Race, Sex & Age Affected By Degos Disease

Race
Some state that Degos Disease generally occurs in white young adults. However, it is reported in blacks in Africa, Arabs in Jordan, Asians in Japan, and elsewhere. Any racial link is uncertain.

Sex
In 1997, Katz et al noted that the disorder usually occurs in young adults, and the male-to-female ratio is approximately 3:1.
Wilson et al reviewed benign cutaneous MAP in 34 men and women (30 adults and 4 kids) and noted that benign MAP is more commonly reported in women, at a female-to-male ratio of 3:1.

Age
All ages are affected. The fatal systemic variant of Degos Disease can occur in children. In 1999, Lankisch et al described a 16-year-old white adolescent girl with acute abdominal pain due to visceral involvement of Degos Disease that required extensive small-bowel resection. The skin manifestations had been present for 2 years before the correct diagnosis was made. She died as a result of CNS involvement from Degos Disease. Jalil et al described a case in a 2-year-old child who presented with chronic abdominal pain of uncertain origin.

The benign cutaneous variant of Degos Disease can occur in adults. Wilson et al looked at 34 patients with benign cutaneous MAP and found their average age was 37.6 years. In 1998, Farrell et al described a case of a 44-year-old woman with Degos Disease and a lupus anticoagulant who, 4 years later, was alive and without systemic involvement. Electron microscopy of the white papules demonstrated interwoven tubular structures within the endothelial cells. This was consonant with reports in previous studies of DD. Farrell et al thought that aspirin (300 mg/d) kept her cutaneous Degos Disease in check. In 1998, Requena et al described a 58-year-old homosexual man with AIDS who developed typical cutaneous lesions of MAP, with no visceral involvement detected 2 years after the diagnosis of Degos Disease.

Degos Disease can occur in infants. Degos Disease has been reported in a 7-month-old girl who showed spontaneous aggregation of platelets. A good clinical response in this patient was obtained by treatment with aspirin and dipyridamole.