BENIGN CUTANEOUS POLYARTERITIS-NODOSA IN CHILDREN BELOW 10 YEARS OF AGE - A CLINICAL-EXPERIENCE

Objective-To report 10 children younger than 10 years of age with beni gn cutaneous polyarteritis nodosa (BCPAN). Methods-Ten children aged 1 .25-10 years (mean 4.7 years; M:F = 7:3) were admitted with an unusual vasculitis. The clinical features, laboratory investigations, treatme nt and follow up data were analysed. Results-Clinical features of thes e patients included: fever (10), peripheral gangrene (eight), livido r eticularis (four), ulceration, nodules and vesiculobullous lesions alo ne or in combination (10), black necrotic patches over limbs and trunk (three), and arthralgia or swelling of large joints (seven). Cryoglob ulinaemia was transient in three children, lasting for eight months in one of them. Histopathology of the skin lesions revealed vasculitis o f small and occasionally medium sized blood vessels in nine of the 10 children. Possible association of BCPAN was noted with diphtheria-pert ussis-tetanus immunisation (one), drugs (two), streptococcal infection (two), wasp sting (one), and falciparum malaria (one). The clinical c ourse was interspersed with remissions and exacerbations. Response to corticosteroids alone occurred in seven patients, while three children needed cytotoxic drugs in addition. In a follow up of 5.6 years (mean ) no evidence of systemic involvement was noted. Conclusions-A rare fo rm of vasculitis, BCPAN, is reported in children. The features that di stinguished our patients from those reported earlier were onset in the first decade of life and higher incidence of peripheral gangrene.