Cutaneous Polyarteritis Nodosa in a Child with Positive Antiphospholipid and P-ANCA

Abstract

A case of juvenile cutaneous polyarteritis nodosa (cutaneous PAN) is presented. Since early infancy the child underwent attacks of fever and cutaneous rash that ocasionally progressed to gangrene and amputations of distal portions of toes and fingers. Although occasional episodes of high blood pressure and persistence of moderate eosinophilia were present, the clinical pattern was mostly restricted to the musculoskeletal system and skin. The authors discuss the definition of the disease and its present therapeutic possibilities, calling attention to a feature not referred in previous reports: the unique co-existence of cutaneous PAN plus antiphospholipid antibodies (aPL) and perinuclear antineutrophil cytoplasmic antibodies (p-ANCA).

Key Words:

• cutaneous polyarteritis nodosa
• childhood vasculitis
• perinuclear antineutrophil cytoplasmic antibodies
• antiphospholipid antibodies