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[Benign familial Degos disease].

A-L Pinault¹, A Barbaud, F Weber-Muller

¹Service de Dermatologie, Hôpital Fournier, 36, quai de la Bataille, 54000 Nancy.

Annales De Dermatologie Et De Venereologie

|December 17, 2004

Summary

Degos' disease, a rare condition, can present with a prolonged benign course, as evidenced by a 26-year case. Familial forms of Degos' disease appear to have a less severe progression than sporadic cases.

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Area of Science:

Dermatology
Genetics
Internal Medicine

Background:

Degos' disease, or malignant atrophic papulosis, is characterized by distinctive skin lesions and often severe systemic involvement.
While typically associated with poor prognosis, benign and familial forms of Degos' disease are rare and under-reported.
The pathogenesis remains unclear, with ongoing debate regarding genetic predisposition versus infectious etiology.

Observation:

A 41-year-old woman with a 26-year history of cutaneous Degos' disease had a son who developed similar skin lesions at age 20.
Neither patient exhibited thrombotic or immunological abnormalities, and karyotype results were normal.

Findings:

This case represents the longest documented benign evolution of Degos' disease.
Familial forms of Degos' disease, based on this and prior literature, suggest a potentially milder disease course compared to sporadic presentations.
The long-term prognosis for the son remains uncertain, as systemic involvement can manifest later in the disease's progression.

Implications:

Degos' disease can exhibit prolonged benign cutaneous manifestations, challenging its typical association with severe systemic outcomes.
Familial clustering suggests a possible genetic component influencing disease severity and progression.
Further research is needed to elucidate the varying clinical spectrum and underlying mechanisms of Degos' disease, particularly in familial contexts.

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