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Balanitis Xerotica Obliterans Last Updated: February 9, 2005
|Synonyms and related keywords: BXO, penile lichen sclerosus, male genital lichen sclerosus, lichen sclerosus et atrophicus, LS, penile LS, sclerosing inflammatory dermatosis, Koebner phenomenon, vitiligo, thyroid disease, diabetes, alopecia areata, pseudoepitheliomatous keratotic and micaceous balanitis, PKMB
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| Author: George C Keough, MD, Chief, Clinical Assistant Professor, Department of Medicine, Dermatology Service, Eisenhower Army Medical Center
| George C Keough, MD, is a member of the following medical societies:
American Academy of Dermatology, and
American Medical Association
| Editor(s): Mark W Cobb, MD, Consulting Staff, WNC Dermatological Associates; Richard Vinson, MD, Chief, Department of Dermatology, William Beaumont Medical Center;
Jeffrey Meffert, MD, Program Director, Dermatology Service, San Antonio Uniformed Services Health Education Consortium;
Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University;
and William D James, MD, Program Director, Vice-Chair, Albert M Kligman Professor, Department of Dermatology, University of Pennsylvania School of Medicine
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Background: Lichen sclerosus (LS) is a chronic, progressive, sclerosing inflammatory dermatosis of unclear etiology. Most reported LS cases (83%) involve the genitalia. In men, this genital involvement has traditionally been known as balanitis xerotica obliterans (BXO). A more accurate term is male genital or penile LS.
Pathophysiology: The etiology of male genital LS is unknown but is thought to be multifactorial.
- In the US: Frequency is similar to that of the international community.
- Internationally: The prevalence of male genital LS has traditionally been estimated at 1 case per 300-1000 males. No recent studies confirm this estimate, but male genital LS is not considered a rare condition.
Race: Male genital LS has no known predilection for any racial or ethnic group.
Sex: Male genital LS occurs most frequently in those who are uncircumcised and middle-aged. One study revealed that 51 (98%) of 52 patients diagnosed with penile LS were uncircumcised (Mallon, 2000).
Age: Although, males with genital LS are most frequently middle-aged, the condition may appear in children as well, from young boys to adolescents.
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History: Early in its course, penile LS is relatively asymptomatic with only mild visually observable changes of the penis and glans. Physical changes occur over months or years and may include color or textural changes. Early symptoms are more prevalent in uncircumcised patients.
- Symptoms occurring with time and progression of penile LS
- Hypoesthesia of the glans penis
- Painful erection with altered sexual function
- Decrease in urinary force or stream caliber
- Urethritis with or without discharge
- Symptoms occurring in late penile LS (in uncircumcised patients)
- Phimosis (inability to retract the foreskin over the glans)
- Paraphimosis (inability to return an already retracted foreskin back over the glans)
Physical: Early penile LS demonstrates only subtle physical findings (eg, mild, nonspecific erythema; mild hypopigmentation).
- As the condition progresses, single or multiple discrete erythematous papules or macules progress and coalesce into atrophic ivory, white, or purple-white patches or plaques.
- Lesions most commonly affect the glans and prepuce.
- The frenulum, urethral meatus, fossa navicularis, penile shaft, and perianal areas may become involved.
- A sclerotic white ring at the tip of the prepuce is diagnostic at this stage.
- Erosions, fissures, petechiae, serous and hemorrhagic bullae, and telangiectasias of the glans have been reported, albeit uncommonly.
- With further disease progression, the glans may become adherent to the prepuce.
- The coronal sulcus and frenulum may be sclerotically destroyed.
- The urethral meatus may narrow to the point of urinary retention.
- Urinary retention may be severe enough to cause retrograde damage to the posterior urethra and to the bladder and kidneys.
- Significant urethral meatal narrowing has led to sloughing of the distal half centimeter of the urethra.
- Phimosis and paraphimosis of uncircumcised patients may occur at this late stage.
- Seventeen percent of LS cases are extragenital, beginning as mild, nonspecific erythema with mild hypopigmentation.
Causes: The etiology of male genital LS is unknown but is thought to be multifactorial. Several contributing factors are possible, as follows:
- Circumcision after age 13 years/uncircumcised state
- This may very well be due to the effect known as the isomorphic, or Koebner, phenomenon. The large majority of inflammatory dermatoses of the male genitalia, including LS, occur in uncircumcised or late-circumcised men.
- The presence of a foreskin may promote chronic irritation or serve to maintain a friendly environment for an as-yet unidentified infectious agent. Such chronic irritation and subsequent inflammation may initiate the changes noted in LS.
- Hormonal factors
- Hormonal influences in the development of LS have long been postulated, mainly in female vulvar LS.
- Most studies have concentrated on the role of testosterone in the pathogenesis of vulvar LS. Childhood vulvar LS frequently resolves with the onset of menarche and the related pubertal increase in testosterone production in genital skin; additionally, adults with LS have been found to have decreased serum levels of free testosterone, androstenedione, and dihydrotestosterone compared with control subjects.
- The underlying defect may be a problem with the function of the enzyme 5-alpha reductase.
- Autoimmune disease
- Various autoantibodies (including antinuclear, thyroid antimicrosomal, antigastric parietal cell, anti-adrenal cortex, antismooth muscle, and antimitochondrial antibodies) have been detected in patients with LS.
- Vitiligo, thyroid disease, diabetes, and alopecia areata have also been commonly reported in association with LS.
- Genetic factors
- LS (not necessarily genital LS) has been reported in families, including twins (identical and nonidentical), sisters, mothers and daughters, and a brother and sister.
- Note, however, that no consistent pattern of genetic inheritance has been identified.
- Presence of human papillomaviruses
- The presence of human papillomaviruses (HPV) has been reported in some cases of childhood penile LS. Whether the LS is directly attributable to HPV infection, or if LS merely promotes HPV infection is unclear.
- Patients with penile LS alone have not been demonstrated to have a higher incidence of HPV infection.
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Balanitis Circumscripta Plasmacellularis
Erythroplasia of Queyrat (Bowen Disease of the Glans Penis)
Other Problems to be Considered:
Pseudoepitheliomatous keratotic and micaceous balanitis
Pseudoepitheliomatous keratotic and micaceous balanitis (PKMB) is a very rare papulosquamous dermatosis of the glans penis. PKMB presents in elderly, uncircumcised men as a slowly growing, coarsely scaling, micaceous, white-to-gold, laminated, well-demarcated plaque. The lesion may grow to involve the coronal sulcus and the distal penile shaft. Symptoms include phimosis, pain, and interference with sexual activity. PKMB is considered to be a premalignant condition. Nearly all reported patients have had malignant degeneration. Reported associated malignancies include squamous cell carcinoma (SCC), verrucous carcinoma, and fibrosarcoma.
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Histologic Findings: Histopathologic changes of genital LS are similar to those of nongenital LS. Epidermal findings include orthokeratotic hyperkeratosis with follicular plugging, atrophy of the stratum malpighii, hydropic degeneration of the basal layer, and occasional dermal-epidermal clefting. Follicular plugging is not apparent in mucosal lesions. The dermis reveals significant edema and homogenization of the collagen in the upper dermis with dilatation of blood and lymph vessels and a loss of elastic fibers. An inflammatory infiltrate consisting of lymphoid cells, plasma cells, and histiocytes occurs in the mid dermis. The inflammatory infiltrate is less pronounced in long-standing lesions.
- Skin biopsy aids in diagnosis of male genital LS.
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- Traditionally, circumcision is the treatment method of choice in uncircumcised patients.
- Further treatment, or treatment of circumcised patients, is more challenging. No consistently effective treatment has been developed; however, the following therapies have varying degrees of reported success:
- Topical and intralesional steroids
- Topical testosterone propionate ointment
- Etretinate (no longer available): Acitretin is the current equivalent.
- Carbon dioxide laser treatment
- Uncircumcised patients usually benefit from therapeutic circumcision. Provide regular follow-up care to observe any changes in involved areas suggestive of malignancy.
- Consider surgical intervention for symptoms or signs of urethral meatal stenosis.
- Consider consultation with urologists for the following:
- Circumcision for symptomatic phimosis or paraphimosis
- Significant narrowing or obstruction of the urethral meatus or changes in urinary flow
Activity: In some cases of male genital LS, painful erections may limit sexual function.
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Topical steroids, especially superpotent topical steroids, are the mainstay of medical therapy. Topical testosterone is mostly ineffective and is not discussed further. Etretinate has been used with limited success but is no longer available for prescription in the United States.
Drug Category: Topical corticosteroids -- Help reduce inflammatory lesions and may reduce or resolve lesions.
|Drug Name||Clobetasol propionate (Temovate) -- Class I superpotent topical steroid; suppresses mitosis and increases synthesis of proteins that decrease inflammation and cause vasoconstriction. Used in most studies dealing with treatment of LS.|
|Adult Dose||Apply to affected areas qd for up to 12 wk, although continuous treatment for >2 wk may begin to cause atrophic changes; atrophic changes will be more pronounced in genital area than other areas of the body; not to exceed 50 g/wk|
|Pediatric Dose||Not established|
|Contraindications||Documented hypersensitivity; viral or fungal skin infections|
C - Safety for use during pregnancy has not been established.
|Precautions||Steroid atrophy commonly occurs when superpotent topical steroids are used in genital area for even short periods; observe patients for any such changes; may suppress adrenal function in prolonged therapy|
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Further Outpatient Care:
- Provide regular follow-up care to observe any changes in involved areas suggestive of malignancy.
- Consider surgical intervention for symptoms or signs of urethral meatal stenosis.
- Early circumcision may decrease the risk of developing male genital LS; nearly all cases have been reported in uncircumcised patients.
- As the disease progresses, urinary retention may be sufficient to lead to retrograde damage to the posterior urethra, bladder, and kidneys.
- As previously noted, painful erections in some cases of male genital LS may limit sexual function.
- Malignancies have been reported to occur in penile lesions (rare). Common signs and symptoms of penile malignancy include nodule or tumor growth, ulceration, blistering, hematuria, erythema, pain, purulent discharge, bleeding, lymphadenopathy, and failure to respond to treatment for presumptive inflammatory or infectious balanitis. For this reason, close follow-up care is indicated in order to quickly diagnose any malignant changes.
- Male genital LS is chronic and often progressive. Regression or improvement of atrophic areas is unexpected.
- Malignancies have been reported to arise in penile LS lesions (rare); most common cancers are SCC, adenosquamous carcinoma, and verrucous carcinoma.
- A recent study of 86 uncircumcised men with genital LS revealed malignant changes (3 SCC, 1 SCC in situ, and 1 verrucous carcinoma) occurring in 5 (5.8%) subjects. The average time between diagnosis of LS and subsequent diagnosis of penile malignancy was 17 years (Nasca, 1999).
- Notably, 4 of the 5 patients with malignant changes were found by polymerase chain reaction to have evidence of HPV-16 in their tissue specimens. It has been suggested that LS may promote HPV infection and perhaps the development of SCC.
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- Failure to diagnose male genital LS early is a potential pitfall.
- Delay in diagnosis or follow-up of patients with urinary symptoms may lead to irreversible damage to the urinary system.
- No singularly effective therapy exists; however, treatment with surgical and/or medical techniques should be quickly instituted upon diagnosis of this often progressive condition.
- Failure to provide regular follow-up care is a potential pitfall. Care should include biopsy of ulcerating or nonhealing areas to detect malignancies (rare) developing from lesions of male genital LS.
- Men with genital LS may delay presenting to a physician because of fear or embarrassment. Accurate diagnosis, aided with appropriate biopsy, helps calm anxiety.
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