Spontaneous necrosis of the skin associated with cryofibrinogenemia, cryoglobulinemia, and homocystinuria.

Autor: Williamson AE; Department of Surgery, Eisenhower Memorial Hospital, Rancho Mirage, Calif., USA., Cone LA, Huard GS 2nd
Jazyk: angličtina
Zdroj: Annals of vascular surgery [Ann Vasc Surg] 1996 Jul; Vol. 10 (4), pp. 365-9.
DOI: 10.1007/BF02286781
Abstrakt: Cryofibrinogenemia has been associated with a variety of skin manifestations including purpura, livedo reticularis, and ulceration. Our patient, who had undergone axillobifemoral bypass 5 years previously, presented following the spontaneous development of a necrotic wound involving the left groin scar. The location of the wound suggested the possibility of underlying graft infection, but indium-111 white blood cell scan and MRI failed to show any evidence of infection. The patient was initially treated with oral antibiotics and outpatient debridement with no improvement. A more aggressive approach with inpatient operative debridement and intravenous antibiotics produced moderate improvement. Three months later, the patient developed an identical necrotic wound in the right groin and subsequently a third lesion involving a scar distant from any of the patient's grafts. No evidence of active vasculitis was seen on microscopic examination of the excised tissues. Cryoglobulin and cryofibrinogen assays were positive, and urinary and plasma homocysteine levels were elevated. The patient was subsequently treated with stanozolol, a low-methionine diet, and outpatient intravenous antibiotics with rapid improvement of her wounds. In patients with spontaneous ulceration of the extremities, particularly when they do not respond appropriately to standard therapy, the possibility of cryoglobulinemia or cryofibrinogenemia should be considered.
Databáze: MEDLINE