[Benign familial Degos disease].

INTRODUCTION

Degos' disease or atrophic malignant papulosis is defined by porcelain white cutaneous lesions with atrophic scarring, often associated with severe and fatal systemic involvement (visceral and neurological). Benign forms are rare or under-reported and the familial forms are exceptional. It is a very rare disease, only two hundred cases have been reported in the literature with a sex ratio of 3M/1F. The pathogenesis of Degos' disease remains controversial. The exceptional observation of familial form raises the question of a genetic predisposition of this disease or an infectious aetiology with a low virus.

OBSERVATION

A 41 year-old woman was known to have Degos' disease for 26 years with only cutaneous manifestations. One of her two sons developed atrophic cutaneous lesions at the age of 20. In both patients, no thrombotic or immunological abnormalities were found. The karyotype was performed with normal results.

DISCUSSION

Degos' disease or malignant atrophic papulosis can have a long lasting benign evolution. Our patient, who had presented a benign form for 26 years, had the longest evolution ever documented in literature. We cannot be sure that her son will have a benign course of his Degos' disease because the diagnosis is recent and because the systemic involvement can appear after many years of evolution. In the familial forms, from our study and the 31 cases previously described in the literature, with ten different families, the course of the disease seems to be less severe than in sporadic forms. Among these familial forms of Degos' disease, only 4 patients presented a malignant form, which in one case did not prove a relationship between the death and the Degos' disease. Are the sporadic forms with only skin involvement less frequently reported? Has the familial form of Degos' disease the same course as a very severe common sporadic form?