Cutaneous polyarteritis nodosa

Abstract

SUMMARYClassic polyarteritis nodosa (PAN) is a segmentary leucocytoclastic vasculitis that affects small-and medium-sized arteries. In 1931, Lindberg described the existence of a cutaneous variant of PAN, without visceral involvement and with a more favourable prognosis. We present four patients diagnosed with cutaneous PAN in our hospital between 1987 and 1998. The study group was composed of three women and one child, whose ages ranged from 11 to 70 years old. The follow-up period was between 2 and 13 years. Each patient was submitted for an initial clinical, histological and laboratory evaluation and subsequent follow-up. The presence of nodules was the most frequent cutaneous lesion, preferentially located in the lower limbs. The erythrocyte sedimentation rate was the only parameter that was altered in all patients. Cutaneous biopsies from all patients showed a segmentary leucocytoclastic vasculitis in the arteries of the deep dermis and⁄or hypodermis. Direct immunofluorescence was positive in just one patient. No visceral involvement was found in any patient. There is confusion about the correct definition of cutaneous PAN. Some clinical findings, such as nodules or livedo reticularis, typical of cutaneous PAN suggest a good prognosis; however, we consider that it is necessary to evaluate these patients for systemic involvement for the possibility of arteritis in other organs as the term polyarteritis suggests.

A case of refractory cutaneous polyarteritis nodosa in a patient with hepatitis B carrier status successfully treated with tumor necrosis factor alpha blockade

“…Most other reports suggested that HB virus might have little relation to CPAN [3,4,8,9]. In our case, elevation of transaminase or HB virus reactivation was not observed at the onset of CPAN.…”

“…Etanercept administration improved the symptoms and showed a corticosteroid sparing effect in this case. CPAN often relapses and some cases need long-term corticosteroid therapy [6,9]. But long-term corticosteroid use is well known to cause osteoporosis or compression fractures like in this case.…”

“…Díaz-Pérez et al [14] implicated immunological mechanisms in CPAN, while Bauza et al [9] suggested a relation of CPAN not to immunological mechanisms but rather infection with Streptococcus or tubercle bacillus. In this case, these possibilities could be excluded because of the presence of a low titer of ASO and negative result of tuberculin skin test.…”

“…However, the relationship between the occurrence of CPAN and HB virus has not 3 been clarified. Reported treatments of CPAN include non-steroidal anti-inflammatory drugs (NSAIDs) for mild cases, corticosteroids for cases recurring or developing extra-cutaneous symptoms [8], azathioprine, methotrexate, cyclosporine, cyclophosphamide, colchicine, dapsone, chloroquine, pentoxyfylline, mycophenolate mofetil, or intravenous immunoglobulin administration [3,9,10]. However, some cases of CPAN are resistant to such treatments and experience repeated exacerbations over prolonged periods [8,11].…”

Refractory cutaneous polyarteritis nodosa with threat of loss of feet: successful treatment with rituximab

“…The true incidence is unknown [3] it has been estimated to be <3% of all cutaneous vasculitides [4]. CPAN is characterized as a "benign, chronic, relapsing vasculitis" [5] requiring conservative management with NSAIDs, low dose steroids, and possibly met otrexate; as opposed to, the malignant course of systemic PAN which necessitates aggressive therapy with high dose steroids and immunosuppressive agents [1]. However, CPAN has been reported to cause distal extremity necrosis resulting in amputation [6,7].…”