A Case of Microscopic Polyangiitis with Acral Necrosis as Skin Manifestation / 대한피부과학회지

ABSTRACT

Microscopic polyangiitis (MPA) is a systemic vasculitis that is histologically characterized by small vessel involvement. Although MPA is primarily associated with necrotizing and crescentric glomerulonephritis and pulmonary capillaritis, MPA often has cutaneous features. We report a case of microscopic polyangiitis with acral necrosis as the skin manifestation. A 56-year-old man presented with acral dry gangrene on tip of finger and toe and reticulated erythema on leg. He had suffered from proteinuria, hemoptysis, and arthralgia. Histopathologically, small vessel vasculitis without granuloma was seen. A high serum level of pANCA was detected by ELISA. This is a relatively rare disease, which has a poor prognosis in the absence of aggressive therapy, and is infrequently reported in dermatologic journals in Korea.