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

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.

Claudication in an adolescent with a hypoplastic femur.

The most common cause of claudication is atherosclerosis obliterans. However, when it presents in adolescence, other causes should be considered. We describe the case of a 15-year-old girl who had severe intermittent claudication 8 years after a limb-lengthening procedure for a hypoplastic femur. The lesion responsible was an isolated fibromuscular dysplastic segment of the distal superficial femoral artery and proximal popliteal artery. The etiology, treatment, and histopathology are discussed.

Effective streptozocin therapy for metastatic pancreatic polypeptide apudoma.

An apparently complete remission of metastases in the liver from a resected pure pancreatic polypeptide apudoma occurred following intra-arterial and intravenous administration of streptozocin to a patient in a family with multiple endocrine adenopathy, type 1. Plasma concentrations of human pancreatic polypeptide constitute an excellent marker system for the detection and assessment of tumor status before and after surgical and streptozocin therapy.