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Cryofibrinogenemia
Other Resources UpToDate PubMed

Cryofibrinogenemia

Contributors: Casey P. Schukow DO, Vivian Wong MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Cryofibrinogenemia is an acquired vascular occlusive disease that results from the cold precipitation of cryofibrinogens. Cryofibrinogens are composed of a complex of fibrin, fibrinogen, fibrin split products, albumin, plasma proteins, and immunoglobulins that reversibly precipitate in the cold. These cryoproteins cause an occlusive vasculopathy of small- to medium-size blood vessels. While many patients are asymptomatic, cryofibrinogenemia can cause significant morbidity. The clinical examination and course is similar to type I cryoglobulinemia. Cryofibrinogenemia can be idiopathic (primary) or associated with malignancy (particularly prostatic cancer, other solid organ tumors, and lymphomas), viral infection, collagen vascular disease, thromboembolic disease, or oral contraceptive pills.

A familial autosomal dominant form has also been reported. In a 2020 study, cryofibrinogenemia was detected in two-thirds of 54 patients with COVID chilblains. More studies are needed to confirm that cryofibrinogenemia is implicated in the pathogenesis of chilblains in COVID-19 patients.

Cryofibrinogenemia may be treatable and reversible, with a relatively prompt resolution of skin lesions with immunosuppressive therapy.

Codes

ICD10CM:
D89.2 – Hypergammaglobulinemia, unspecified

SNOMEDCT:
10934005 – Cryofibrinogenemia

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Updated:02/23/2022
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Cryofibrinogenemia
A medical illustration showing key findings of Cryofibrinogenemia : Acral distribution, Cryofibrinogen increased, Gangrene, Palpable purpura, Skin ulcer, Ecchymosis, Cold intolerance, Painful skin lesion
Clinical image of Cryofibrinogenemia - imageId=4477923. Click to open in gallery.  caption: 'Erythematous and purpuric papules and plaques on the distal toes of a patient with lupus.'
Erythematous and purpuric papules and plaques on the distal toes of a patient with lupus.
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