| Abstract: | Eosionophilic fasciitis (EF) was observed in a girl who suddenly developed indurations of the limbs accompanied by hypergammaglobulinaemia and peripheral eosinophilia. The deep fascia was impressively thickened and infiltrated with numerous inflammatory cells showing focal accumulations of eosinophils. The disease was progressive in spite of application of corticosteroids, and within three years, symmetrical sclerodermatous lesions developed on the face and trunk, going along with a severe sclerodactyly without Raynaud's phenomenon, as well as contractures of the upper and lower limbs causing complete disability. Visceral involvement was slight (decreased motility of the esophagus and restriction of pulmonal function); slight calcium deposits were disclosed at the soft tissue of the ankles; and nuclear antibodies of the speckled type appeared in a titer up to 320. The fascia was still the most involved tissue, but there was no peripheral or tissue eosinophilia. EF seems to be a special variant within the broad spectrum of scleroderma; and although it usually has a benign course, it may change into an unclassified variety of scleroderma and lead to severe disability. |
