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Benign cutaneous Degos' disease
A M Farrell1, J Moss, C Costello
1Department of Histopathology, Charing Cross and Westminster Medical School, London, U.K.
Insights
A woman with Degos disease and lupus anticoagulant showed tubular structures in endothelial cells. Aspirin therapy halted her skin lesions, with no organ involvement after four years.
Area of Science:
- Vascular Biology
- Dermatology
- Immunology
Background:
- Degos disease is a rare condition characterized by skin lesions.
- Lupus anticoagulant is an autoimmune antibody associated with thrombotic events.
Observation:
- A 44-year-old woman presented with Degos disease and a lupus anticoagulant.
- Electron microscopy revealed interwoven tubular structures within endothelial cells of mature lesions.
- The patient developed cutaneous lesions without systemic organ involvement over four years.
Findings:
- The observed endothelial cell changes are consistent with previous Degos disease findings.
- Aspirin treatment (300 mg daily) effectively arrested the progression of cutaneous lesions.
- The patient showed no evidence of internal organ involvement during the four-year follow-up.
Implications:
- This case highlights the potential link between lupus anticoagulant and Degos disease.
- Low-dose aspirin may be a viable treatment option for managing cutaneous Degos disease.
- Further research is needed to elucidate the pathogenesis of Degos disease and its association with autoimmune conditions.
Abstract:
We report the case of a 44-year-old woman with Degos' disease who also had a lupus anticoagulant. Electron microscopy of the mature lesions showed interwoven tubular structures within the endothelial cells, as have been observed in previous cases of Degos' disease. Four years after her first cutaneous lesions, there is no evidence of involvement of other organs. Aspirin (300 mg daily) has arrested the cutaneous disease.