Eosinophilic fasciitis is a rare
connective tissue disease in children characterized by hardening and thickening of the skin and soft tissues, peripheral
eosinophilia, elevated erythrocyte sedimentation rate, and
hypergammaglobulinemia. In this study, we report three pediatric patients with
eosinophilic fasciitis whose clinical presentation and response to
therapy differed from those reported in the literature. All three patients were female. Two of them had Raynaud's phenomenon as an initial feature, and the third had hepatosplenomegaly. Muscle involvement with weakness was present in two patients, as documented not only by increased
aldolase levels at the time of presentation but also by inflammatory changes in the muscle biopsy. Serum
immunoglobulin G levels were increased only in the most severely involved patient. Peripheral
eosinophilia was present in all three cases. All three patients responded well to
prednisone therapy. Two patients had a uni-phasic course, and one required further
therapy to control a postinfection relapse. In summary, we found that Raynaud's phenomenon and hepatosplenomegaly can be part of the spectrum of clinical manifestations of childhood
eosinophilic fasciitis. Identification as
eosinophilic fasciitis and not scleroderma, despite hand involvement and Raynaud's phenomenon, can suggest that the illness may be
steroid responsive.