Cryofibrinogenemia-Induced Cutaneous Ulcers: A Review and Diagnostic Criteria

• Published in: American journal of clinical dermatology Vol. 18; no. 1; pp. 97 - 104
• Main Authors: Grada, Ayman, Falanga, Vincent
• Format: Journal Article
• Language: English
• Published: Cham Springer International Publishing 01.02.2017; Springer Nature B.V
• Subjects: Animals; Cancer; Chronic Disease; Cryoglobulinemia - complications; Cryoglobulinemia - diagnosis; Cryoglobulinemia - pathology; Dermatology; Disease; Humans; Hyperpigmentation - etiology; Hypotheses; Immunoglobulins; Immunology; Medical diagnosis; Medicine; Medicine & Public Health; Pharmacology/Toxicology; Pharmacotherapy; Review Article; Skin; Skin Ulcer - diagnosis; Skin Ulcer - etiology; Skin Ulcer - pathology; Steroids; Ulcers; Wound Healing; Skin Ulcer; Cryoglobulinemia; Streptokinase; Anabolic Steroid; Danazol
• ISSN: 1175-0561; 1179-1888
• DOI: 10.1007/s40257-016-0228-y

Chronic skin ulcers are frequently encountered in clinical practice and are often due to very heterogeneous etiologies. Cryofibrinogenemia is an unusual cause of non-healing skin ulcers. It is a small-vessel occlusive vascular disorder that results from the precipitation of cryofibrinogens in plasma. The lack of definitive diagnostic criteria means cryofibrinogenemia remains an under-diagnosed entity that causes significant morbidity. One of the most common manifestations of cryofibrinogenemia is skin ulceration. The presence of non-healing ulcers in otherwise healthy patients with no evidence of large-vessel disease should raise the suspicion of essential cryofibrinogenemia. An important clinical feature is the presence of microlivedo, which represents short hyperpigmented linear streaks around the ulcer or even distally about the foot. Histopathologic findings are microthrombi in the dermis and not confined exclusively to the ulcerated area. Cryofibrinogenemia can be secondary to an underlying disorder, so careful investigation to exclude other etiologies is always necessary.