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 .

Diseases
Validation

Diseases presenting "sepsis" symptom

acute rheumatic fever

carcinoma of the gallbladder

congenital diaphragmatic hernia

cushing syndrome

cystinuria

dystrophic epidermolysis bullosa

epidermolysis bullosa simplex

focal myositis

harlequin ichthyosis

heparin-induced thrombocytopenia

hirschsprung disease

homocystinuria without methylmalonic aciduria

inclusion body myositis

junctional epidermolysis bullosa

kindler syndrome

lamellar ichthyosis

locked-in syndrome

malignant atrophic papulosis

megacystis-microcolon-intestinal hypoperistalsis syndrome

monosomy 21

primary effusion lymphoma

primary hyperoxaluria type 1

pyomyositis

scrub typhus

systemic capillary leak syndrome

typhoid

This symptom has already been validated