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Cryofibrinogenemia: report of a case
1Department of Dermatology, National Taiwan University Hospital, Taipei, R.O.C.
Journal of the Formosan Medical Association = Taiwan Yi Zhi
|January 1, 1991
Summary
Cryofibrinogenemia, a rare plasma disorder, causes skin lesions like erythema and gangrene. Early diagnosis and treatment with immunosuppressants can improve symptoms and reduce cryofibrinogen levels.
Area of Science:
- Hematology
- Dermatology
- Immunology
Background:
- Cryofibrinogenemia is a rare condition characterized by reversible cryoprecipitate formation in plasma.
- It can lead to thromboembolic or hemorrhagic complications.
- Cutaneous manifestations are not widely recognized as a primary symptom.
Observation:
- A 66-year-old female presented with recurrent winter-associated reticulate erythema, purpura, and gangrene on extremities and face.
- Histopathology showed eosinophilic cylinders (fibrinogen) in dermal and subcutaneous blood vessels, without vasculitis.
- Immunofluorescence confirmed fibrinogen nature of deposits; immunofixation revealed an IgG-kappa M protein.
Findings:
- Cryofibrinogenemia was diagnosed based on plasma cryoprecipitate and histopathologic findings.
- The patient's cryocrit decreased from 13.6% to less than 2% after treatment.
- Treatment involved debridement, skin grafts, prednisolone, and cyclophosphamide.
Implications:
- This case highlights cryofibrinogenemia as a potential cause of severe, recurrent cutaneous manifestations.
- Early consideration of cryofibrinogenemia is crucial for patients with similar skin symptoms.
- Effective management can lead to clinical improvement and reduced cryofibrinogen levels.