Diagnosis: Penile lichen sclerosus (balanitis xerotica obliterans)
When lichen sclerosus (LS), a chronic, progressive, sclerosing inflammatory dermatosis, involves the penis, it is termed balanitis xerotica obliterans (BXO). BXO manifests as ivory, white, or purple-white patches or plaques on the glans and prepuce of the penis and sometimes on the frenulum, urethral meatus, fossa navicularis, penile shaft, and anus. Lesion consistency can range from sclerotic to atrophic. Surface texture can range from keloidlike to cigarette-paperlike. Scar tissue is hard and can prevent retraction of the foreskin.
BXO affects 0.6% of males of all ages. Its cause is unknown. Chronic irritation likely plays a role, but whether irritation augments or initiates BXO is unclear, as uncircumcised men are disproportionately and overwhelmingly affected. Papillomavirus has not been isolated from BXO lesions. Patients with BXO more commonly suffer from autoimmune diseases than do controls. Vitiligo, thyroid disease, diabetes, and alopecia areata occur more often in patients with BXO. A few articles hypothesizing spirochete infection abated by antibiotics have not been widely accepted.
Early BXO has few symptoms. The sclerosis and cutaneous hypopigmentation of the penis manifest over years—earlier in uncircumcised men than in the rare circumcised patient. Symptoms reported with BXO progression include pruritus, burning, hypoesthesia of the glans, dysuria, pain with erection and sexual intercourse, decrease in urinary force and caliber of the urinary stream, urethritis, and discharge.
Symptoms of advanced BXO include phimosis (inability to retract the foreskin over the glans) and paraphimosis (inability to return an already retracted foreskin back over the glans). In clinically advanced BXO, the glans may become adherent to the prepuce, and the coronal sulcus and frenulum may be effaced due to sclerotic tissue remodeling. BXO patients may also experience destruction of the distal half-centimeter of the urethra and urethral-meatal narrowing so severe that it causes urinary retention. In turn, urinary retention can result in retrograde damage to the posterior urethra, bladder, and kidneys. Long-standing BXO that manifests only scar tissue without active inflammation is termed “burned out.”
In addition to compromised ability to urinate, sexual function may be lost in late-stage BXO because of pain from phimosis and meatal stenosis. Moreover, long-standing BXO can evolve into squamous cell carcinoma (SCC).
The differential diagnosis of BXO includes a number of conditions. Balanitis circumscripta plasmacellularis (Zoon’s plasma cell balanitis) has a glistening appearance, sometimes with an orange tinge. In penile candidiasis, the characteristic white material can often be scraped off. Erythroplasia of Queyrat is marked by erosions and erythema, while penile SCC can be ulcerated and erythematous. The eruption of lichen planus is usually purple and sometimes annular. Psoriasis and Reiter syndrome (which some think is a variant of psoriasis) can manifest with moist, white, somewhat eroded plaques in areas of tissue occlusion, such as occurs on the penises of uncircumcised men. Vitiligo does not demonstrate dermal changes and lacks sclerosis. Infectious balanitis will be ruled in or out by tissue culture.
Circumcision is the most definitive cure for BXO. I would argue that the treatment of choice in a first-time BXO patient is a topical calcineurin inhibitor, as these agents do not induce cutaneous atrophy and they achieve good anti-inflammatory effect on thin skin. (The black box warning that they are related to an increased risk of skin cancer, while not evidence-based, must be mentioned to patients.) Topical and intralesional corticosteroids should be considered second-line agents because they can cause cutaneous atrophy. Topical testosterone has been used as an adjunct to topical anti-inflammatory agents. Acitretin (Soriatane) can be considered if topical treatments have failed.
BXO-induced scar tissue is removable with the carbon dioxide laser. Surgical procedures that treat late-stage BXO include dorsal onlay buccal mucosal urethroplasty and full-thickness skin grafts from the eyelids. Split-thickness grafts can be used to replace scarred tissue. Urethral replacement with buccal mucosa is an effective approach when the urethra is effaced. Burned-out BXO must be treated surgically.
Education is crucial to successful treatment of this condition. Patients can be taught to dilate a narrowed urethral meatus to facilitate urination.
In our patient, twice-daily applications of tacrolimus 0.1% provided some abatement of symptoms. Later, therapeutic circumcision led to complete resolution of his BXO.
Dr. Scheinfeld is assistant clinical professor of dermatology at Columbia University in New York City, where he has a private practice.