Cutaneous polyarteritis nodosa: therapy and clinical course in four cases

Cutaneous polyarteritis nodosa (PN) has a benign and chronic course; relapses are frequently associated with steroid dependence. We have observed four cases of cutaneous PN in the past 15 years and followed up two of the four cases long-term for 13 and 10 years after diagnosis. There has been a marked contrast in the clinical courses of these two cases: one case has shown a complete remission for 12.5 years without treatment during the most recent 11 years; the other case had four relapses and has never experienced cessation of treatment. The only difference between the two cases was careful therapy with adequate prednisolone in the long-term remission case. The other two cases clinically showed erythema nodosum-like features, and they had antecedent sore throats and embedded chronic tonsillitis; one was associated with presumed streptococcal infection. These two cases may simply be an accelerated process of post-streptococcal erythema nodosum rather than typical cutaneous PN. We performed tonsillectomies as adjuvant therapy in these two cases. No relapse of the disease has been observed in these two cases, and the tonsillectomy allowed us to taper the dose of steroids, resulting in discontinuation of the treatment in one of the two cases. The duration of the remission as well as the adjuvant therapy was variable in each of our cutaneous PN cases. Tonsillectomy can be recommended as an adjuvant to steroids for PN cases with chronic tonsillitis and/or streptococcal infection.