Korean J Dermatol.  2007 Dec;45(12):1298-1302.

A Case of Microscopic Polyangiitis with Acral Necrosis as Skin Manifestation

• Shin MK
• Hong KK
• Lew BL
• Kim NI

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.

Keyword

Acral necrosis; Microscopic polyangiitis; P-ANCA

MeSH Terms

Antibodies, Antineutrophil Cytoplasmic
Arthralgia
Enzyme-Linked Immunosorbent Assay
Erythema
Fingers
Gangrene
Glomerulonephritis
Granuloma
Hemoptysis
Humans
Korea
Leg
Microscopic Polyangiitis*
Middle Aged
Necrosis*
Prognosis
Proteinuria
Rare Diseases
Skin Manifestations*
Skin*
Systemic Vasculitis
Toes
Vasculitis
Antibodies, Antineutrophil Cytoplasmic