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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.
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