Rare Diseases Symptoms Automatic Extraction

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 presenting "abdominal pain" symptom

  • 22q11.2 deletion syndrome
  • adrenal incidentaloma
  • alpha-thalassemia
  • benign recurrent intrahepatic cholestasis
  • carcinoma of the gallbladder
  • child syndrome
  • cholangiocarcinoma
  • congenital diaphragmatic hernia
  • cushing syndrome
  • cutaneous mastocytosis
  • cystinuria
  • dedifferentiated liposarcoma
  • erdheim-chester disease
  • erythropoietic protoporphyria
  • fabry disease
  • familial hypocalciuric hypercalcemia
  • familial mediterranean fever
  • focal myositis
  • liposarcoma
  • lymphangioleiomyomatosis
  • malignant atrophic papulosis
  • pleomorphic liposarcoma
  • primary effusion lymphoma
  • primary hyperoxaluria type 1
  • proteus syndrome
  • scrub typhus
  • sneddon syndrome
  • systemic capillary leak syndrome
  • typhoid
  • von hippel-lindau disease
  • well-differentiated liposarcoma

This symptom has already been validated