Abstract | BACKGROUND: Cryofibrinogenaemia is an under-recognized cutaneous thrombotic vasculopathy that may be revealed by purpura or chronic necrotic ulcerations. We report two original cases characterized by their severity, their association with a monoclonal gammopathy and their excellent response to treatment. PATIENTS: CONCLUSION: Routine screening for cryofibrinogen in plasma should be performed to explore cutaneous symptoms of unexplained thrombotic vasculopathy, even in the presence of a non-specific skin biopsy. Specific treatment of cryofibrinogenaemia associated monoclonal gammopathy appears to be highly effective against manifestations of cryofibrinogenaemia.
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Authors | J Delyon, M Bezier, F Cordoliani, M Malphettes, R Szalat, M Bagot, M Rybojad |
Journal | Annales de dermatologie et de venereologie
(Ann Dermatol Venereol)
Vol. 140
Issue 1
Pg. 30-5
(Jan 2013)
ISSN: 0151-9638 [Print] France |
Vernacular Title | Manifestations cutanées révélant une cryofibrinogénémie associée à une gammapathie monoclonale. |
PMID | 23328357
(Publication Type: Case Reports, English Abstract, Journal Article)
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Copyright | Copyright © 2012 Elsevier Masson SAS. All rights reserved. |
Chemical References |
- Anti-Inflammatory Agents
- Antineoplastic Agents
- Boronic Acids
- Cryoglobulins
- Fibrinogens, Abnormal
- Immunoglobulin kappa-Chains
- Immunoglobulin lambda-Chains
- Pyrazines
- cryofibrinogen
- Bortezomib
- Dexamethasone
- Cyclophosphamide
- Prednisone
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Topics |
- Acute Kidney Injury
(diagnosis, pathology, therapy)
- Adult
- Aged, 80 and over
- Anti-Inflammatory Agents
(administration & dosage)
- Antineoplastic Agents
(administration & dosage)
- Biopsy
- Boronic Acids
(administration & dosage)
- Bortezomib
- Cryoglobulinemia
(diagnosis, pathology, therapy)
- Cryoglobulins
(metabolism)
- Cyclophosphamide
(administration & dosage)
- Dexamethasone
(administration & dosage)
- Drug Therapy, Combination
- Female
- Fibrinogens, Abnormal
(metabolism)
- Humans
- Immunoglobulin kappa-Chains
(blood)
- Immunoglobulin lambda-Chains
(blood)
- Kidney
(pathology)
- Leg Ulcer
(diagnosis, pathology, therapy)
- Microscopy, Fluorescence
- Necrosis
- Paraproteinemias
(diagnosis, pathology, therapy)
- Plasmapheresis
- Prednisone
(administration & dosage)
- Pyrazines
(administration & dosage)
- Skin
(pathology)
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