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Lethal systemic Degos disease with prominent cardio-pulmonary involvement
Ali Y Notash1, Hamed Mazoochy, Mostafa Mirshams
1Department of Surgery, Sina Hospital, School of Medical Sciences, University of Tehran, Tehran, Iran.
Saudi Medical Journal
|January 8, 2008
Summary
Degos disease (DD) is a rare obstructive vasculopathy. This case highlights a lethal systemic form of DD, emphasizing the importance of recognizing widespread skin lesions for early diagnosis and management.
Area of Science:
- Vascular Medicine
- Dermatology
- Pathology
Background:
- Degos disease (DD) is a rare obstructive vasculopathy.
- Characterized by distinctive skin lesions, DD can affect various organs.
- Gastrointestinal and central nervous system involvement often indicates a poor prognosis.
Observation:
- A 48-year-old man presented with widespread skin lesions, including palmoplantar and genital involvement, initially ignored for three years.
- The disease progressed rapidly, affecting the nervous, gastrointestinal, and cardiopulmonary systems.
- The patient experienced severe restrictive cardio-pulmonary insufficiency leading to death.
Findings:
- The autopsy revealed diffuse fibrotic changes in serosal membranes and internal organs.
- This case illustrates a lethal systemic manifestation of Degos disease.
- Delayed recognition of skin lesions contributed to the unfavorable outcome.
Implications:
- Early diagnosis and prompt management of Degos disease are crucial for improving patient outcomes.
- Widespread skin lesions, especially with palmoplantar and genital involvement, warrant thorough systemic evaluation.
- Understanding the diverse manifestations of DD is vital for clinicians managing rare vasculopathies.
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