Degos' disease (malignant atrophic papulosis) as a fatal cause of acute abdomen: report of a case.

[malignant atrophic papulosis]

Degos ' disease , otherwise known as "malignant atrophic papulosis ," is a rare condition characterized by typical cutaneous lesions . Its involvement of the gastrointestinal (GI ) tract is usually associated with a poor prognosis . We report a case of Degos ' disease with GI involvement , which ultimately caused peritonitis , sepsis , and death , despite all treatment measures . A 59 -year old woman was admitted to our hospital with acute generalized abdominal pain . The patient had presented initially with multiple skin lesions 2 years earlier , and even with surgery for small -bowel perforation 10 months before this admission , Degos ' disease had not been diagnosed . Explorative laparotomy revealed multifocal , ischemic changes in the small bowel with perforation in the mid-jejunum . After the operation , she suffered recurrent small -bowel fistulas and died within 3 months . In a patient with acute abdominal pain and typical atrophic papules , clinicians should retain a high index of suspicion for Degos ' disease with GI involvement , even though it is rare .

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alexander disease

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carcinoma of the gallbladder

child syndrome

cowden syndrome

cushing syndrome

cystinuria

epidermolysis bullosa simplex

erdheim-chester disease

hirschsprung disease

lamellar ichthyosis

lymphangioleiomyomatosis

malignant atrophic papulosis

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well-differentiated liposarcoma

wiskott-aldrich syndrome

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