Male genital diseases
Lichen sclerosus of penis
Introduction
Lichen
sclerosus is a common idiopathic inflammatory scarring dermatosis with a
predilection for ano‐genital
skin. It
is principally a disease of uncircumcised men, although it can persist or recur
after circumcision. LSc can be the cause of significant morbidity expressed as
preputial dysfunction and male dyspareunia.
In male
patients, familial cases are uncommon, and HLA and autoimmune associations are
rarely observed.
Epidemiology
In adults, ano‐genital
lichen sclerosus is said to be about 10 times more common in women than men.
Perianal disease is very rare in the male.
The
age of presentation is bimodal, school‐age
boys and
men in the 4th or 5th decade. LSc may be much more frequent than is generally
supposed in young boys. In boys, persistent primary phimosis or the secondary
development of phimosis in a previously retractable foreskin should be viewed
with suspicion: some, many, or most of such cases will be due to LSc.
Pathophysiology
The predilection is for the male
genitalia in the uncircumcised. Male genital lichen sclerosus being due to
chronic occluded exposure of susceptible epithelium to urine due to post
micturition micro‐incontinence (‘dribbling’) seen in 90–100% patients and
meticulous physical examination often reveals distortion naviculo‐meatal anatomy causing dysfunction of the naviculomeatal
valve. Of note, lichen sclerosus of the penis does not occur in men
circumcised at birth.
Pathology
The
epidermis is atrophic with basal cell hydropic degeneration. The superficial
dermis is edematous and hyalinized. Deep to the hyalinized zone is a band‐like lymphohistiocytic infiltrate. Telangiectatic vessels
are common, as is purpura.
Clinical
features
Lichen sclerosus of
the penis may be asymptomatic, but patients may describe itching, burning,
bleeding, tearing, splitting, hemorrhagic blisters, dyspareunia, discomfort
with urination and narrowing of the urinary stream, and/or they may be
concerned about the changing anatomy of their genitalia.
In boys and men, acquired phimosis or recurrent balanitis are the
primary presenting features. In uncircumcised males, the glans and the
inner aspect of the foreskin may show smooth, atrophic, shining bluish white
sclerotic patches or plaques, with telangiectasia, purpura, bullae, erosions
and even ulceration may be encountered. The constriction may cause pain on
erection and, at advanced stages, dysuria and urinary obstruction due to meatal
‘pin hole’ narrowing as the disease encroaches into the urinary meatus. If the
foreskin is affected, lichen sclerosus invariably leads to phimosis (difficulty
in retracting the foreskin to uncover the glans), with the additional risk of
paraphimosis (when a foreskin retracted behind the glans cannot be returned to
its original position); the latter represents a
urologic emergency. A constrictive lichenoid posthitis is commonly seen
associated with a fibrotic preputial band causing “hourglass” “waisting” of the
penile shaft. Progressive disease can lead to effacement of the normally
sharply defined structures, especially of the frenulum and the coronal sulcus
and rim. While circumcision is clearly a first-line therapy, lichen sclerosus
may recur at the site of a circumcision.
Posthitis
xerotica obliterans refers to chronic damage to the prepuce by lichen
sclerosus, whereas balanitis xerotica obliterans properly describes involvement
of the glans penis. Balanitis xerotica obliterans can be a consequence of other
scarring dermatoses such as lichen planus and cicatricial pemphigoid.
Most
cases of LSc can be diagnosed clinically. If there is clinical doubt, then a
biopsy should be performed.
Symptoms and Signs of Male Genital Lichen Sclerosus
Asymptomatic Spontaneous · Itching,
burning, soreness, pain, adhesions, blisters, white patches, purpura, balanoposthitis,
phimosis Dyspareunia · Itching,
burning, soreness, pain, bleeding, tearing, splitting, Effacement
of normal architectural features · Phimosis,
narrow meatus, dissolution of frenulum, short frenulum, broad frenulum,
“bunched” frenulum, white papules and patches, loss of coronal definition,
adhesions, telangiectasia, scarring, fibrotic band (“waisting” or
“bunching”), constrictive posthitis · Posthitis
and balanitis xerotica Dysuria,
voiding difficulties, urinary retention Renal
failure Cancer |
Complications
SCC of the penis is the most worrying complication of
LSc: the risk is 2%–12.5% and the latent period may be 10–30 years. Involvement
of the glans with LSc is a significant risk.
Treatment
The
aims in male genital disease are to minimize or abolish male sexual dysfunction
(dyspareunia), urinary dysfunction, and the risk of penis cancer. Contact with soap, urine and pubic hair should be avoided
by use of a soap substitute and a barrier preparation. Very potent topical
steroids achieve remission in 50%–60% and the majority of the remainder is
cured by circumcision. An ultrapotent topical
corticosteroid (usually clobetasol propionate) used under supervision for a
finite course is effective, but herpes simplex and wart reactivation do occur.
Patients with a history of genital herpes simplex virus (HSV) should be
prescribed prophylactic aciclovir. There is significant remodelling of the
plasticity of the male genital epithelium with the relief of phimosis,
paraphimosis or constrictive posthitis and avoidance of circumcision. Secondary candidal and bacterial infection
should be treated. The use of topical calcineurin inhibitors is restricted
because of the risk of accelerated carcinogenesis. If medical treatment with
ultrapotent topical steroid is not possible or fails, then surgery is
indicated. Circumcision, frenuloplasty, meatotomy and sophisticated plastic
repair, depending upon the clinical presentation, can be offered. Carbon dioxide laser circumcision has been
described. In both boys and adult male patients, complete circumcision is the
treatment of choice because all affected tissue is removed and any secondary
involvement of the glans probably regresses or resolves. Surgery works in male
genital lichen sclerosus if it relieves susceptible epithelium from chronic
occluded exposure to urine. Surgery fails if it does not achieve this
goal. About 40% of patients will respond
to medical treatment: the majority of the remainder will be cured by surgery,
usually circumcision.
Squmous
cell carcinoma is the most serious potential complication of LS. Involvement of
the glans penis confers a greater risk. Patients should be followed‐up long term, especially if circumcision has not been
performed or if symptoms persist or recur after any form of treatment.
Lichen planus of penis
Introduction
It
is a common idiopathic inflammatory dermatosis with a particular predilection
for the oro‐genital epithelium. Approximately 25% of men with
cutaneous lichen planus have genital involvement.
Clinical
features
LP
has a particular predilection for the mucosa, which is perhaps partly explained
by the Koebner phenomenon. Lichen planus
can present in, and remain localized to, the ano‐genital
area, including the groins and perianal skin. Like the classical disease at
other sites, it presents as itchy red‐purple
papules, plaques and annular lesions with Wickham striae on the glans and shaft
of the
penis. It may also present as phimosis.
The male genitalia represent the commonest site for the annular subtype of
lichen planus. Occasionally, an erosive form is encountered.
Although
LP is self-limiting, some patients experience relapses and remissions.
Adhesions can form. Post‐inflammatory
hyperpigmentation can persist for months or years. Chronic mucosal erosive
lichen planus is associated with a risk of progression to squamous carcinoma.
Investigations
A
biopsy is frequently necessary for diagnostic purposes.
Treatment
Potent and ultrapotent topical
corticosteroids usually suffice and the treatment should be continued until the
lesions are non‐itchy and flat; they are warned about post‐inflammatory hyperpigmentation. Reactivation of genital
warts may occur. Topical calcineurin inhibitors should be used with caution. Circumcision may be
necessary if there is phimosis and should be seriously considered in cases of
refractory disease, especially the erosive form, because the removal of
“koebnerizing” influences may allow the LP to remit.
Psoriasis of penis
Introduction
Psoriasis is probably the most common dermatosis of the
male anogenitalia, either in isolation or in association with frank or mild,
subtle extragenital disease. The Koebner phenomenon is a likely factor in site
predilection. Drugs such as lithium, β
blockers, antimalarials, and angiotensin-converting enzyme inhibitors may be
responsible for the onset or exacerbation of psoriasis. The penile lesions have the same
histopathology as psoriasis.
Epidemiology
Approximately
2% of the population are said to have psoriasis but it is possible that many
more than 2% of men may develop ano‐genital
psoriasis at some time.
Clinical
features
Psoriasis
of the anogenital region may look quite different from psoriasis at other
sites. It is not usually itchy; significant itch should arouse suspicions of
another dermatosis such as an eczema or tinea. Soreness occurs with super
infection, especially with Candida. Other typically affected sites
should be examined for signs of the disease.
The
diagnosis is usually easier in the circumcised male where the morphology is
similar to extragenital lesions. In circumcised men, genital psoriasis presents
with silvery-scaled, erythematous well-defined patches or plaques on the glans
and also seen on the shaft of the penis. Persistent, painful fissuring of the
perianal area and the intergluteal cleft can be a severe problem.
The diagnosis is difficult
especially in the uncircumcised patient, because a mucosal site is affected
rather than keratinized skin. On the glans or in the preputial sac of the uncircumcised
patient, the typical silvery scale is absent from the patches or plaques, where its occurrence is probably brought about by the
Koebner phenomenon.
Psoriatic balanoposthitis can be part of the spectrum of
inverse-pattern psoriasis and may be associated with intertriginous disease of
the axillae, intergluteal cleft, gluteal folds, and groin.
Investigations
Usually,
the diagnosis of psoriasis is clinical, but a biopsy may be necessary (e.g. of
a solitary mucosal lesion in an uncircumcised individual) to distinguish
psoriasis from Zoon balanitis, lichen planus, carcinoma in situ (Bowen
disease, erythroplasia of Queyrat) or Kaposi sarcoma.
Treatment
Topical treatment includes
emollients, soap substitutes, corticosteroids combined with antibiotic and
antifungal agents. Atrophy
is a risk with long-term use of potent topical steroids, and anogenital skin
has a heightened tendency to absorb topical agents. The vitamin D analogue calcipotriol can be helpful.
Topical ciclosporin (100 mg/ml in wet dressings three times daily) has
been advocated. Topical calcineurin inhibitors may be helpful and appear to be
well tolerated but should be used with caution in the uncircumcised because of
the squamous cancer risk. Severe ano‐genital
psoriasis can be an indication for systemic treatment.
Balanitis, plasma cell (Zoon)
Overview
· Zoon
plasma cell balanitis (ZB; properly balanoposthitis) affects the middle-aged
and older uncircumcised male.
· ZB
is a chronic, reactive, irritant mucositis.
· ZB
is characterized by silent symptomatology and florid signs.
· Zoonoid
inflammation is a common corollary of other dermatoses that cause a
dysfunctional prepuce. Asymmetric, atypical, or unusual morphology should be
viewed with suspicion.
·
Circumcision
is the definitive treatment in the majority of cases.
Introduction
It is
an asymptomatic, inflammatory and irritant condition of the glans and mucosal
prepuce.
Epidemiology
Zoon
plasma cell balanitis is
usually seen from the third decade onwards in uncircumcised men.
Pathophysiology
ZB is a chronic,
reactive, principally irritant mucositis that causes, or is due to, a
dysfunctional foreskin. Irritation is induced by retention of urine, squames,
commensally hypercolonized and increased secretion between two tightly apposed
and infrequently and inadequately separated and/or infrequently or
inappropriately bathed, epithelial surfaces due to poor penile hygiene that leads to a disturbed ‘preputial ecology’ and excessive
frictional trauma (Zoon balanitis is often located on the dorsal aspect of the
glans and the adjacent prepuce, sites of maximal friction on foreskin
retraction). The
condition does not occur in circumcised men.
Pathology
The classic
histology is of epidermal attenuation with absent granular and horny layers, sparse
dyskeratosis and spongiosis, and diamond- or lozenge-shaped basal cell
keratinocytes. In the dermis, there is a band of plasma cells infiltration in
the upper and mid dermis, extravasated erythrocytes, hemosiderin, fibrosis, and
vascular proliferation.
Clinical
features
The presentation is classically asymptomatic, or gives rise to
pruritus or dysuria and staining of the underclothes
with blood has been reported. Well‐demarcated,
glistening, moist, shiny, bright red discrete patches with a dark red stippling or
“cayenne pepper” spots and an orange hue due to hemosiderin deposition symmetrically
involving the glans and inner prepuce, sparing the keratinized penile shaft or
foreskin are seen. The
navicular fossa may be involved. “Kissing” lesions are typical with
involvement of adjacent, touching areas, e.g. around the urethral meatus.
Shallow erosions may resolve slowly, leaving a rusty stain.
Investigations
The
diagnosis is usually clinical.
Treatment
Although Zoon balanitis can improve
with altered washing habits and micturition practices and
the intermittent application of a mild or potent topical corticosteroid (with
or without an antibiotic and anticandidal agent) and topical tacrolimus, it
usually persists or relapses. The use of topical calcineurin inhibitors should
be restricted as there is risk of penis cancer in the long term. Definitive curative
treatment is surgical circumcision.