[Keratosis lichenoides chronica with mucous membrane involvement and ocular pseudo-pemphigus. Follow-up study over 18 years. Retinoid therapy (author's transl)].

A very uncommon ocular and mucocutaneous syndrome is reported. Cutaneous manifestations include keratotic plugs occasionally disposed into verrucous plaques (in mountainous chain), lichenoid lesions on face, neck and hand's back, and nail's involvement. Histologically three types of lesions are observed: marked hyperkeratosis, lichenoid aspect, and follicular pustulosis. Mucous membranes alterations, present for almost as long as 20 years, include stomatitis, chronic balanitis with ulcerative and keratotic lesions leading to synechiae; ocular abnormalities has had a follow up into lesions similar to those of cicatricial pemphigoid; direct immunofluorescence study in the vicinity of a buccal erosion showed a complement deposit on basement membrane; immunofluorescence studies on involved and uninvolved skin, and indirect immunofluorescence were negative. Several diagnosis were considered, in first place an atypical Reiter's syndrome. A part the possibility of a new entity, the most attractive hypothesis is this of a keratosis lichenoides chronica with severe mucous cicatrical lesions. A large number of treatment had been inefficacious but a clear improvement of keratotic lesions was noted with oral retinoid therapy.