Lichen sclerosis is a chronic inflammatory skin disease that causes substantial discomfort and morbidity, most commonly in adult women, but also in men and children. Any skin site may be affected (and, rarely, the oral mucosa) but lichen sclerosus is most common in the anogenital area, where it causes intractable itching and soreness. In children, the disorder may be confused with changes seen in sexual abuse. Progression to destructive scarring is common. There is increased risk of developing vulval cancer, and there are links with penile cancer. Patients should be kept under long-term review. Lichen sclerosus can occur without symptoms, and the exact prevalence is uncertain. It occurs most commonly in women at times of low sex hormone output. The underlying cause is unknown, but there seems to be a genetic susceptibility and a link with autoimmune mechanisms. The wart virus and the spirochaete borrelia have been suggested but not substantiated as infective triggers. The Koebner phenomenon is known to occur (lichen sclerosus occurs in skin already scarred or damaged), so trauma, injury, and sexual abuse have been suggested as possible triggers of symptoms in genetically predisposed people. The treatment of choice for anogenital lichen sclerosus is potent topical corticosteroid ointment for a limited time. Circumcision may be indicated in men, and surgery may be considered in women, to relieve effects of scarring or to treat coexisting carcinoma. Current research aims to identify a treatable cause of lichen sclerosus, to identify patients at risk of scarring and of malignant disorders, and to find target pathways for therapeutic intervention.

To record the clinical features, symptomatic response to topical steroids, and resolution of clinical signs in a large cohort of female patients with vulvar lichen sclerosus.Design: Descriptive cohort study with a mean follow-up of 66 months. Setting:The vulvar clinics of a teaching hospital and of a district general hospital in Oxfordshire, England.Patients: Three hundred twenty-seven patients (74 girls and 253 women) with a definite clinical diagnosis of vulvar lichen sclerosus. Interventions:The patients received topical steroids as part of their normal care.Main Outcome Measures: Symptomatic response to treatment (good, partial, or poor); response of the vulvar signs (total, partial, minor, or poor); and the presence or absence of moderate or severe scarring. Results:The mean age at onset was 5.4 years in girls and 55.1 years in women and first-choice therapy was an ultrapotent topical steroid for 50% of the girls and 89% of the women. Response to treatment was recorded in 255 patients. In 244 patients (96%) symptoms improved with treatment, as 168 (66%) became symptom free and 76 (30%) showed partial response; 11 (4%) had poor response. Among the 253 patients in whom a response of the vulvar signs to topical steroid was recorded, 58 (23%) showed total response, with return to normal skin texture and color; and 173 (68%) showed partial, 18 (7%) showed minor, and 4 (2%) showed poor response. Moderate or severe scarring occurred less often in girls (PϽ.001). Squamous cell carcinoma developed in 6 women (2.4%). Conclusion:Topical ultrapotent steroid is an effective treatment for vulvar lichen sclerosus, giving relief of symptoms in most and completely reversing the skin changes in approximately one fifth of patients.

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In a series of 18 patients with folliculitis decalvans attending the Oxford hair clinic, eight were found to have areas of tufted folliculitis either at presentation or follow-up. There was no difference between these two groups in their presentation, clinical course, growth of causative organism (Staphylococcus aureus) or investigations including histology. We suggest that these two entities form part of a spectrum of a single disease. We performed lymphocyte staining on affected scalp biopsies, including CD4: CD8 and T-cell/B-cell ratios, but found no evidence of local immune suppression or failure which would explain the abnormal host response to a common pathogen in this rare condition. We introduced a new treatment regimen for these patients, oral rifampicin and oral clindamycin together for 10 weeks. Ten of the 18 patients have responded well with no evidence of recurrence 2-22 months after one course of treatment, and 15 of the 18 responded after two or three courses.

Lichen sclerosus (LS) is an inflammatory skin disease that usually involves the anogenital area. All patients with symptoms or signs suspicious of lichen sclerosus should be seen at least once initially by a physician with a special interest in the disease in order to avoid delay in diagnosis, as early treatment may cure the disease in some and reduce or prevent scarring. The diagnosis is made clinically in most cases. Biopsies should only be performed under certain circumstances. The gold standard for treatment remains potent to very potent topical steroids; however, mild and moderate disease in boys and men may be cured by circumcision. Certain triggers should be avoided.

Lichen sclerosus is among the most frequently seen paediatric vulval disorders. In adults a strong association between lichen sclerosus and autoimmune diseases, and also with HLA class II locus DQ7, has been well demonstrated in women and a weaker association in men. These associations have not previously been studied in children, although in other autoimmune diseases, the HLA associations have been strongest in children. We performed HLA tissue typing and looked for autoimmune associations in a group of 30 children with vulval lichen sclerosus. HLA DQ7 was present in 66% of female children with lichen sclerosus compared with 31% in controls. Previous studies reported DQ7 in 51% of adult female patients and 45% of male patients. Sixteen per cent of the children were homozygous for DQ7 as opposed to 5% of controls. In the childhood group, only 4% had another autoimmune disease, but 56% of their parents or grandparents did. Age differences make comparison difficult, but the family history of autoimmunity appears to be strong in the early-onset group, in addition to the stronger association with DQ7.

There appears to be a definite association between SCC of the penis and the presence of LS, similar to that reported between LS and vulval SCC in women. Of the 20 patients with penile SCC studied, 11 had a clinical history and/or histological evidence of LS. However, clinical presentation of the LS or need for circumcision may precede the SCC by many years. As follow-up is impractical, counselling at the time of diagnosis is very important, and it is essential that medical practitioners are aware of this association so that the subsequent risk from SCC is reduced.