Balanitis Xerotica Obliterans

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Background

Lichen sclerosus is a chronic, progressive, sclerosing inflammatory dermatosis of unclear etiology. Most reported lichen sclerosus 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 lichen sclerosus. The image below shows the condition.


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Balanitis xerotica obliterans (lichen sclerosus). Courtesy of Wilford Hall Medical Center Slide collection.

Yardley et al[1] believe that the prevalence of BXO is greater than previous series have shown and that it may manifest in children at an earlier age than previous series have shown. This belief is based on a study of 422 boys at a median age of 6 years 2 months (range, 3 mo to 16 y), of whom 186 (44.1%) received treatment involving surgery (148 circumcision, 33 preputial adhesiolysis, 5 frenuloplasty). Of the 186 boys, 110 had histological tissue examination; 84.8% of skin samples were pathologic. Specifically, tissue showed chronic inflammation (n = 69; 46.6%), BXO (n = 51; 34.5%), and fibrosis (n = 4; 2.7%).

Related Medscape Reference articles include Lichen Sclerosus et Atrophicus, Balanitis Circumscripta Plasmacellularis, and Balanitis in Emergency Medicine.

Pathophysiology

The etiology of male genital lichen sclerosus is unknown, but it is thought to be multifactorial. Balanitis xerotica obliterans (BXO) has occurred in monozygotic twins, which suggests a genetic basis for the disease in some cases. Human papillomavirus type 6 or type 16 has not been detected in patients with BXO, which strongly suggests that genital papillomaviruses do not have a strong association with BXO.

Epidemiology

Frequency

United States

Kizer et al[2] noted that of 153,432 male patients discharged from Brooke Army Medical Center, 108 (0.070%) had a diagnosis of balanitis xerotica obliterans (BXO). The age distribution was similar over a range of 2-90 years, with the exception of the third decade, when the incidence almost doubled. Black and Hispanic patients had twice the rate found in white patients (10.59 cases, 10.67 cases, and 5.07 cases per 10,000 patients, respectively).

International

The prevalence of male genital lichen sclerosus (balanitis xerotica obliterans [BXO]) has traditionally been estimated at 1 case per 300-1000 males. No recent studies confirm this estimate, but male genital lichen sclerosus is not considered a rare condition. Huntley et al[3] reported on 100 consecutive patients seen in pediatric urology clinics who were followed to discharge. Eighteen referrals for circumcision were for religious reasons. Of the other 82 patients, the main reason for referral was retractability or phimosis. Six patients were identified as having BXO, a condition that had not been suggested on referral. Epidemiological data continue to show that BXO can effect boys.[4, 5] Some in Italy claim that the incidence of BXO has been understated.[6]

In Austria, 75 boys younger than 10 years were treated for phimosis; phimosis grade 2 or 3 (schema by Kikiros) was suspected of being BXO. Boys were given either circumcision or conservative therapy with circumcision secondarily (only if therapy did not yield good results in the conservative group). A pathologist examined every circumcision specimen. Doctor performed circumcision primarily in 29 boys and secondarily in 17 boys (mean age, 3.7 y; range, 1-10 y). The pathologist found BXO, chronic inflammation, and normal histological results in 8 (17.4%), 26 (56.5%), and 12 (26.1%) of patients, respectively. The average follow up was approximately 8 months. Doctors did not report recurrences. BXO appeared to be more common than previously reported. The clinical appearance can be confusing in boys, and preoperative BXO suspicion failed to correlate with the final biopsy results.[7]

Race

Male genital lichen sclerosus (balanitis xerotica obliterans [BXO]) has no known predilection for any racial or ethnic group.

Sex

Male genital lichen sclerosus (balanitis xerotica obliterans [BXO]) occurs most frequently in persons who are uncircumcised and who are of middle age. One study[8] revealed that 51 (98%) of 52 patients clinically diagnosed with penile lichen sclerosus were uncircumcised.

Age

Although males with genital lichen sclerosus (balanitis xerotica obliterans [BXO]) are most frequently of middle age, the condition also may appear in children, ranging from young boys to adolescents. The incidence of BXO in pediatric patients is higher than most physicians realize. Additionally, the incidence of BXO is high in boys with phimosis.[9, 10, 11]

History

Early in its course, penile lichen sclerosus (balanitis xerotica obliterans [BXO]) 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 lichen sclerosus are as follows:

Symptoms occurring in late penile lichen sclerosus (in uncircumcised patients) are as follows:

The development of multifocal squamous cell carcinoma (SCC) in persons with lichen sclerosus et atrophicus of the penis and hepatitis C virus infection has been reported. SCC of the penis arising from BXO alone has also been noted.

A urethral stone manifesting as a stop valve, a rare complication of BXO, has been reported.

In older patients, BXO with phimosis can be a cause of difficulty with urination; thus, older patients should be examined to see if they have BXO in they have symptoms of difficulty with urination.[12]

Physical

Early penile lichen sclerosus (balanitis xerotica obliterans [BXO]) demonstrates only subtle physical findings (eg, mild, nonspecific erythema; mild hypopigmentation).

Causes

The etiology of male genital lichen sclerosus (balanitis xerotica obliterans [BXO]) is unknown but is thought to be multifactorial. Several contributing factors are possible, as follows:

Laboratory Studies

Procedures

Histologic Findings

Histopathologic changes of genital lichen sclerosus are similar to those of nongenital lichen sclerosus.

Epidermal findings include orthokeratosis, hyperkeratosis with follicular plugging, hyperkeratosis without follicular plugging, stratum malpighii atrophy, basal layer hydropic degeneration, and dermoepidermal clefting (in some cases).

Follicular plugging is not apparent in mucosal BXO. Significant dermal edema and homogenization of the collagen in the upper dermis occurs, with dilatation of blood and lymph vessels and a loss of elastic fibers.

The immune cells moving into areas of BXO include lymphocytes, plasma cells, and histiocytes in the mid dermis. The inflammatory infiltrate is less pronounced in long-standing lesions.

Medical Care

Surgical Care

A variety of surgical techniques can be used to treat more severe penile lichen sclerosus (balanitis xerotica obliterans [BXO]).

Uncircumcised patients usually benefit from therapeutic circumcision. Provide regular follow-up care to observe any changes in involved areas suggestive of malignancy.

Foreskin preputioplasty combined with intralesional triamcinolone might be a tenable alternative as against circumcision to treat BXO.[20]

Consider surgical intervention for symptoms or signs of urethral meatal stenosis.

Dubey et al[21] report that in BXO-related strictures with a viable urethral plate, 1-stage dorsal onlay buccal mucosal urethroplasty achieves superb medium-term results. They also state that the intervention created a normal, wide-caliber, slitlike glans, and a 2-stage procedure provides effective treatment but is associated with a higher revision rate.

Full-thickness skin grafts from eyelids to penis, plus split-thickness grafts in chronic BXO have been reported.

Buccal mucosa appears to be a durable source of nongenital tissue for urethral replacement. Attention to detail in terms of graft harvest, graft preparation, and graft fixation helps to avoid major postoperative complications. Onlay grafts appear to be preferable to tube grafts, and patients with a diagnosis of BXO do not appear to be candidates for the 1-stage urethral reconstruction using buccal mucosa.

Circumferential laser vaporization for severe meatal stenosis secondary to BXO reportedly is effective.

In 2007, Levine et al[22] reported on buccal mucosa graft urethroplasty for anterior urethral stricture repair. They evaluated the impact of stricture location and lichen sclerosus on surgical outcome. When lichen sclerosus affects the penis, complete excision of the diseased urethra with multistage repair decreases the rate of stricture recurrence associated with a 1-stage repair.

Palminteri et al[23] treated 17 patients, performing y resurfacing or reconstruction of the glans penis for benign, premalignant, and malignant penile lesions (5 glans skinning and resurfacing; 5 glans amputation and reconstruction of the neoglans, and 7 partial penile amputation and reconstruction of the neoglans). Four patients had lichen sclerosus. Glans resurfacing and reconstruction were performed with the use of a skin graft harvested from the thigh. Patients who received glans resurfacing reported glandular sensory restoration and complete sexual ability. Patients receiving glansectomy or partial penectomy with neoglans reconstruction maintained sexual function and activity, albeit with reduced sensitivity secondary to glans/penile amputation. Palminteri et al concluded that glans resurfacing or reconstruction can ensure a normal-appearing and functional penis, without jeopardizing cancer control.

A review published in 2013 found that BXO likely is more common than believed, and, while circumcision is its primary treatment, topical or intralesional treatments can be co-adjuvants of treatment.[24]

Consultations

Activity

In some cases of male genital lichen sclerosus, painful erections may limit sexual function.

Medication Summary

Topical steroids, especially superpotent topical steroids, are the mainstay of medical therapy. Zavras et al reported successful treatment of 1079 (91.1%) of 1185 boys with a diagnosis of phimosis using fluticasone propionate 0.05%, including boys with mild balanitis xerotica obliterans (BXO).[25]

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.

Clobetasol (Temovate)

Clinical Context:  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 lichen sclerosus.

Class Summary

Help reduce inflammatory lesions and may reduce or resolve lesions.

Tacrolimus ointment 0.1% or 0.03% (Protopic)

Clinical Context:  Mechanism of action in atopic dermatitis is not known. Reduces itching and inflammation by suppressing release of cytokines from T cells. Also inhibits transcription for genes that encode IL-3, IL-4, IL-5, GM-CSF, and TNF-alpha, all of which are involved in early stages of T-cell activation. Additionally, may inhibit release of preformed mediators from skin mast cells and basophils and may down-regulate expression of FCeRI on Langerhans cells. Can be used in patients ≥ 2 y. More expensive than topical corticosteroids. Available as ointment in concentrations of 0.03 and 0.1%. Indicated only after other treatment options have failed.

Class Summary

Topical calcineurin inhibitors are immune suppressants that block early T-cell activation, degranulation of mast cells, and multiple cytokines.

Further Outpatient Care

Deterrence/Prevention

Complications

Prognosis

Author

Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Weil Cornell Medical College; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Assistant Attending Dermatologist, New York Presbyterian Hospital; Assistant Attending Dermatologist, Lenox Hill Hospital, North Shore-LIJ Health System; Private Practice

Disclosure: Optigenex Salary Employment

Coauthor(s)

Daniel S Lehman, MD, Fellow in Minimally Invasive Urology/Oncology, Department of Urology, Columbia University Medical Center

Disclosure: Nothing to disclose.

George C Keough, MD, Chief, Clinical Assistant Professor, Department of Medicine, Dermatology Service, Eisenhower Army Medical Center

Disclosure: Nothing to disclose.

Specialty Editors

Mark W Cobb, MD, Consulting Staff, WNC Dermatological Associates

Disclosure: Nothing to disclose.

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD, Assistant Clinical Professor of Dermatology, University of Texas School of Medicine at San Antonio

Disclosure: Nothing to disclose.

Catherine M Quirk, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD, Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

Disclosure: Nothing to disclose.

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Balanitis xerotica obliterans (lichen sclerosus). Courtesy of Wilford Hall Medical Center Slide collection.

Balanitis xerotica obliterans (lichen sclerosus). Courtesy of Wilford Hall Medical Center Slide collection.