Faces of eosinophilic fasciitis in childhood

Ana I. Quintero-Del-Rio, Marilyn Punaro, Virginia Pascual

Research output: Contribution to journalArticle

15 Scopus citations

Abstract

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 uniphasic 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.

Original languageEnglish (US)
Pages (from-to)99-103
Number of pages5
JournalJournal of Clinical Rheumatology
Volume8
Issue number2
Publication statusPublished - 2002

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Keywords

  • Aldolase
  • Eosinophilia
  • Hepatosplenomegaly
  • Muscle weakness
  • Prednisone
  • Raynaud's phenomenon

ASJC Scopus subject areas

  • Rheumatology

Cite this

Quintero-Del-Rio, A. I., Punaro, M., & Pascual, V. (2002). Faces of eosinophilic fasciitis in childhood. Journal of Clinical Rheumatology, 8(2), 99-103.