Twenty-three patients with cutaneous polyarteritis nodosa (C-PAN)—do rheumatologists see a more severe form of the disease?

ObjectiveTo describe the characteristic features of ulcerative subset of cutaneous polyarteritis nodosa (C-PAN) and its response to therapeutic interventions.MethodsTwenty-three biopsy proven cases of C-PAN were treated and followed up for one year or more.ResultsA median follow-up of 2 years (range 1 to 16 years) was obtained. Median age and disease duration at recruitment were 35 years and 5 years, respectively. All patients showed irregular chronic waxing and waning, painful and tender nodulo-ulcerative and/or vesiculobullous lesions on the lower leg, periankle area, and/or dorsum of feet. The lesions were bilateral but asymmetrical in severity. Objective joint disease was seen in approximately one-third of patients, mainly in the ankle and occasionally in the joints of the mid-foot with severe destructive disease in 2 patients. Significant neuralgic pains, neuropathy or mononeuritis multiplex were seen in approximately 40% of the patients. Systemic disease with constitutional symptoms and organ involvement was not seen in any patient. Raised erythrocyte sedimentation rate was seen in approximately 40% of the patients. Other laboratory investigations were unremarkable. Different modalities of treatment gave disappointing results; only about 20% patients achieved complete remission and could be successfully weaned off all medicines. A majority of patients continued to follow a fluctuating course with healing and reappearance of ulcerative lesions and gradually progressive scarring. Severe aggressive scarring and disfigurement was seen in one patient who was completely unresponsive to any form of treatment.ConclusionThis study confirms that the ulcerative subset of C-PAN is a more aggressive disease with unsatisfactory response to standard therapeutic interventions.