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[Essential cryofibrinogenemia with generalized livedo racemosa]
M W Baumgärtel1, C Sunderkötter, W M Glöckner
1Hautklinik, Universität Münster.
Summary
Essential cryofibrinogenemia, a cold-induced blood disorder, caused livedo reticularis and neuropathy. Treatment with dexamethasone and cyclophosphamide resolved symptoms and cryofibrinogen levels.
Area of Science:
- Vascular Medicine
- Rheumatology
- Hematology
Background:
- Cryofibrinogenemia is a rare condition characterized by the presence of cryofibrinogen in the blood.
- Cold exposure can trigger symptoms in individuals with cryofibrinogenemia.
Observation:
- A 45-year-old male presented with cold-induced livedo reticularis, purpura, and peripheral polyneuropathy.
- Symptoms included Raynaud's phenomenon, malaise, vertigo, and transient amaurosis upon cold exposure.
- Skin biopsies showed dermal vessel dilation and leukocytoclastic vasculitis.
Findings:
- Essential cryofibrinogenemia was diagnosed due to cryoprotein detection in chilled plasma with increased viscosity.
- The patient exhibited a constellation of symptoms directly related to cold exposure.
Implications:
- Pulsed dexamethasone and cyclophosphamide therapy led to significant symptom relief.
- Post-treatment, cryofibrinogen was undetectable in the patient's plasma.
- This case highlights the efficacy of immunosuppressive therapy in managing severe cryofibrinogenemia.