Related Experiment Video

Updated: Jun 15, 2026

An Intravital Microscopy-Based Approach to Assess Intestinal Permeability and Epithelial Cell Shedding Performance
07:32

An Intravital Microscopy-Based Approach to Assess Intestinal Permeability and Epithelial Cell Shedding Performance

Published on: December 3, 2020

[Köhlmeier-Degos disease (Malignant Atrophic Papulosis): a cause of recurrent multiple intestinal perforations]

M Beuran1, Al L Chiotoroiu, S Morteanu

  • 1Clinica de Chirurgie, Spitalul Clinic de Urgenţă Bucureşti. drmirceabeuran@yahoo.com

Chirurgia (Bucharest, Romania : 1990)
|March 2, 2010
PubMed

Insights

Malignant Atrophic Papulosis (MAP), a rare vasculopathy, can be fatal when it affects the gastrointestinal tract or central nervous system. This case highlights the severe prognosis and high mortality associated with systemic Degos disease.

Area of Science:

  • Vascular Medicine
  • Dermatology
  • Gastroenterology

Background:

  • Malignant Atrophic Papulosis (MAP), also known as Degos disease, is an extremely rare systemic vasculopathy.
  • It is characterized by a high mortality rate, particularly when gastrointestinal or central nervous system involvement occurs.

Observation:

  • A 29-year-old male presented with acute abdominal pain and skin lesions.
  • He had a history of appendectomy and subsequently developed multiple small bowel perforations requiring three reoperations.
  • Neuroradiologic imaging revealed central and peripheral nervous system involvement with a meningovascular pattern.

Findings:

  • The patient experienced progressive clinical deterioration despite aggressive medical and surgical interventions.
  • Systemic involvement led to peritonitis and sepsis.

Implications:

  • This case underscores the devastating potential of systemic Degos disease.
  • Early recognition and multidisciplinary management are crucial for patients with this rare condition.
  • Further research into effective treatments for Degos disease is warranted.

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