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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.

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

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

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Autosomal dominant inheritance is observed in familial cases.

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

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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.

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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.

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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.

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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.

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

Degos R, Delort J, Triscot R: Dermatite papulo squameuse atrophiante. Bull Soc Franc Derm Syph 49:148, 1942.
Ojeda Cuchillero RM, Sanchez Regana M, Umbert Millet P: Benign cutaneous Degos' disease. Clin Exp Dermatol 28:145, 2003.
Torrelo A, Sevilla J, Mediero IG, et al: Malignant atrophic papulosis in an infant. Br J Dermatol 146:916, 2002.  [PubMed: 12000397]

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