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Lysosomal-associated membrane protein-2 (LAMP-2) is a target antigen for anti-neutrophil cytoplasmic anti-bodies (ANCAs), which are closely linked to a subset of primary systemic vasculitides. Cutaneous polyarteritis nodosa (CPN) is a necrotizing vasculitis of small to medium-sized arteries within the skin. We measured levels of serum anti-LAMP-2 antibody in 50 patients with CPN, 8 with microscopic polyangiitis (MPA), and 34 healthy persons. We also investigated the presence of ANCA in patients with CPN using indirect immunofluorescence 0IF), a direct ELISA and a capture ELISA specific for myeloperoxidase (MPO) and proteinase 3 (PR3). Serum anti-LAMP-2 antibody levels differed significantly between patients with CPN (0.263 U/ml) and those with MPA (0.180 U/ml) (p = 0.0102). Serum of all patients with CPN was negative for MPO-ANCA and PR3-ANCA by both direct ELISA and capture ELISA. In contrast, IIF assay revealed ANCA in 42 (84.0%) of the 50 CPN patients. Serum anti-LAMP-2 antibody levels in the perinuclear ANCA (P-ANCA) group were significantly elevated compared with the non-ANCA group (p=0.0147). We suggest that anti-LAMP-2 antibody could play an important role in the pathogenesis of CPN in the presence of P-ANCA detected by IIF. [ABSTRACT FROM AUTHOR]

Microscopic polyangiitis is a systemic small vessel vasculitis, which often has cutaneous and musculoskeletal features. Microscopic polyangiitis is a member of the family of anti-neutrophil cytoplasmic auto-antibody (ANCA)-associated vasculitides and is strongly associated with anti-myeloperoxidase (MPO)-ANCA. Titres of MPO-ANCA may reflect disease activity and play a pathogenic role. Patients with microscopic polyangiitis usually present with erythematous macules on the extremities as the first cutaneous manifestation. Skin biopsy specimens from the erythema reveal small-sized vessels that are infiltrated with neutrophils, consistent with leukocytoclastic vasculitis, in the deep dermis to the subcutaneous fat tissue. The cutaneous involvement is present at an early stage of microscopic polyangiitis with other non-specific symptoms, such as arthralgias and myalgias. The initial cutaneous manifestations are important in early diagnosis of possible ANCA-associated vasculitides with elevated ANCA titres. [ABSTRACT FROM AUTHOR]

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