Cutaneous polyarteritis nodosa in children: three cases

INTRODUCTION: Polyarteritis nodosa is a necrotizing vasculitis of small and medium-size arteries. The cutaneous form of polyarteritis nodosa follows a chronic course, characterized by recurrent episodes limited to skin, muscles and joints. This entity differs from systemic polyarteritis nodosa in the absence of visceral involvement. This form is rare in children, we describe three cases. CASE REPORTS: We describe three girls with a mean age of 11 years (range: 8-13). They presented painful subcutaneous edematous nodules, arthralgia and fever. Physical examination revealed livedo reticularis (2 cases) and pharyngeal infection (1 case). Laboratory findings showed an inflammatory syndrome. Skin biopsy supported diagnosis of polyarteritis nodosa. The course was characterized by periods of remission disrupted by exacerbations, well controlled by salicylotherapy, colchicine, dapsone or penicillin. Corticosteroid therapy was used only for invalidating symptoms. There was no systemic involvement after 2, 5 and 6 years of follow up. DISCUSSION: Cutaneous polyarteritis nodosa in children must be suspected in presence of fever, subcutaneous nodules, livedo reticularis and arthralgia. Prognosis is usually benign, so we recommend no aggressive treatment. In view of the tendency to relapse, long-term follow-up is appropriate, before confirming diagnosis.