Vaillant J M, Couly G, Oger P, Flageul G, Aubinière E, Leyder P
Rev Stomatol Chir Maxillofac. 1978;79(3):255-63.
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. Treatment is first preventive (decrease in exposure to sun and protection). Otherwise, surgery remains the most effective method: either excision as required, or extensive cervicofacial prophylactic excision followed by grafting.
这种病症是一种遗传性皮肤病，主要见于地中海沿岸，其特征为早期色素紊乱，几乎不可避免地会发展为弥漫性上皮瘤病，通常会导致患者在20岁前死亡。紫外线照射会促使病变进展。这些病变主要见于暴露部位，尤其是眼睑部位。治疗首先是预防性的（减少阳光照射和防护）。否则，手术仍然是最有效的方法：根据需要进行切除，或者进行广泛的颈面部预防性切除并随后进行植皮。
