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[Malignant atrophic papulosis (Degos' syndrome)]
A Stejskalová1, M Stanová, F Vosmík
1Hlavův I. patologicko-anatomický ústav fakulty vseobecného lékarství Univerzity Karlovy, Praha.
Sbornik Lekarsky
|January 1, 1990
Summary
Degos' syndrome, a rare condition, presented as skin papules and gastrointestinal issues. Autopsy revealed thrombi, confirming the diagnosis of papulosis maligna atrophicans.
Area of Science:
- Dermatology
- Gastroenterology
- Pathology
Background:
- Papulosis maligna atrophicans (Degos' syndrome) is a rare disorder characterized by skin lesions and potential systemic involvement.
- Initial diagnosis in this case was scleroderma guttata, highlighting diagnostic challenges.
Observation:
- A 49-year-old female presented with a six-year history of lentil-shaped papules and recent onset of dyspeptic complaints, weight loss, and polyserositis.
- Surgical exploration revealed whitish foci on the intestinal serosa and omentum, with skin biopsy showing a bland infarct.
- Autopsy confirmed thrombi and bland infarcts in the small intestine, with jejunal perforation.
Findings:
- The clinical presentation and pathological findings were consistent with Degos' syndrome.
- Histopathology revealed bland infarcts in the skin and small intestine.
- Thromboangiitis of Bürger's type was identified as the underlying vascular pathology.
Implications:
- This case underscores the importance of considering rare conditions like Degos' syndrome in patients with unexplained skin and gastrointestinal symptoms.
- Early recognition and diagnosis are crucial for managing this potentially fatal disorder.
- Further research into the pathogenesis and treatment of Degos' syndrome is warranted.