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(A) Tender, brownish induration on both forearms. (B) A deep tissue biopsy specimen from the lesion shows chronic inflammation with fibrosis (H&E, ×25). (C) The fascia is fibrotic with mixed inflammatory cell infiltration (H&E, ×100). (D) Mixed inflammatory cell infiltration composed of lymphohistiocytes and plasma cells (H&E, ×400).
Source publication
Eosinophic fasciitis (EF) is an uncommon connective tissue disease characterized by scleroderma-like cutaneous changes, peripheral eosinophilia, hypergammaglobulinemia, and an elevated erythrocyte sedimentation rate (ESR). Typical histopathologic findings include chronic inflammatory infiltration affecting the deep fascia with lymphocytes, histiocy...
Contexts in source publication
Context 1
... 36-year-old man presented with a 2-month history of brownish tender induration on both forearms (Fig. 1A). He had experienced immobility of both wrists. There was no history of sclerodactyly, Raynaud's phenomenon, dyspha- gia, dyspnea nor L-tryptophan ingestion. Chest X-ray was unremarkable. Laboratory investigations revealed white blood cell count at 6,800/mm 3 , with 8.1% eosinophils. ESR, liver, renal and thyroid function tests were ...
Context 2
... Serology was traced for anti-nuclear antibody with mixed pattern of speckled and anticytoplasmic antibody, and was negative for anti-ds DNA antibody, anti-Scl-70 antibody, anti-centromere antibody and rheumatoid factor. Deep tissue biopsy demonstrated fibrotic fascia with mixed infiltrate composed of lymphocytes, histiocytes, and plasma cells (Fig. 1B∼D). EF was diagnosed based on typical skin sclerosis, hist- opathology results and eosinophilia. He was treated with 60 mg prednisolone daily. Within 1 week, the induration softened and he experienced mildly improved joint mobility. Prednisolone dosage was reduced progressively until complete remission was achieved 2 months follow- ing ...
Citations
... Currently, there is no agreed-upon therapy or treatment for EF. Systemic steroid therapy is usually initial treatment of choice and is effective in 70% cases [9][10][11][12]. Other treatment options included D-pencillamine, cyclosporine, azathioprine, chloroquine and infliximab. ...
Eosinophilic fasciitis (EF) is an uncommon localized fibrosing disorder affecting the fascial layers of the human body. To date less than 300 cases of EF have been reported worldwide. Due to the limited prevalence, extensive studying of its pathogenesis and treatment has not yet been established. Furthermore, little is known regarding the long-term prognosis and comorbidities associated with EF. In this case study, we discuss a 72-year-old female patient who was diagnosed with EF and subsequently developed squamous cell carcinoma (SCC) of the skin in areas of previously exposed EF. This case represents the first reported case of SCC of the skin in a patient with previously active EF in the same area of skin.
... Occasionally, antinuclear antibodies and rheumatoid factor present in EF patients is the reason why some authors have suggested autoimmune patho-mechanisms [2]. Associated hypergammaglobulinemia (usually IgG), peripheral eosinophilia, high erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) have also been found [3]. ...
The hallmark of eosinophilic fasciitis (EF) is painful symmetric skin stiffness and limb sclerosis with deep fascia inflammation. Two cases of unilateral EF have been reported in the literature but were not related to strenuous physical activity. In this report, a young female presenting with unilateral scleroderma-like skin changes, blood and tissue eosinophilia, and hypergammaglobulinemia proven to be EF after strenuous physical activity is described. This case represents the first case reported in the current literature of unilateral EF after vigorous exercise. A skin to muscle biopsy and magnetic resonance imaging (MRI) study indicated classic EF features. Cases of unilateral eosinophilic fasciitis are under-recognized and have rarely been described in the literature. EF requires a high index of suspicion to be diagnosed accurately and differentiated from another scleroderma variant, especially localized types. Early diagnosis and management of unilateral EF are very crucial because EF management and prognosis are completely different from other scleroderma mimics. EF heals rapidly in response to glucocorticoid or combination therapy. In the current case, corticosteroids alone failed to induce complete disease remission, but with the addition of methotrexate, complete resolution of both skin and systemic features was obtained. She is now in good health with no medication use. The current literature review indicates that this the first reported case of unilateral EF after vigorous exercise.
... Eosinophilic fasciitis is a rare disease characterized by skin alterations such as scleroderma, peripheral eosinophilia, hypergammaglobulinemia, and high erythrocyte sedimentation rate. 1 It more frequently involves the inferior limbs. The absence of Raynaud's phenomenon or sclerodactyly distinguishes Eosinophilic fasciitis from sclerodermia. 1 Literature has described fewer than 300 cases. ...
... The absence of Raynaud's phenomenon or sclerodactyly distinguishes Eosinophilic fasciitis from sclerodermia. 1 Literature has described fewer than 300 cases. Shulman syndrome, as it is also known, was first described in 1974. ...
... There are also cases described as being associated with hematologic disturbances, such as aplastic anemia, hemolytic anemia, self-immune peripheric thrombocytopenia, and pernicious anemia. 1 Self-immune diseases like Hashimoto Thyroiditis, morphea, systemic sclerosis, Sjögren syndrome, and antiphospholipid antibody syndrome were also reported in some patients. 1 In our case, the patient has not presented any malign disease so far. She was already under treatment and supervision for type 1 diabetes mellitus and hypothyroidism. ...
Eosinophilic fasciitis, or Shulman's disease, is a rare disease of unknown etiology. It is characterized by peripheral eosinophilia, hypergammaglobulinemia, and high erythrocyte sedimentation rate. The diagnosis is confirmed by a deep biopsy of the skin. The first line of treatment is corticotherapy. We present a rare case of eosinophilic fasciitis in a 27-year-old woman with an atypical presentation with symmetrical peripheral edema and a Groove sign. The patient responded well to treatment with corticosteroids at high doses and, in this context, was associated with hydroxychloroquine and azathioprine. After two and a half years, peripheral eosinophilia had increased, and more of her skin had hardened. At that time, the therapy was modified to include corticoids, methotrexate, and penicillamine. It is of great importance to publicize these cases that allow us to gather experience and better treat our patients.
... In 1974, Shulman provided an early description of eosinophilic fasciitis as a disorder characterized by peripheral eosinophilia and fasciitis that could be differentiated from scleroderma by the distinctive pattern of skin involvement that spares the digits, involves fascia rather than dermis, and is not accompanied by Raynaud phenomenon [40][41][42][43]. ...
Autoimmune connective tissue diseases are a heterogeneous group of pathologies that affect about 10% of world population with chronic evolution in 20%–80%. Inflammation in autoimmune diseases may lead to serious damage to other organs including the gastrointestinal tract. Gastrointestinal tract involvement in these patients may also due to both a direct action of antibodies against organs and pharmacological therapies. Dysphagia is one of the most important symptom, and it is caused by failure of the swallowing function and may lead to aspiration pneumonia, malnutrition, dehydration, weight loss, and airway obstruction. The videofluorographic swallowing study is a key diagnostic tool in the detection of swallowing disorders, allowing to make an early diagnosis and to reduce the risk of gastrointestinal and pulmonary complications. This technique helps to identify both functional and structural anomalies of the anatomic chain involved in swallowing function. The aim of this review is to systematically analyze the basis of the pathological involvement of the swallowing function for each rheumatological disease and to show the main features of the videofluorographic study that may be encountered in these patients.
... Eosinophilic fasciitis is a scleroderma-like syndrome that was fi rst described by Shulman in 1974 [1][2][3]. Its onset it typically acute and fi ndings include erythema, swellivng and induration of the extremities, usually accompanied by eosinophilia, polyclonal hypergammaglobulinemia and elevated erythrocyte sedimentation rate [9][10][11]. Typical histologic fi ndings include chronic infl ammatory infi ltration affecting deep fascia with lymphocytes, histiocytic, and occasionally eosinophils [1]. ...
... Eosinophilic fasciitis is a scleroderma-like syndrome that was fi rst described by Shulman in 1974 [1][2][3]. Its onset it typically acute and fi ndings include erythema, swellivng and induration of the extremities, usually accompanied by eosinophilia, polyclonal hypergammaglobulinemia and elevated erythrocyte sedimentation rate [9][10][11]. Typical histologic fi ndings include chronic infl ammatory infi ltration affecting deep fascia with lymphocytes, histiocytic, and occasionally eosinophils [1]. ...
... [5][6][7] The absence of Raynaud's phenomenon and internal organ involvement distinguishes this condition from collagen vascular disease such as scleroderma. 1,[8][9][10][11] An unusual feature of EF is the presence of symmetric pitting edema presumed to be secondary to vascular leakage. 3,6,8 Full-thickness skin biopsy showing perivascular inflammation composed of lymphocytes, plasma cells, and eosinophils in the subcutis and fascia or characteristic MRI findings are required to confirm the diagnosis. ...
Eosinophilic fasciitis (EF) is a rare condition involving inflammation of the fascia and peripheral eosinophilia of unknown etiology leading to tissue fibrosis. Clinical presentation includes peripheral eosinophilia, symmetrical skin thickening with subcutaneous tissue induration of the extremities and rashes developing acutely over a period of days to weeks. An unusual feature of EF is the presence of symmetric pitting edema presumed to be secondary to vascular leakage. This is a case of eosinophilic fasciitis presenting in atypical fashion with pitting peripheral edema in addition to the classic symptoms.
... While the initial oedematous phase may be indistinguishable from early sclerodermatous skin changes, the irregular, woody, peau d'orange texture of EF is distinct from the morphea-like plaques or large confluent sclerotic areas with atrophic and shiny surface seen in patients with sclerotic GVHD. Lack of internal organ involvement, and sparing of the head and mucosa area, are characteristics of EF (6)(7)(8). In contrast, all skin and mucosa may be invaded in sclerodermoid GVHD, and diarrhoea, liver dysfunction and systemic disorder can occur frequently (9)(10)(11) (Table SI 1 ). ...
... Histopathologically, in EF the epidermis is usually intact with mild dermal fibrosis. Subcutis and fascia thickening associated with inflammatory cells infiltration, comprising lymphocytes, plasma cells, histiocytes, and eosinophils in variable degrees, are the hallmarks of EF (6)(7)(8). By contrast, sclerodermoid GVHD is characterized by atrophic epidermis, and the dermis is thickened with sclerotic change and destroyed adnexal structures, whereas the subcutis and fascia in cGVHD are usually unremarkable (9-11) (Table SI 1 ). ...
... The erythrocyte sedimentation rate (ESR) is used to assess the acute phase response of many inflammatory diseases. Contemporary dermatology uses the ESR test for assessing disease severity in patients with erysipelas, 1-4 psoriasis, [5][6][7] eosinophilic fasciitis, 8 dermatomyositis, 9 and Behçet's disease. [10][11][12] An elevated ESR is used as a key diagnostic criterion for polymyalgia rheumatica 13 and an independent predictor of giant cell arteritis. ...
In contemporary medicine, the erythrocyte sedimentation rate (ESR) is used to assess severity in patients with such diseases as erysipelas, psoriasis, eosinophilic fasciitis, dermatomyositis, and Behçet's disease. We remember the scientific achievements of a Polish physician, the discoverer of the erythrocyte sedimentation rate (ESR), Edmund Faustyn Biernacki (1866-1911), on the 100th anniversary of his death. The practical application of ESR in clinical diagnostics in 1897 by Biernacki was little known for many years, because it was often neglected owing to the work of Robert Fåhraeusand Alf Westergren from 1921. In addition, it is also frequently omitted that before Westergren's and Fåhraeus's reports were published, ESR was also noticed by Ludwig Hirschfeld in 1917.
