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Degos' disease.

F O Güven1, K E Bozdağ, M Ermete

  • 1Dermatology and Pathology Departments of Atatürk Training Hospital, Izmir, Turkey.

International Journal of Dermatology
|June 10, 2000
PubMed
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Degos disease, a rare condition, presented in a 47-year-old woman with characteristic skin lesions and internal organ involvement. This case highlights the multisystemic nature of Degos disease.

Area of Science:

  • Dermatology
  • Internal Medicine
  • Pathology

Background:

  • Degos disease (Malignant atrophic papulosis) is a rare systemic vasculopathy characterized by cutaneous and gastrointestinal manifestations.
  • The condition often presents with characteristic papular lesions that progress to atrophic scars.

Observation:

  • A 47-year-old woman developed pruritic, burning skin lesions with porcelain-like atrophy and erythematous borders on her trunk, legs, and arms.
  • She also experienced thoracic pain, diagnosed as pleuritis, and severe abdominal pain, diagnosed as peritonitis.
  • Histopathology revealed hyperkeratosis, epidermal atrophy, dermo-epidermal separation, edema, necrosis, and vascular thrombosis.

Findings:

  • Clinical and histopathologic findings confirmed the diagnosis of Degos disease.

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  • The patient was treated with anti-inflammatory drugs.
  • One year later, she developed pericarditis and pleuritis, indicating ongoing systemic involvement.

    • Implications:

    • This case underscores the importance of recognizing the diverse clinical presentations of Degos disease.
    • Early diagnosis and management are crucial due to the potential for severe systemic complications.
    • Further research into the pathogenesis and treatment of Degos disease is warranted.