Eosinophilic fasciitis is a recently described rheumatic disease, some 20 cases having been reported in abstract form. Previous descriptions have stressed the localized nature of skin involvement, the absence of visceral changes or Raynaud's phenomenon, an association with hypergammaglobulinemia and eosinophilia, and a good response to corticosteroid therapy. The most conspicuous feature of this entity has been a massive thickening of the subcutaneous fascia, when an adequate (skin down to muscle) biopsy was performed. The skin biopsy in the case presented showed this thickening together with collagenous hypertrophy and an intense infiltration of lymphocytes and plasma cells. Cellular infiltration was most prominent in a perivascular distribution; eosinophils were not a prominent feature. Immunofluoresent studies, with fluorescein conjugated anti IgG, IgM, IgA, and C3 showed only a nonspecific staining of collagen bundles and a scattered cytoplasmic staining for IgG (assumed to be in plasma cells). Another case is reported conforming to the general features, with the exception that Raynaud's phenomenon was a prominent symptom. A critical review of the literature suggests tht eosinophilic fasciitis should tentatively be regarded as a variant of scleroderma.
ASJC Scopus subject areas
- Immunology and Allergy
- Biochemistry, Genetics and Molecular Biology(all)