Lichen sclerosus - Lichenoid lesions

Lichen sclerosus

Definition

Lichen sclerosus (previously lichen sclerosus et atrophicus) is an uncommon chronic progressive disorder of the skin characterised by inflammation and epithelial thinning.

Age

Most common in postmenopausal women, but can occur at any age.

Sex

F > M

Aetiology/pathophysiology

The cause is unknown; however, there is a familial tendency possibly with an HLA association. There is also an association with autoimmune conditions such as thyroid disorders. Trauma may play a role as lesions occur at sites of skin trauma (Koebner¨ phenomenon).

Clinical features

Lichen sclerosis is most commonly seen in the anogenital region. Patients may complain of itching, dysuria and dyspareunia. On examination there are atrophic, white macules on vulva or penis, occasionally extending to involve the perineum. There may be fissures, excoriation and secondary lichenification with loss of architecture (phymosis in males). Extragenital white plaques due to hyperkeratosis may occur on other areas of skin or rarely, the oral cavity.

Complications

Longstanding disease predisposes to squamous cell carcinoma.

Investigations

A biopsy may be required if the diagnosis is not clear. The epidermis may show areas of thinning and hyperkeratosis. A lymphocytic infiltrate is seen in the lower dermis, and immunofluorescence may be required to exclude cicatricial pemphigoid.

Management

Genital lesions may be treated with potent topical steroid ointments. Hydroxychloroquine is used in refractory cases. Maintenance therapy may be required to prevent recurrence. Surgery is avoided due to the Koebner¨ phenomenon but may be required for adhesions, phymosis or introital stenosis. Long-term follow-up with biopsy of any area suspicious of squamous cell carcinoma is recommended.

Prognosis

The condition responds well to potent topical steroids. Spontaneous remission may occur in childhood cases around puberty.