Chronic recurrent multifocal osteomyelitis and pustulosis palmoplantaris

Abstract

Based on nine patients 4 to 26 years of age, we describe the clinical characteristics of chronic recurrent multifocal osteomyelitis. This disorder was characterized by an insidious onset of fever, local swelling and pain in affected bones, and radiologic findings suggesting osteomyelitis. The lesions were mainly localized to the clavicles and the metaphyses of tubular bones. The clinical course was characterized by intermittent periods of exacerbation and improvement over several years. Six of the patients had recurrent pustulosis palmaris and plantaris, which closely parallelled the exacerbations of the bone lesions. Biopsies from the bone lesions showed nonspecific inflammatory changes with granulocytic infiltration. Repeated bacterial and fungal cultivations from blood, bone biopsies, and pustules were negative. Immunologic investigation revealed no abnormality common to the patients. There was no indications of a genetic etiology. The pathogenesis of the disorder is unknown. Antibiotic treatment had no obvious effect but corticosteroid therapy appeared to be of benefit in some patients. The long-term prognosis appears to be relatively good.