Malignant atrophic papulosis (MAP) is a rare disease with an extremely grim prognosis, death being due to gastrointestinal perforation and neurological disorders. We report a severe case of MAP in a 37-year old woman. The patient had three emergent laparotomies in 3 months for recurrent acute peritonitis due to omental thrombosis resulting in necrosis, multiple intestinal and mesenteric lesions, and severe gastrointestinal perforations. Multiple papular skin lesions were present for 1 year prior to surgery. Pathological findings revealed MAP. Surgical intervention was successful and the patient recovered smoothly but later died of malnutrition and septicaemia.
BallENewburgerAAckermanAB: Degos' disease: A distinctive pattern of disease, chiefly of lupus erythematosus, and not a specific disease per se. Am J Dermatopathol2003; 25: 308–320.
8.
KatzSKMuddLJRoenigkHHJr: Malignant atrophic papulosis (Degos' disease) involving three generations of a family. J Am Acad Dermatol1997; 37: 480–484.
9.
HabbemaLKischLSStarnikTM: Familial malignant atrophic papulosis (Degos' disease) additional evidence for heredity and a benign course. Br J Dermatol1986; 114: 134–135.
Fernández-PérezERGrabscheidEScheinfeldNS: A case of systemic malignant atrophic papulosis (Köhlmeier–Degos' disease). J Natl Med Assoc2005; 97: 421–425.
19.
ChenXLShenBSunM: One case of malignant atrophic papulosis. Chin J Dermatol2005; 38: 247–248 [in Chinese].
20.
KimDWKangSBLeeKH: Degos' disease (malignant atrophic papulosis) as a fatal cause of acute abdomen: Report of a case. Surg Today2008; 38: 866–870.
21.
LankischMRJohstPScolapioJS: Acute abdominal pain as a leading symptom for Degos' disease (malignant atrophic papulosis). Am J Gastroenterol1999; 94: 1098–1099.
