Lichen Sclerosis

Key Points
Chronic skin condition, most commonly affecting the perianal and genital regions
Cause is unknown, but contributing factors may include genetics, hormones, or infection, and it is thought to be an auto-immune disorder
Consists of patches of skin which turn white and thick or crinkled

Lichen sclerosus (LS) is a chronic skin condition which affects the perianal and genital regions. It consists of patches of skin which turn white, and may become thickened or crinkled. When it appears in the vulva, LS causes thickened white patching on the affected area, which may be very small or involve adjacent areas, but will never affect the interior of the vagina. This will be accompanied by severe itching and some soreness, occasional bruising, blisters and ulcers. Additionally, it can cause shrinking of the labia, clitoris and even the entrance of the vagina. Intercourse may cause fissuring of the skin in the affected area. Penile LS typically affects the glans, which becomes firm to the touch and white in color. This may be accompanied by a narrowing of the urethra and can force affected men to be circumcised. In approximately 1 out of 10 cases, LS may appear in other areas, including the thighs, breasts, buttocks, neck, armpits or shoulders.

The cause of LS is unknown, but it is thought to be an auto-immune disorder. Likely causes include genetic factors, hormonal factors, and infection. In some cases, it is associated with other conditions, including Vitiligo, Thyroid disease, anemia, Alopecia areata, and Psoriasis. Women are ten times as likely to develop LS, which may appear at any age, though it typically presents after 50.

Differential Diagnosis (Other conditions with similar appearance)
Acrodermatitis Chronica Atrophicans
Graft versus host disease
Albinism
Idiopathic guttate hypomelanosis
Anetoderma
Leukoplakia, Oral
Lichen Nitidus
Lichen Planus
Lupus erythematosus, Discoid
Bowen Disease
Morphea
Squamous Cell Carcinoma
Cutaneous T-cell lymphoma
Tinea Versicolor
Vitiligo
Extramammary Paget Disease

Diagnosis
Key Points
Diagnosis based on appearance of the affected area
Skin biopsy should be performed to confirm diagnosis and rule out other conditions

LS is typically diagnosed based on the characteristic appearance of the affected area. A skin biopsy should be performed to confirm the diagnosis and to rule out other, similar appearing conditions.

Treatment
Treatment typically consists of topical therapies
Goal of treatment is the alleviation of symptoms
Other therapies may be required in treating complications of the condition

Treatment of LS typically consists of the application of topical therapies, most often potent steroid ointments or creams, with the goal of that treatment being the alleviation of symptoms. Other therapies which may be prescribed include calcipotriol, topical or systemic retinoids, systemic steroids, and photodynamic therapy (procedure can be extremely painful, however). In cases which see shrinking of the vaginal opening, restretching procedures may be recommended, which may require surgery in very severe cases.