Sickle Cell Ulcer in Hand Attributable to Arterio‐Venous Fistula

Introduction:  Sickle cell ulcers are a frequent dermatologic manifestation of sickle cell disease. They are almost exclusively described in the legs near the malleoli and are often bilateral. Such ulcers in the upper extremities are not described. We report a case of sickle cell ulcer in hand presumably related to arterio-venous fistula in the forearm placed for hemodialysis in a patient with chronic kidney disease. Case:  A 45-year-old African-American man with a history of sickle cell disease, hypertension, and kidney failure treated with hemodialysis presented with a painful wound in his left hand near the base of his thumb, which had been present for 6 weeks prior to presentation. There was no history of trauma. 6 months prior to the development of the ulcer, the patient had an arterio-venous fistula placed in the affected hand. The ulcer did not improve with use of antibiotics. Venous ultrasound of the left upper extremity showed occlusion of the cephalic vein and enlargement of the basilic vein. Subsequently, biopsy of the ulcer showed vascular proliferation with thickening of the vessel and obliteration of the vascular lumen. The blood vessels were plugged with sickled red blood cells, characteristic of sickle cell disease. Histological features of the lesion were indicative of vaso-occlusive disease, suggesting sickle cell ulcer. Patient was treated with surgical debridement and skin grafting after hyperbaric oxygen therapy that only resulted in partial recovery of the lesion. Discussion:  The pathogenesis of sickle cell ulcer is presumed to be related to a vaso-occlusive process, leading to tissue necrosis resulting in skin ulcers. Ulcers can occur spontaneously or with local trauma. Low oxygen-carrying capacity of the blood, impaired venous hemodynamics, and low hemoglobin are recognized contributing factors. The arterio-venous fistula causing diversion of arterial blood probably contributed to the development of sickle cell ulcer. Conclusion:  Sickle cell ulcers in the hand, although extremely rare, can occur in sickle cell patients with chronic kidney disease on hemodialysis with arterio-venous fistula. This results from the altered local vascular hemodynamics that predispose to vaso-occlusive conditions, eventually leading to ulceration.