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Degos disease was named after Dr Degos who recognized this disease as a distinct entity in 1942. However, it had already been described Dr Kohlmeier one year before. Less than 200 cases have been reported in the literature. Degos disease, also called malignant atrophic papulosis, is due to a vaso-occlusion. Age at onset usually lies between 20 and 40 years with probably a male predominance. The pathophysiology has not been elucidated yet but genetic (familial cases have been described), immunological, infectious or thrombotic factors may be involved. Skin lesions are the first manifestations of the disease. They consist in pink, asymptomatic and diffuse papules usually on the trunk and extremities. Those lesions appear with a telangiectatic border. Umbilication develops afterwards with a porcelain-white center. Lesions at different stages of evolution may coexist. Cutaneous symptoms are followed by systemic manifestations, sometimes a few years later. They are due to ischemic lesions leading to infarcts. Gastrointestinal tract is the organ most commonly involved (40% to 60%), predominantly the small bowel. Symptoms are abdominal pain, bleeding and diarrhea. Then central nervous system is involved (about 20%) with cerebrovascular accident, more rarely medullar. Pleural/pericardial, ocular, pulmonary involvement and other lesions have been described. Biological findings are normal. Skin biopsy is helpful for diagnosis. Histological studies show a wedge-shaped area of cutaneous ischemia, arising from epidermis and reaching hypodermis, with sclerosis and necrosis. Thrombotic vessels can be seen, without vasculitis, but with a thickened wall and sometimes fibrinoid necrosis. Prognosis is poor; intestinal perforations and peritonitis are the leading causes of death. No specific treatment is available, nevertheless some authors recommend anti-platelet therapy or anticoagulants. |