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- Title
A 43-year-old woman with a porcelain rash and recurrent bowel perforations.
- Authors
Wiens, Jeremy; Dunham, Michael; Walker, Simon; Au, Selena
- Abstract
This document discusses Köhlmeier-Degos (KD) disease, a rare vasculopathy that can manifest as either a benign or malignant form. The disease primarily affects the gastrointestinal and central nervous systems. The presence of specific skin plaques is a characteristic feature of KD disease and should prompt immediate referral to a dermatologist and specialized center. Malignant transformation of lesions is common and can lead to high mortality if left untreated. Treatment options are limited, and early recognition and monitoring are important for improving outcomes. The document also presents a case study of a patient with the malignant form of KD disease, highlighting the challenges and limited treatment options.
- Subjects
SURGERY; GASTROINTESTINAL system; FECAL occult blood tests; THERAPEUTICS; PAROXYSMAL hemoglobinuria; INTESTINAL perforation
- Publication
Canadian Medical Association Journal (CMAJ), 2024, Vol 196, Issue 29, pE1006
- ISSN
0820-3946
- Publication type
Article
- DOI
10.1503/cmaj.240353