Cutaneous periarteritis nodosa. Diagnosis and treatment in 9 cases

Background. Cutaneous periarteritis nodosa (PAN) is distinguished from syst emic PAN by the tack of visceral involvement. The aim of this study was to describe the clinical presentation, laboratory findings, clinical course, a nd treatment in cutaneous PAN. Patients and methods. We retrospectively reviewed the files of patients hos pitalized for vasculitis in our Dermatology unit where approximately 20 cas es of vasculitis are seen each year, inclusion criteria were skin signs sug gestive of PAN and a histological image of leukocytoclastic vasculitis of a n arteriole. Results. Nine cases of cutaneous PAN were treated in our unit between 1976 and 1997 Follow-up ranged from 32 months to 22 years. No cases of systemic PAN had been diagnosed during this period. These 9 cases of cutaneous PAN a ll had the same clinical presentation: nodules on the lower limbs in all ca ses associated with nodules on the upper limbs in half of the cases. Neurop athy was found in 3 of the 9 cases. No systemic involvement was observed. T he most frequently used treatment protocol was general corticosteroid thera py (0.5 mg/kg/d prednisone or prednisolone). Immunosuppressive drugs, colch icine, dapsone, non-steroidal anti-inflammatory drugs and intravenous immun oglobulins were also used with efficacy. Discussion. Cutaneous PAN is a particular form of vasculitis associating sk in signs with locoregional neuromuscular involvement. The differential diag nosis with other types of vasculitis is sometimes a difficult task. The cli nical course is the fundamental diagnostic clue in cutaneous PAN. A benign course and the absence of visceral involvement allow initiating a symptomat ic treatment such as colchicine. The development of neuromuscular signs may warrant the use of general corticosteroid therapy.