, possibly due to the decreased risk of poor hygiene associated
with the deposition of high-risk detritus, including smegma, HPV,
oils, and toxins. In a series of 100 cases, 22% of the patients
developed recurrence, 8% developed invasive squamous cell carcinoma,
and 2% developed squamous cell carcinoma with distant metastases.
Other groups also have reported metastases.
Described by Bowen in 1912, this disease (see Bowen Disease) was
considered unique due to its association with invasive squamous
cell carcinoma and internal visceral malignancy. Subsequently, the
rate of progression has been similar to that of erythroplasia of
Queyrat. Association with invasive malignancy has thus been called
Occurring in men aged 30-50 years, Bowen disease is an in situ histologic
change involving the shaft of the penis. It may be associated with
agents that damage skin, including ultraviolet light exposure and
topical toxins such as arsenic. Recently reported isolation of HPV
DNA places it in the category of virally induced squamous neoplasia.
Bowen disease usually presents on the shaft of the penis. It does
not have the red color of erythroplasia of Queyrat but is a more
crusted, sharply demarcated, scaly plaque. In rare examples, it
can be more papillomatous. The plaque measures from millimeters
to centimeters across. Often, it is silver-to-white in appearance
and can resemble psoriasis or dermatitis. Lesions may be pigmented.
Differential diagnosis includes Paget disease or squamous cell carcinoma
(see Squamous Cell Carcinoma). The silver scaly appearance can resemble
psoriasis or other dermatitis. Pigmented examples also can mimic
melanoma or benign nevi. Pathologists can misdiagnose histologic
atypia as condyloma with podophyllin treatment; thus, a clinical
history of podophyllin should be noted.
Often, treatment centers on excision and/or ablation. Surgical excision
provides a histologic diagnosis. In severe cases, partial penectomy
could be considered but only after more conservative measures have
been exhausted. Yttrium-aluminum-garnet (YAG) laser ablation also
has been reported as yielding satisfactory results.
The rate of progression to squamous cell carcinoma is 5-10%, similar
to that observed with erythroplasia of Queyrat. Of these cases,
30-50% are potentially metastatic. Initial reports suggested that
as many as 33% of patients with Bowen disease develop visceral malignancies
including respiratory, gastrointestinal, or urogenital cancers.
More recent studies cast doubt on this association.
In 1978, Wade first described bowenoid papulosis (see Bowenoid Papulosis)
as a specific disorder of young sexually active men. Patients presented
with multiple small red papules on the genitalia that, while histologically
concerning, tended to pursue an indolent clinical course.
Typically, bowenoid papulosis occurs in men aged 20-40 years. The
mean age at presentation is 29.5 years. Association with HPV infection
and isolation of high-risk HPV serotypes 16 and 18 from lesions
of bowenoid papulosis have been documented, although other serotypes
have been detected (31-32, 34, 39, 42, 49, 51-54). As with the histologically
similar lesions of Bowen disease and erythroplasia of Queyrat, an
association exists between bowenoid papulosis and progression to
squamous cell carcinoma, although the rate (2.6%) is lower than
that of erythroplasia of Queyrat or Bowen disease.
In addition, a link exists between bowenoid papulosis in males and
cervical dysplasia in their female partners. Some studies seem to
indicate that bowenoid papulosis is a clonal disorder. In some cases,
development of bowenoid papulosis is associated with anergy and
diminished helper T-cell response (not related to HIV), leading
to the notion that decreased antiviral immunity may lead to the
development of these premalignant lesions.
Patients typically present with multiple multicentric erythematous
papules on the penile shaft or the perineum. The papules, which
range from 2-10 mm, are not particularly itchy and do not tend to
scale or ulcerate. Lesions tend to develop over a short time in
young sexually active men who have multiple partners.
Lesions may be clinically mistaken for condyloma (see Condyloma
Acuminatum), seborrheic keratoses (see Seborrheic Keratoses), or
nevi. Lesions that histologically tend to be verrucous could resemble
condyloma but lack the microscopic full-thickness atypia. Usually,
seborrheic keratoses have histologic evidence of a keratinizing
granular layer, which is different from typical Bowen disease. Differential
diagnosis also would include nonspecific balanitis, including Zoon
balanitis (see Balanitis Circumscripta Plasmacellularis). The inability
to identify a causative organism or a lack of response to a trial
of antibiotics would warrant a biopsy to rule out bowenoid papulosis.
Histologic features, in particular the lack of an extensive plasmacytic
infiltrate, would differentiate from Zoon balanitis.
Because progression to carcinoma is rare, these lesions usually
are treated conservatively. Treatment centers on the ablation of
the gross lesion, often with the use of local excision, topical
vesicants, cryotherapy, or laser ablation.
Progression from bowenoid papulosis to squamous cell carcinoma differs
from Bowen disease and erythroplasia of Queyrat. While rare case
reports exist and dermatologic specialists see some cases, incidence
appears to be lower than other premalignant lesions. In some cases,
bowenoid papulosis may regress spontaneously. Because of the association
with HPV, this is considered a sexually transmitted disease. Studies
of partners of patients with bowenoid papulosis found them to be
at a higher risk for cervical dysplasia.
Squamous cell carcinoma (see Squamous Cell Carcinoma) is the most
common tumor of the penis, accounting for more than 95% of all malignant
penile tumors. Although rare in persons younger than 40 years, the
actual age range is extensive, from 20-90 years. Most cases are
present in uncircumcised men and are associated with poor hygiene.
Areas of high rates of penile squamous cell carcinoma include Uganda,
Brazil, Jamaica, Mexico, and Haiti.
Initial reports linked penile squamous cell carcinoma with the uncircumcised
penis and documented high rates of tumors in uncircumcised Hindus
and low rates among Jews who practice ritual circumcision. Later
studies documented the low rate of penile tumors among Scandinavians
who do not practice circumcision and indicated that the crucial
factor was not the foreskin itself, but the link between the presence
of the foreskin and hygiene. Squamous cell carcinoma of the penis
has been associated with phimosis, or retention of the prepuce,
although the presence of phimosis in itself indicates scarring from
a previous possible infectious inflammatory reaction. Recent attention
also has focused on smegma (oils, organisms, and debris that collect
under the prepuce with lack of good hygiene), associated penile
rashes, and infection by Mycobacterium smegmatis.
HPV infection is associated with 22-63% of these cases. Subtypes
linked to penile squamous cell carcinoma include high-risk types
16 and 18, as well as 6, 11, and 30. An association also exists
between penile cancer and spousal cervical cancer, although these
statistics have been debated. The microscopic appearance is that
of poorly differentiated cells, and these tumors are prone to high
rates of local spread and metastasis. They demonstrate invasive/vertical
growth patterns and often have associated necrosis and perineural
and vascular invasion.
The clinical presentation is not of a subtle lesion. Often, patients
delay seeking medical care for 6 months or longer because of embarrassment
or shame. Frequently, bacterial superinfection or induration of
the lesion results from attempts to self-treat with home remedies.
The majority of lesions are present on the glans, on the prepuce,
or at the coronal sulcus.
Presenting complaints often include penile pain, malignant priapism,
discharge, and difficulty voiding. Lymphadenopathy is present in
as many as 15% of presenting cases, particularly with the invasive/ingrowing
subtype. At the time of presentation, palpation for involved lymph
nodes is only 70% sensitive. Palpable nodes are present in 58% of
patients at presentation, but less than half of these are subsequently
found to have metastases. Often, the mass effect present at palpation
is due to inflammation of the nodes from tumor superinfection.
Tumors are examined after penectomy. They usually are pathologically
staged and are graded using the American Joint Committee on Cancer
(AJCC) tumor, node, and metastasis (TNM) staging and modified Broders
systems, respectively. The TNM system classifies cases into stages
I-IV based on the extension of the tumor. Most patients present
as stage I (confined to the glans) or II (involving the glans and
invading into the penile shaft). Histologic grading using the modified
Broders classification divides tumors into 4 histologic grades ranging
from well differentiated to poorly differentiated. Another grading
system uses a 3-tier (well, moderate, poor) scale for differentiation,
with 50% of cases being well differentiated. Of note, well-differentiated
tumors metastasize to regional lymph nodes at a rate of 50%, while
moderate or poorly differentiated tumors have an 80-100% nodal metastasis
rate. Hematogenous metastasis is uncommon, accounting for fewer
than 2% of cases at diagnosis.
While clinical presentation is unlikely to cause diagnostic dilemma,
the ability to recognize variants of squamous cell carcinoma is
of some clinical benefit. Specific subtypes and variants include
the verrucous exophytic-fungating/superficial spreading type and
the invasive/vertical growth pattern type.
The warty or verrucous variants, called the giant condyloma of Buschke-Lowenstein
(see Giant Condyloma Acuminata of Buschke and Lowenstein), are controversial
because of their histologic overlap and the unclear biologic separation
between these lesions. Often, these tumors have a prominent papillary
configuration that also is observed upon histologic evaluation.
Additional histologic features include a broad front of invasion
and minimal cytologic atypia. Metastasis is rare and, when present,
is associated with tumors of higher pleomorphism and microinvasion.
Instead, these tumors are characterized by indolent growth and local
recurrences at a rate of up to 80%. This rate can be reduced to
30% with partial penectomy. Aggressive treatment with radiation
can be detrimental, with subsequent dedifferentiation. In rare cases,
verruciform xanthoma mimics verrucous squamous cell carcinoma, which
can be separated based on biopsy.
Usually, sarcomatoid or spindle cell variants of squamous cell carcinoma
present as polypoid masses and appear to grow slowly. The actual
prognosis for these variants is mixed, with some doing as poorly
as typical squamous cell carcinomas. Cases with poor prognosis also
often have a carcinomatous component (carcinosarcoma) that is readily
identifiable. Differential diagnosis includes a synovial sarcoma
of the penis, of which case reports exist.
Treatment of choice is partial penectomy to control the spread of
low-stage disease. Some groups have argued for local excision with
conversion to partial penectomy upon recurrence, although this is
not widely accepted. The effectiveness of prophylactic bilateral
inguinal node dissection is controversial. Drainage from the penis
is midline and can involve either side of the pelvic lymph node
chain. In addition, high morbidity is associated with inguinal node
surgery. On the other hand, lymphadenectomy can be curative of regional
disease. The sensitivity for detecting involved lymph nodes drops
from 70% to 15% after surgery due to postoperative inflammatory
changes. Based on this, some advocate waiting for a few weeks until
the inflammation subsides and operating on residual nodal masses.
Groups also have applied lymph node mapping to penile cancers. Attempts
at organ-sparing surgery have produced mixed results.
Some have attempted to control local disease with radiation therapy
or have used radiation therapy for patients who are not candidates
for penectomy. Brachytherapy has been used with success for local
control of disease. The role of chemotherapy is unclear because
of the difficulty in gathering a sizable number of patients. Commonly
used agents include cisplatin and bleomycin and have yielded response
rates of 15-23%. Combination chemotherapy and radiation using bleomycin-derived
treatments have been successful in some cases, with response rates
as high as 43%.
Squamous cell carcinoma of the penis tends to be a locally advanced
aggressive disease. Nodal metastases, usually to the inguinal and/or
iliac nodes, are the most common route of dissemination. Of note,
5-15% develop second primary lesions, often located along the residua
of the penis. Usually, complications are due to recurrence, which
occurs in as many as 7% of patients.
Treatment usually focuses on local issues or the presence of bulky
nodal disease. While hepatic, pulmonary, and osseous metastases
have been documented, the development of hematogenous metastasis
is rare and occurs in untreated cases. The 5-year survival rates
for locally resected lesions range from 65-90%, but decrease to
20-50% if nodal metastasis initially were present. Usually, death
occurs within 2 years if left untreated.
BASAL CELL CARCINOMA
While basal cell carcinomas (see Basal Cell Carcinoma) are the most
common human tumor, they are distinctly uncommon on the male genitalia.
Most cases documented in medical literature have been case reports
or letters. Treatment is related to local control. Metastases are
uncommon. With complete removal, patient outcome is very good.
The propensity for development on sun-exposed skin explains the
rarity of basal cell carcinoma on the male genitalia. Originating
from the basal cells that generate the epidermis, these tumors tend
to be locally destructive and do not readily metastasize. Besides
sun exposure, risk factors include ionizing radiation, arsenic,
and familial heritable forms including nevoid basal cell carcinoma
syndrome and xeroderma pigmentosum.
Patients range in age at presentation from 22-81 years, with most
cases in older men. The majority of patients have fair skin, although
cases also have been reported in Japanese males and blacks. Most
lesions have occurred on the shaft and scrotum and were present
for years before patients sought treatment. The lesion is raised,
with an area of central ulceration, pearly white borders, and telangiectatic
edges. Histologic sections reveal nests of basaloid cells with peripheral
palisading and a notable retraction artifact separating the nests
from the dermis due to connective tissue mucin accumulating in the
The most important differential diagnosis is primary adnexal carcinoma,
such as eccrine porocarcinoma (see Eccrine Carcinoma). These tumors
tend to have a more aggressive growth pattern and higher rate of
metastasis and must be treated more aggressively. Differential diagnosis
is based on histologic examination and the identification of syringotropism,
lumen formation, and adnexal differentiation. Primary squamous cell
carcinomas (see Squamous Cell Carcinoma) also must be separated
because of their more aggressive course. Because of the nature of
the differential diagnosis, histologic evaluation is required.
Treatment for basal cell carcinoma focuses on local excision or
ablation. This is in contrast to partial penectomy, which would
be the choice of treatment for more aggressive tumors such as squamous
cell carcinoma. Different treatment modalities stress the need for
a definitive histologic diagnosis.
Complete excision of the tumors can lead to uneventful follow-up.
Incomplete treatment often results in local recurrence that can
be treated by removal.
Melanoma (see Malignant Melanoma) is a relatively uncommon lesion
of the male genitalia. Patients with melanoma have a poor prognosis
due to presentation at an advanced stage because of embarrassment.
While treatment still focuses on surgical intervention, long-term
survival has not been reported. Recent rare cases of melanoma in
situ with treatment by local excision may fair better.
Accounting for less than 2% of primary tumors of the penis, melanoma
presents on the penis in less than 0.2% of cases. It may originate
from the epidermal melanocytes or from the penile urethral urothelium.
Most patients present with advanced disease. Inguinal nodal metastases
are common (present in 43% of cases at presentation in one series).
Patients usually present when aged 50-70 years. One series documented
an age range of 57-77 years. While the most common presentation
is on the glans (55% in one series), melanoma has been described
on both the prepuce (28%) and shaft (9%). The lesions often are
macular with an irregular border and lack symmetry in their overall
appearance. Often, areas of variegated pigmentation ranging from
blue to black can be observed. When lesions have been present for
some time, they may ulcerate or erode the underlying skin. Often,
inguinal nodal enlargement can be palpated.
Differential diagnosis centers around melanoma in situ and benign
nevi or melanosis, both of which are rare on the penis. Postinflammatory
hyperpigmentation also may confuse the clinical presentation but
lacks the dark pigmentation observed in melanocytic neoplasia.
Treatment usually is partial penectomy with possible inguinal lymph
node dissection. Mapping is performed in some cases. Local treatment
of melanoma in situ, with 0.5-cm borders, has been performed with
Because of the advanced stage at presentation, prognosis for most
patients is poor, regardless of treatment. Survival for more than
2 years is extremely rare. In a series of 9 patients (2 with lymph
node metastases), 7 died of disease, most within 2 years. However,
2 were alive 2 and 14 years after treatment.
Although it once was a rare neoplasm of the lower extremities in
older Mediterranean men, Kaposi sarcoma (see Kaposi Sarcoma) has
increased in incidence 7000-fold in the HIV/AIDS era. Now, Kaposi
lesions often are a presenting symptom that heralds the onset of
AIDS in individuals infected with HIV.
While the original form rarely was seen in the genital area, currently
20% of men with HIV and Kaposi sarcoma have genital involvement,
and 3% of men positive for HIV present with Kaposi sarcoma of the
genitalia. Association of this vascular tumor with herpesvirus infection
has now been documented in HIV and non-HIV forms of disease.
Kaposi sarcoma tends to present on the genitalia as small violaceous-to-tan
macules or papules but may eventually coalesce to form plaques.
Occasionally, ulceration occurs, and complications can include lymphatic
or urethral obstruction.
In even more rare circumstances, other vascular tumors present on
the penis and scrotum. Rare cases of angiosarcoma have been reported,
but they are the exception when compared to Kaposi sarcoma of the
same area. Benign hemangiomas can occur but usually are identified
readily by the gross appearance of a smaller, unchanging macule
While no cure has been identified, care is centered on limiting
potential complications (obstruction) or cosmetic treatment. Intralesional
chemotherapeutics, radiation therapy, and local tissue destruction
by cryotherapy or laser therapy have been used with varying success.
While no cure currently is known, the use of multidrug regimens
to treat patients who are HIV-positive has led to increased patient
survival and an increasing desire for local and cosmetic control
of this tumor.