Eosinophilic fasciitis: An early variant of scleroderma

Jarratt, Michael; Bybee, J. David; Ramsdell, William M.

doi:10.1016/s0190-9622(79)70013-2

Cited by 35 publications

(4 citation statements)

References 17 publications

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“…The positive Congo Red stain in the synovium of patient 1, consistent with amyloid deposition, appears to be unique among reported cases of eosinophilic fasciitis, to the best of our knowledge. In addition, we and others have noted hematologic abnormalities, pulmonary function abnormalities, colonic diverticulae, and other systemic manifestations (18,19). Pulmonary function abnormalities noted in our two patients may represent nonspecific findings, but are in accord with pulmonary involvement noted by others.…”

Section: Discussionsupporting

confidence: 87%

Eosinophilic fasciitis

1981

Arthritis & Rheumatism

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Two patients with clinical and pathologic features of eosinophilic fasciitis manifested serologic and systemic abnormalities that raised the question of the fundamental nature and relationship of eosinophilic fasciitis to scleroderma. In addition to the characteristic features of eosinophilic fasciitis, both patients exhibited arthritis, a predominantly mononuclear cell infiltration of muscles with normal serum muscle enzyme levels, weakly positive serum antinuclear factor, IgA deficiency, and abnormalities of pulmonary function. In addition, one patient had wide-mouthed colonic diverticulae and synovial deposits consistent with amyloid; the second patient had bone marrow hypoplasia. Although corticosteroid therapy was of benefit, hydroxychloroquine and potassium para-aminobenzoate were of further help in controlling the disorder. Biopsies from the two patients revealed inflammatory lesions to be heaviest deep in the skeletal muscle; fascia was only minimally inflamed with mild fibrosis. The findings suggest that striking fibroinflammatory lesions noted in the fascia in some patients with eosinophilic fasciitis may derive largely from spillover of lesions in neighboring skeletal muscle. Shulman recently described a scleroderma-like disease characterized clinically by thickening of the skin and pain and swelling of the distal extremities, often with rapid development of contractures (1,2). Unlike scleroderma, however, the onset was relatively rapid and followed episodes of unusual physical exertion. Furthermore, there was striking peripheral blood eosinophilia. Raynaud's phenomenon, telangiectasia, myositis, and extracutaneous manifestations were absent, as were various serologic markers including antinuclear antibodies and antibodies to the extractable nuclear antigen. Most importantly, the disease usually responded well to steroid therapy. Pathologically, the syndrome apparently differed from scleroderma in that the fibroinflammatory lesions showed a predilection for fascia, as opposed to subcutis and dermis. Patients with similar features were soon thereafter reported by others, and it was suggested that this was a clinicopathologically distinct connective tissue disease for which the name "Eosinophilic fasciitis" was proposed (3).As clinical and pathologic experience with this syndrome accumulated, the distinction between scleroderma and eosinophilic fasciitis became somewhat blurred. Much discussion has ensued as to the exact features which define this entity and differentiate it from scleroderma (43). We have recently seen two patients whose clinical and pathologic features warrant classification of their disease process as eosinophilic fasciitis. Clinical scrutiny and correlative pathologic examination, including ultrastructural study, provide some insights into the relationship of eosinophilic fasciitis and scleroderma. This report addresses the clinical, immunologic, and histologic aspects. The immunohistochemical and ultrastructural findings and the pathogenesis are discussed in detail in a separate rep...

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Section: Discussionsupporting

confidence: 87%

Eosinophilic fasciitis

1981

Arthritis & Rheumatism

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“…Eosinophilic fasciitis (EF), Shulman's syndrome, is a rare disease that is classified by some authors to scleroderma-like syndromes [1][2][3]. Its position in the classification of rheumatic diseases has not been established yet, as concerns the disease entity, syndrome or variant of scleroderma [4][5][6][7]. Most authors [8][9][10] agree that the EF forms a separate disease entity.…”

Section: Introductionmentioning

confidence: 99%

Fasciitis eosinophilica: personal observations and a review of the literature

Bobrowska-Snarska¹,

Ostanek²,

Brzosko³

2007

Polish Archives of Internal Medicine

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Eosinophilic fasciitis is a rare disease that is classified by some authors to scleroderma-like syndromes. Symmetrical induration of skin and subcutaneous tissue associated by eosinophilia in peripheral blood are characteristic features of the disease. Internal organ involvement is uncommon. It is often difficult to diagnose eosinophilic fasciitis and its course may be variable. Glucocorticosteroids are most commonly used in the treatment but in many cases they are ineffective. Then other immunosuppressive therapy must be considered. Prognosis is rather favorable. The remission is not always achieved and sometimes flares of the disease are observed as evidenced by the described cases. It should be emphasized that a majority of our patients were females. In four out of five patients anti-thyreoglobulin antibodies and/or anti-thyroid peroxidase antibodies were present suggesting their involvement in the pathogenesis of eosinophilic fasciitis. Neither indicators of inflammation nor peripheral blood eosinophilia were pathognomonic. Results of glucocorticosteroid treatment were satisfactory in three patients, but two patients required combined immunosuppressive treatment.

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“…Recently the direct transformation of previously DFE-like appearing lesions into PSS within three months has been described (7). Accordingly late occurring interna( involvement cannot be ruled out in other cases (8). There are, however two fäets that distinguish DFE from PSS: the good prognosis in most of the reported patients without involvement of interna!…”

Section: Discussionmentioning

confidence: 99%

Diffuse fasciitis with eosinophilia associated with morphea and lichen sclerosus et atrophicus

Mensing,

Schmidt

1985

Acta Derm Venereol

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Diffuse fasciitis with eosinophilia (DFE) is a well described syndrome, among the connective tissue disorders. However, DFE is not commonly accepted as an own entity, because of its rare evolvement in scleroderma and the often indistinguishable histological pattern of both diseases. Here we report on the association of DFE and morphea in two patients and an additional lichen sclerosus in one of them. This points to a close relationship of DFE and other connective tissue diseases.

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Eosinophilic fasciitis: An early variant of scleroderma

Cited by 35 publications

References 17 publications

Eosinophilic fasciitis

Eosinophilic fasciitis

Fasciitis eosinophilica: personal observations and a review of the literature

Diffuse fasciitis with eosinophilia associated with morphea and lichen sclerosus et atrophicus

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