Lichen Sclerosis et Atrophicus

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Kraurosis Vulvae / Balanitis Xerotica Obliterans

Patients can be divided into three clinical groups for management: prepubertal children, adults with anogenital disease, and adults with extragenital disease. Prepubertal children at times have spontaneous clearing at menarche. Extragenital disease is often quite refractory to therapy, but is often asymptomatic while adults with anogenital disease often have symptoms of burning and pruritus and do respond to therapy.

First Steps

Adults with anogenital lesions often respond to topical application of superpotent steroids (Class 1). For most patients, daily application of a superpotent steroid in ointment or cream base will produce a dramatic response in weeks.

Subsequent Steps

1. The therapeutic response may be maintained with daily application of a mid potent steroid in association with weekend application of the superpotent steroid.
2. When symptoms are stable, topical steroids can be further tapered with regard to strength and frequency depending on the duration and quality of therapeutic response.

Alternative Steps

1. Topical tacrolimus 0.1% ointment applied once or twice daily is also effective and is used to avoid atrophy induced by superpotent topical steroids but should be reserved for patients in whom topical steroids are ineffective or not tolerated.
2. Topical calcipotriene (once or twice daily) or a mild topical retinoid (up to daily as tolerated) may also be effective in patients failing topical steroids or tacrolimus alone.
3. For extragenital lichen sclerosis which does not respond to superpotent steroids, potassium p-aminobenzoic acid, PUVA, UVA1 or antimalarials may be useful.

Subsequent Therapy

If topical treatment is ineffective, hydroxychloroquine 200 mg twice daily may be tried.
Cyclosporine 3--4 mg/kg/day can be effective in refractory cases.
Photodynamic therapy may be attempted in the most refractory and symptomatic cases.

Pitfalls

1. Lichen sclerosis of the genitalia is associated with an increased risk of genital squamous cell carcinoma, and patients should be followed for this possibility. Topical tacrolimus may increase the risk for the development of neoplasia.
2. Superpotent topical steroid treatment is often associated with coexistent candidiasis, which can be managed with appropriate oral or topical agents.
3. Laser and excisional surgery may be followed by recurrence, and should not be undertaken except by experts in this disease and should not be used except in those patients failing all other forms of treatment. Vulvectomy is not indicated except for cases of neoplasia.

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