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Background: Lichen sclerosus is a chronic inflammatory autoimmune disease causing significant sclerosis, atrophy and pruritus. Treatment remains unsatisfactory, with potent corticosteroids being the most effective therapy., Objectives: To conduct a multicentre, phase II trial to assess the safety and efficacy of tacrolimus ointment 0.1% for the treatment of lichen sclerosus with a follow-up period of 18 months at 10 university and teaching hospitals in Germany and Austria., Methods: Eighty-four patients (49 women, 32 men and three girls) aged between 5 and 85 years with long-standing, active lichen sclerosus (79 with anogenital and five with extragenital localization) were treated with topical tacrolimus ointment 0.1% twice daily for 16 weeks. Computerized analysis of the lesional area was performed. The primary endpoint was clearance of active lichen sclerosus. Secondary endpoints were time to optimal response, reduction of sclerosis and duration of remission., Results: The primary endpoint (clearance of active lichen sclerosus) was reached by 43% of patients at 24 weeks of treatment. Partial resolution was reached in 34% of patients. Maximal effects occurred between week 10 and 24 of therapy. Treatment led to a significant reduction of the total lesional area (P < 0.01) and to a significant decline in the total symptom score (P < 0.005). Symptoms (e.g. itching) and findings (erythema, erosions and induration) showed significant improvement. No serious adverse events were observed. There were three (9%) recurrences during the follow-up period., Conclusions: Topical tacrolimus ointment 0.1% was safe and effective for the treatment of long-standing active lichen sclerosus.

Pyodermatitis-pyostomatitis vegetans is a rare, polymorphous inflammatory disorder of the skin and oral mucosa first described by Hallopeau in 1898. On the skin papules, pustules and reddish brown annular vegetating plaques develop, most frequently in the intertriginous areas. In the mouth, yellowish flat ulcerations arise, typically in the shape of "snail tracks". The association with inflammatory bowel disease is very common. An unusual case with a chronic relapsing course of 2 decades is presented. Gastrointestinal inflammation was absent. Prednisolone in high and medium doses suppressed most lesions. Various attempts with other drugs (dapsone, isotretinoin, azathioprine) to reduce the corticosteroid dose failed. This is the first report of the successful treatment of pyodermatitis-pyostomatitis vegetans with cyclosporin A, which proved to be highly effective in this regard. The unknown aetiopathology of pyodermatitis-pyostomatitis vegetans is discussed.