Panarteritis cutanea benigna – an entity limited to the skin or cutaneous presentation of a systemic necrotizing vasculitis? Report of seven cases and review of the literature

Abstract

In 1931 Lindberg described a limited and benign subcutaneous form of panarteritis nodosa, which, in contrast to systemic panarteritis, only affects the skin. The terms panarteritis nodosa cutanea benigna, cutaneous polyarteritis nodosa, apoplexia cutanea Freund as well as livedo with nodules are used synonymously for this vasculitis which predominantly affects women in the fifth decade of life. Cutaneous lesions characteristically comprise painful subcutaneous nodules or vasculitis racemosa at the lower extremities. The cutaneous panarteritis may be regarded as its own entity or an isolated skin manifestation within systemic panarteritis nodosa. Seven patients (M=3, F=4) presented with subcutaneous, painful nodules (n=2), erythema and edema (n=2) or livedo racemosa (n=3) on the lower extremities. Histological examination revealed perivascular lymphocyte inflammatory infiltrates with fibrinoid necrosis and bulging of endothelial cells. In a direct immunofluorescence examination neither immunoglobulin nor complement deposits could be found. Serological autoimmune parameters, abdominal ultrasound examination, and chest X-rays showed no systemic manifestation. Erythrocyte sedimentation rate was slightly raised, and hepatitis B/C serology was negative. Topical corticosteroids under occlusion (n=3), oral methyl-prednisolone (n=4) in combination with either azathioprine (n=4) or mycophenolate mofetil (n=2) showed no relapses [follow up 28.43 (6-82) months]. The etiology of panarteritis nodosa cutanea is unknown in detail. Both focal infections and hypersensitivity reactions are discussed. Differential diagnosis includes systemic panarteritis, livedo reticularis, Sneddon's syndrome or erythema nodosum. Despite the prognostically favorable but prolonged chronic course of the disease over decades, patients should be regularly examined to exclude possible transition to systemic disease.