Balanitis xerotica obliterans: A form of lichen sclerosus. (Editorial).

Lichen sclerosus is a chronic dermatosis characterized by atrophic white papules or plaques of skin and mucosa. This dermatosis can affect any part of the body; it occurs most commonly on the anogenital skin of postmenopausal women, though it is seen in all age groups and both sexes. It is rare in males, with the glans and prepuce often the only sites involved. Lichen sclerosus is also known as vulvar dystrophy, lichen sclerosus et atrophicus, kraurosis vulvae, hypoplastic dystrophy, and balanitis xerotica obliterans, depending on its anatomic location.

The etiology of lichen sclerosus is unknown. The likely but unproved etiology is an autoimmune mechanism, with about 20% of the general population having at least one autoimmune disease, and a higher percentage having circulating autoantibodies. In Europe and Japan, infection with Borrelia burgdorferi (B. burgdorferi) has been linked to lichen sclerosus based upon finding spirochetes, B. burgdorferi antigens, and B. burgdorferi DNA in the lesions; however, such an etiology is not universally accepted. Other theories regarding etiology include abnormalities in keratin synthesis, defects in androgen metabolism, abnormal keratins, decreased collagenase activity, and increased elastase activity. Trauma can induce lesions and may explain the improvement in the condition in boys after circumcision and its frequent appearance after surgical removal of the lichen sclerosus. Finally, there is a known, but small, hereditary predisposition for the disease.

Lichen sclerosus begins asymptomatically in most patients. It can present at any time from childhood to old age, and occurs in all races. Both sexes are affected, both before and after puberty, with females predominating at all ages by a 10:1 ratio.

Early lichen sclerosus shows clinically nonspecific findings of erythema, with or without hypopigmentation. In time, if untreated, typical ivory or porcelain white macules and plaques develop. The surface of the lesion is usually smooth, but may be hyperkeratotic. Lesions may become eroded or ulcerated. Itching may be severe, especially in the anogenital area. Extragenital lesions are most frequent on the upper back, chest, and breasts, and are usually asymptomatic. The tongue and oral mucosa may also be involved, either alone or without lesions elsewhere. Lichen sclerosus has a predilection for the anogenital area, however.

Anogenital lesions in females are initially pruritic, but as the condition becomes chronic, symptoms due to erosion supervene. The initial plaques may become sclerotic, bulbous, ulcerated, or hemorrhagic, and the epidermis may be thin and shiny, or thick and white. In end-stage vulvar lichen sclerosus, there is total loss of identifiable structures, and the introitus can be reduced to a slit-like opening with fusion of the labia. The perianal area is frequently involved, giving rise to constipation. Resorption of the labia minora, scarring of the clitoral hood burying the clitoris, and obliteration of the urethral meatus may occur with longstanding disease. Lichen sclerosus never affects the vagina.

Balanitis xerotica obliterans is the penile counterpart of lichen sclerosus (lichen sclerosus et atrophia). It may occur in patients of any age. It is most common in middle-aged men, but has been reported in boys. In one study of prepubertal boys requiring circumcision for phimosis, 14% were found to have lichen sclerosus. While it may occur in circumcised or uncircumcised individuals, it is more frequently associated with chronic balanitis or phimosis in uncircumcised males. It most commonly occurs on the glans and inner surface of the prepuce; involvement of the shaft of the penis is uncommon. Its onset and evolution are usually insidious, but may progress over many years. The condition originates as small, erythematous areas that coalesce, forming white plaques with well-defined margins. The surface is usually smooth and shiny, but may be wrinkled. A white patch on the prepuce or glans may extend to and around the urethral meatus, frenulum, and fossa navicularis. The meatus may appear white, indurated, and /or edematous. Glandular erosion, ulceration, fissures, and mental stenosis may occur. Lesions may be asymptomatic, or produce varying degrees of local pain, discharge, pruritus, painful erections, or urinary obstruction. In uncircumcised males, a sclerotic, constrictive band may form 1 to 2 cm from the distal end of a thickened, contracted, fissured prepuce that cannot be retracted.

Fully developed lichen sclerosus with hypopigmentation and distinctive shiny or crinkled texture changes is easily recognized. Extragenital lichen sclerosus must be differentiated from guttate morphea and lichen planus. Anogenital lichen sclerosus must be distinguished from genital lichen planus, lichen simplex chronicus, Bowen's disease, and Paget's disease. The differential diagnosis of a white plaque on the glans or distal shaft of the penis includes vitiligo, erythroplasia of Queyrat, lichen planus, localized scleroderma, and leukoplakia. When doubt of the diagnosis exists, a biopsy is indicated.

There is no effective, curative medical treatment for lichen sclerosus. Treatment with high-potency topical glucocorticoids, such as 0.05% clobetasol propionate, provides significant resolution of lichen sclerosus. Most patients will respond to twice-daily applications of these agents, tapered to once or twice weekly. Lichen sclerosus can be controlled, but not eradicated, by glucocorticoid therapy; it gradually recurs when therapy is discontinued. Oral retinoid therapy and treatment with topical tretinoin may be useful in both men and women with anogenital lichen sclerosus. Antimalarial agents may also be beneficial. Topical antibiotics or anti-infectious agents are indicated in patients in whom secondary candidal or bacterial infections exist. Significant urethral meatal stenosis due to balanitis xerotica obliterans is treated with hydrocortisone, but urethral dilation and/or meatotomy may be required. Circumcision may be curative if the lesions are localized to the prepuce.

Lichen sclerosus of the genitalia in both men and women carries a small but real risk of developing into a local squamous cell carcinoma. Patients should be monitored periodically, and focal persistent, hyperkeratotic, or ulcerated sites should be biopsied.

Accepted July 9, 2002.

Suggested Reading

(1.) Arndt KA, Leboit PE (eds). Cutaneous Medicine and Surgery: An Integrated Program in Dermatology. Philadelphia, W.B. Saunders Co., 1996, pp 895-899, 1351-1352.

(2.) Freedberg IM, Eiser AZ, Wolff K, et al (eds). Fitzpatrick's Dermatology in General Medicine. New York, McGraw-Hill Professional, 1999, vol 2, ed 5, pp 1351-1352, 1382-1384.

(3.) Gillenwater JY, Grayhack JT (eds). Adult and Pediatric Urology. St. Louis, C.V. Mosby, 1991, ed 2, p 2000.

(4.) Ledwig PA, Weigand DA. Late circumcision and lichen sclerosus et atrophicus of the penis. J Am Acad Dermatol 1989;20:211-214.

(5.) Odom RB, James WD, Berger TG (eds). Andrews' Diseases of the Skin: Clinical Dermatology. Philadelphia, W.B. Saunders Co., 2000, ed 9, pp 280-283.

(6.) Walsh PC, Retik AB (eds). Campbell's Urology. Philadelphia, W.B. Saunders Co., 1992, ed 6, pp 872-873, 1265-1266.

From the Department of Urology, University of Arkansas Medical Center, Little Rock, AR.

Reprint requests to Alex E. Finkbeiner, MD, Department of Urology, University of Arkansas Medical Center, 4301 W. Markham Street, Slot 540, Little Rock, AR 72205-7199. Email: [e-mail]

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