Degos Syndrome

Rare form of systemic occlusive arteriopathy of small and medium-size arteries with a typical two-stage evolution. Disease affects vessels of the dermis with an aspect of erythematous, dome-shaped papules that develop a central area of necrosis and leave a porcelain-like scar. Gastrointestinal tract and CNS can be affected. Can be fatal.

Malignant Atrophic Papulosis; Papulosis, Malignant Atrophic; Degos Disease; Degos-Kohlmeier Disease; Kohlmeier-Degos Disease.

Disease was first described in 1941 by W. Kohlmeier, a German radiologist, and first individualized in 1942 by Robert Degos, a French dermatologist.

Autosomal dominant inheritance is observed in familial cases.

Unknown. Familial cases have been described. Immunologic, infectious, or thrombotic factors have been evocated.

Clinically evocated onset usually in young adults between 20 and 40 years old, probably with male predominance. Characteristic skin lesions (pink, asymptomatic, diffuse papules with a telangiectatic border followed by umbilication and a porcelain-white center) appear. Histologic appearance is typically characterized by a wedge-shaped area of necrosis from the epidermis through the dermis.

Skin lesions can be followed by systemic manifestations, sometimes a few years later. Features can involve the eyes (avascular patches), CNS (20% cerebrovascular or medullar accident), and GI system (40-60%, particularly small bowel with abdominal pain, bleeding, and diarrhea; intestinal perforations and peritonitis are the leading causes of death). Pleural and pericardial effusion can be observed.

Obtain full history. Evaluate neurologic lesions (clinical, CT/MRI). Pleural and pericardial effusion must be searched and treated before anesthesia if necessary (clinical, ECG, chest radiographs, echography). Full blood count is necessary.

Eye protection is needed. Spinal and perimedullar blockades probably should be avoided because of the frequency of medullar accidents. Should obtain a bleeding time and coagulation profile.

Aspirin is often used to treat patients. Heparin substitution should be considered.