[Shulman's syndrome: eosinophilic fasciitis (author's transl)]. 1979

J Thivolet, and R Jeune, and M Faure, and C Hermier, and F Michel

A detailed report is made of the clinical, histological, biological manifestations of eosinophilic fasciitis, i. e. the Shulman's syndrome, about a 53-year-old man. An extreme induration of sub-cutaneous tissues from arms, legs and trunk, without involvement of the face and extremities, was associated with severe thickening of deep peri-muscular fascias. Raynaud's phenomenon was absent, as were morpheas and visceral involvement. Results of biopsies studied by standard, electron and I. F. microscopy, revealed sclerosis and cellular infiltrates (lymphocytes, plasma cells, histiocytes and eosinophils) in fascia and muscular septa; no changes were seen in epidermis, dermis or sub-cutaneous fat tissue. An elevated ESR, eosinophilia and hyperimmunoglobulinaemia with high levels of circulating immune complexes were the only biological abnormalities. A good response to systemic corticosteroid therapy was observed. These features were similar to those seen in other cases of eosinophilic fasciitis. The etiology and pathogenesis of the Shulman's syndrome remain unclear. A critical review of the literature suggests that eosinophilic fasciitis should be separated from scleroderma and pseudoscleroderma, although this opinion has been discussed.

UI MeSH Term Description Entries
D007249 Inflammation A pathological process characterized by injury or destruction of tissues caused by a variety of cytologic and chemical reactions. It is usually manifested by typical signs of pain, heat, redness, swelling, and loss of function. Innate Inflammatory Response,Inflammations,Inflammatory Response, Innate,Innate Inflammatory Responses
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D004804 Eosinophils Granular leukocytes with a nucleus that usually has two lobes connected by a slender thread of chromatin, and cytoplasm containing coarse, round granules that are uniform in size and stainable by eosin. Eosinophil
D005205 Fascia Layers of connective tissue of variable thickness. The superficial fascia is found immediately below the skin; the deep fascia invests MUSCLES, nerves, and other organs.
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000305 Adrenal Cortex Hormones HORMONES produced by the ADRENAL CORTEX, including both steroid and peptide hormones. The major hormones produced are HYDROCORTISONE and ALDOSTERONE. Adrenal Cortex Hormone,Corticoid,Corticoids,Corticosteroid,Corticosteroids,Cortex Hormone, Adrenal,Hormone, Adrenal Cortex,Hormones, Adrenal Cortex
D012595 Scleroderma, Systemic A chronic multi-system disorder of CONNECTIVE TISSUE. It is characterized by SCLEROSIS in the SKIN, the LUNGS, the HEART, the GASTROINTESTINAL TRACT, the KIDNEYS, and the MUSCULOSKELETAL SYSTEM. Other important features include diseased small BLOOD VESSELS and AUTOANTIBODIES. The disorder is named for its most prominent feature (hard skin), and classified into subsets by the extent of skin thickening: LIMITED SCLERODERMA and DIFFUSE SCLERODERMA. Sclerosis, Systemic,Systemic Scleroderma,Systemic Sclerosis
D013577 Syndrome A characteristic symptom complex. Symptom Cluster,Cluster, Symptom,Clusters, Symptom,Symptom Clusters,Syndromes

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