Lichen Sclerosis et Atrophicus

This chronic skin disease most commonly affects the skin of the anogenital region in women, and the glans penis and foreskin in men. It occurs less commonly in extra-genital sites. It most often affects post-menopausal women. The female to male ratio is approximately 7:1. Children may also be affected (about 15% of cases) and it is an important differential diagnosis of the signs of suspected sexual abuse. Lichen sclerosis et atrophicus does not cause any systemic disease outside the skin. Its cause remains unclear. Genetic, autoimmune and infective mechanisms have been considered, but no definitive data has ever been found. There are no reliable figures for population incidence or prevalence. It is exceedingly rare in circumcised men.

Male patients present with lesions on the glans penis and foreskin – but generally not the perianal region. As well as soreness and itching, there may be difficulty in retracting the foreskin and a poor urinary stream. Often a non-retractile foreskin requires circumcision. Lichen sclerosus commonly recurs in the circumcision scar. There is an increased risk of developing squamous cell carcinoma within genital lesions and careful follow up is necessary.

Presentation:
The condition may have no symptoms. It is sometimes discovered during evaluation for another problem. Usually one experiences severe itching and soreness of the genitals. White plaques develop on the skin and form shiny, white areas. Female patients usually have an hourglass shape of inflamed skin around the vulva and anus. Pain with urination, sexual intercourse and defecation are common. There may be traumatic tearing of the skin during intercourse or defecation. Scarring is common – the labia minora may fuse making urination difficult, and the entrance of the vagina can become very narrow. Occasionally the disease presents before puberty, where the ‘bruised’ red, purpuric signs appear to suggest abuse.

Lichen sclerosus affects skin outside the genitals in 15-20%. The Koebner phenomenon may occur: disease arises in scars, burned or repeatedly traumatized skin. Rarely, it may affect the oral mucosa. Itching of the extra-genital form is unusual. Extra-genital lesions may require no therapy if asymptomatic or not causing significant cosmetic effect. There is no increased risk of squamous cell carcinoma in extra-genital lesions.

Investigations:
Skin punch biopsy is useful to confirm the diagnosis and exclude malignancy.

Treatment:
Administration of potent topical steroid preparations, (clobetasol propionate 0.05% ointment) given once daily for 1–2 months is the treatment of first choice. The frequency of applications is gradually reduced to zero. Treatment is then usually given on as needed basis. Potent steroids should be used under the advice/supervision of a dermatologist. Wash with bland emollients to avoid topical irritants and avoid tight clothing; use lubricants if necessary. Non-healing erosive or warty lesions may indicate cancerous change.

A 6-8 week course of ultrapotent topical corticosteroid is a safe and effective treatment for genital lichen sclerosus in pediatric patients, although spontaneous resolution may occur. Patients may suffer psychosexual problems; a psychologist/psychiatrist or appropriate counselor may be necessary. Surgical procedures are sometimes necessary, although vulvectomy is not indicated unless there is a tumor. Phimosis needs circumcision. Surgery may be needed in women to repair damage by scarring. Follow-up with topical steroids and dilators is needed to prevent recurrence.

Prognosis:
With early and careful evaluation by dermatology/gynecology/urology, the burden of this disease is lessening and many patients lead happy lives with normal urinary and sexual function. However, the course is highly variable and some patients will inevitably be severely affected. There is a 4-5% risk of squamous-cell carcinoma (SCC) of the vulva, and there may be a slightly increased risk of SCC of the penis. Long term follow-up is suggested. Biopsies of any suspicious lesions are advised. Extra-genital lesions do not appear to have any increased risk of skin cancer.

Valerie Nozad, D.O.
Department of Dermatology
Philadelphia College of Osteopathic Medicine/Frankford Hospital
Philadelphia, Pennsylvannia.