Digital infarct and mononeuritis in a middle aged female: always suspect Antineutrophil cytoplasmic antibodies associated vasculitis

Vasculitis is a process caused by inflammation of blood vessel walls and results in a variety of disorders. Small-vessel vasculitis (vasculitis involving arteries, venules and capillaries) should be suspected in any patient who presents with a multisystem disease that is not caused by an infectious or malignant process. Testing for Antineutrophil cytoplasmic antibody (ANCA) is the basis of classification of small vessel vasculitis into ANCA associated and non - ANCA associated vasculitis. Apart from cutaneous manifestations like palpable purpura and vasculitic urticaria, digital gangrene in a patient with evidence of mononeuritis multiplex is highly suggestive of ANCA associated vasculitis (AAV). Clinically most of these vasculitides have overlapping clinical presentations and similar treatment. Early diagnosis and rapid initiation of treatment of AAV is recommended rather than ordering for definitive tests (e.g. histopathology or angiograms) since delay in treatment can result in serious end organ damage (pulmonary or renal).

A Case of Systemic Lupus Erythematosus Diagnosed by Initial Presentation of Digital Gangrene / 대한내과학회지

Although digital gangrene is an uncommon clinical feature, it may lead to serious complications, such as amputation. Therefore, it requires prompt evaluation and treatment. Digital gangrene is often seen in systemic sclerosis, but is rare in systemic lupus erythematosus (SLE). In SLE, digital gangrene results from vasculitis, vasospasm, and thromboembolism. Here, we report a 15-year-old male SLE patient who initially presented with digital gangrene, and present a review of the relevant literature.

A Case of Primary Antiphospholipid Syndrome Presenting as Digital Gangrene / 대한류마티스학회지

Antiphospholipid syndrome (APS) is characterized by a combination of arterial/venous thrombosis or obstetric morbidity and antiphospholipid antibodies such as anticardiolipin antibody or lupus anticoagulant. Digital gangrene is an uncommon manifestation of primary APS. A 61 year-old woman was admitted because of acrocyanosis and progressive digital gangrene in both hands for 1 month. Serologically, lupus anticoagulant was positive. Angiography showed obstruction of digital arteries with collaterals, but there was no evidence of atheromatous plaque or vasculitis. Cardiac echogram did not reveal intracardiac thrombosis or valvular vegetations. She had no evidence of other thrombotic disorders, connective tissue disease, infections or malignancy. Treatment of intravenous heparin and prostaglandin E1 led to stabilization of the symptom, and the necrotic digits were surgically removed. Pathological examination of the amputed digital stump showed arterial and arteriolar thrombotic occlusion, proliferation of capillaries, and ischemic necrosis. After oral anticoagulation therapy, she has been followed without recurrence.We describe the first case of primary APS presenting as digital gangrene in Korea and review the literatures.