Generalized atrophic benign form of junctional epidermolysis bullosa.

There are at least six variants of junctional epidermolysis bullosa (JEB). About 20 cases of the generalized atrophic benign variant of JEB (GABEB) have been previously reported. We present an additional case of GABEB, occurring in a 14-year-old girl.

[Xeroderma pigmentosum. Therapeutic indications on the basis of 3 cases seen recently (author's transl)].

This condition is a genodermatosis, seen chiefly around the shores of the Mediterranean, characterised by early pigment disturbances which progress virtually inexorably towards a diffuse epitheliomatosis which usually results in death before the age of 20 years. Progression of the lesions is precipitated by exposure to UV rays. These lesions are seen essentially in exposed areas, in particular oculopalpebral.