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    1 OF 13

    Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review.
    Clin Rev Allergy Immunol 2017 Jul 16. Epub 2017 Jul 16.
    Section of Dermatology, Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, Italy.
    Scleroderma refers to an autoimmune connective tissue fibrosing disease, including three different subsets: localized scleroderma, limited cutaneous systemic sclerosis, and diffuse cutaneous systemic sclerosis with divergent patterns of organ involvement, autoantibody profiles, management, and prognostic implications. Although systemic sclerosis is considered the disease prototype that causes cutaneous sclerosis, there are many other conditions that can mimic and be confused with SSc. They can be classified into immune-mediated/inflammatory, immune-mediated/inflammatory with abnormal deposit (mucinoses), genetic, drug-induced and toxic, metabolic, panniculitis/vascular, and (para)neoplastic disorders according to clinico-pathological and pathogenetic correlations. Read More

    Scleroderma-like Disorders.
    Curr Rheumatol Rev 2017 06 11. Epub 2017 Jun 11.
    Fortis Escorts Hospital, Jaipur, India
    Scleroderma is a term used to describe diseases that involve hardening and tightening of the skin and the underlying subcutaneous connective tissue. It could be localized to skin and subcutaneous tissue, or may involve the internal organs too in systemic sclerosis. There are disorders that can cause hardening and tightening of skin and mimic scleroderma but are rarely associated with Raynaud phenomenon, sclerodactyly, and autoantibodies in the serum, features specific to scleroderma/systemic sclerosis. Read More

    Scleroderma Mimickers.
    Curr Treatm Opt Rheumatol 2016 Mar 5;2(1):69-84. Epub 2016 Feb 5.
    Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
    Cutaneous fibrosing disorders encompass a diverse array of diseases united by the presence of varying degrees of dermal sclerosis. The quality and distribution of skin involvement, presence or absence of systemic complications and unique associated laboratory abnormalities often help to distinguish between these diseases. It is imperative that an effort is made to accurately differentiate between scleroderma and its mimics, in order to guide long-term management and facilitate implementation of the appropriate treatment modality where indicated. Read More

    Hypereosinophilic syndrome: endomyocardial biopsy versus echocardiography to diagnose cardiac involvement.
    Postgrad Med 2017 Jun 25;129(5):517-523. Epub 2017 Apr 25.
    a Division of Allergic Diseases, and Mayo Clinic Program for Mast Cell and Eosinophilic Disorders , Mayo Clinic , Rochester , MN , USA.
    Objective: To compare echocardiograms and endomyocardial biopsies to diagnose cardiac involvement in hypereosinophilic syndrome.

    Methods: We examined the agreement between echocardiography and endomyocardial biopsies to detect cardiac involvement in hypereosinophilic syndrome by reviewing cases identified as hypereosinophilia or hypereosinophilic syndrome in Mayo Clinic databases from January 1978 through June 2009. Single-organ cases of eosinophilia such as eosinophilic fasciitis and eosinophilic gastroenteritis were excluded. Read More

    Th1- and Th17-polarized immune infiltrates in eosinophilic fasciitis-A potential marker for histopathologic distinction from morphea.
    J Cutan Pathol 2017 Jun;44(6):548-552
    Pathology Service, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts.
    Background: Morphea (localized scleroderma) and eosinophilic fasciitis (EF) are rare fibrosing disorders which may present a diagnostic challenge. While histopathologic features are often distinct, in some cases there may be overlap. T-cells contribute to etiopathogenesis of both autoimmune conditions. Read More

    Morphea and Eosinophilic Fasciitis: An Update.
    Am J Clin Dermatol 2017 Aug;18(4):491-512
    Department of Dermatology, Radboud University Medical Centre, Nijmegen, The Netherlands.
    Morphea, also known as localized scleroderma, encompasses a group of idiopathic sclerotic skin diseases. The spectrum ranges from relatively mild phenotypes, which generally cause few problems besides local discomfort and visible disfigurement, to subtypes with severe complications such as joint contractures and limb length discrepancies. Eosinophilic fasciitis (EF, Shulman syndrome) is often regarded as belonging to the severe end of the morphea spectrum. Read More

    Eosinophilic fasciitis.
    An Bras Dermatol 2016 Sep-Oct;91(5 suppl 1):57-59
    Escola Superior de Ciências da Saúde (ESCS) - Brasília (DF), Brazil.
    Eosinophilic fasciitis is a rare sclerodermiform syndrome of unknown etiology. It is characterized by the thickening of the muscular fascia and subcutaneous tissue, with a variable infiltration of eosinophils. Peripheral eosinophilia, poly or monoclonal hypergammaglobulinemia and increased erythrocyte sedimentation rate can be seen. Read More

    Basidiobolomycosis complicated by hydronephrosis and a perinephric abscess presenting as a hypertensive emergency in a 7-year-old boy.
    Paediatr Int Child Health 2017 Jan 23:1-4. Epub 2017 Jan 23.
    a Department of Pediatrics , Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER) , Pondicherry 605006 , India.
    A 7-year-old boy presented with a chronic, indurated, tender left thigh swelling in association with a hypertensive emergency. He had a bilateral moderate degree of hydronephrosis and a left perinephric abscess, and MRI features of posterior reversible encephalopathy syndrome. Histopathological examination of the biopsy specimen demonstrated eosinophilic fasciitis with filamentous fungi. Read More

    Eosinophilic fasciitis with subjacent myositis.
    Muscle Nerve 2016 Nov 22. Epub 2016 Nov 22.
    Department of Neurology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, Florida, 32224, USA.
    Introduction: Eosinophilic fasciitis (EF) is a rare disorder that can present with muscle symptoms that mimic other neuromuscular diseases.

    Methods: We report the case of a 43-year-old woman with chronic muscle aches, tightness, and stiffness with hypertrophied, well-defined muscles despite physical inactivity, and thickened skin with reduced elasticity and discoloration.

    Results: Except for mild peripheral eosinophilia, laboratory studies, including blood count, electrolytes, paraneoplastic panel, muscle enzymes, thyroid function, and serum protein electrophoresis, were normal. Read More

    A literature review of eosinophilic fasciitis with an illustrated case.
    Curr Rheumatol Rev 2016 Oct 7. Epub 2016 Oct 7.
    Clinical fellow,Neurology department, Salford Royal NHS Foundation Trust,Stott Lane, Salford, UK M6 8HD.
    Eosinophilic fasciitis (EF), a rare connective tissue disorder, was first reported by Lawrence Shulman in 1974. Since then over 300 cases have been reported worldwide. EF has variable clinical presentations and currently does not have internationally accepted diagnostic criteria. Read More

    A rare case of unilateral eosinophilic fasciitis associated with ipsilateral extragenital lichen sclerosus.
    Indian Dermatol Online J 2016 Sep-Oct;7(5):386-389
    Department of Dermatology, Indian Naval Hospital Ship Asvini, Mumbai, Maharashtra, India.
    Eosinophilic fasciitis, also known as Shulman's syndrome, is a fibrosing scleroderma-like syndrome, which is a distinct entity. A 55-year-old man, presented with progressive skin darkening, thickening, and tightening over the left lower limb since 6 months. Dermatological examination revealed a hyperpigmented indurated area on the left thigh, extending to the anterior aspect of the left leg. Read More

    Shulman disease (eosinophilic fasciitis) in X-linked agammaglobulinemia.
    Pol J Pathol 2016 Jun;67(2):183-8
    Prof. Anna Pituch-Noworolska MD, Department of Clinical Immunology, Polish-American Institute of Pediatrics, Medical College, Jagiellonian University, Wielicka 265, 30-663 Krakow, Poland, tel./fax +48 12 658 17 56, e-mail:
    X-linked agammaglobulinemia (XLA) diagnosed in the first year of life is an immunodeficiency with a life-long indication for substitution of immunoglobulins, due to lack of B lymphocytes in the periphery. The decrease of bacterial infection frequency and severity is an effect of immunoglobulin replacement. However, in the majority of patients bronchiectasis and chronic sinusitis with an overgrown mucous membrane develop despite regular substitution. Read More

    High-Dose Intravenous Pulse Methotrexate in Patients With Eosinophilic Fasciitis.
    JAMA Dermatol 2016 Nov;152(11):1262-1265
    Department of Dermatology, Radboud University Medical Centre, Nijmegen, the Netherlands.
    Importance: Eosinophilic fasciitis (EF) is a connective tissue disorder in which conventional treatment leads to disappointing results in a proportion of patients. Therefore, we investigated high-dose intravenous (IV) pulse methotrexate (MTX) as a treatment for EF.

    Objective: To examine safety and effects of monthly high-dose IV pulse MTX in EF. Read More

    Successful treatment of aplastic anemia-paroxysmal nocturnal hemoglobinuria associated with eosinophilic fasciitis with matched unrelated donor allogeneic peripheral blood stem cell transplantation.
    Clin Case Rep 2016 Aug 6;4(8):765-7. Epub 2016 Jul 6.
    Department of Leukemia The University of Texas MD Anderson Cancer Center Houston Texas USA.
    We report the first patient case of successful treatment intervention for both eosinophilic fasciitis and aplastic anemia with allogeneic peripheral blood stem cell transplantation from a matched unrelated donor after multiple immunosuppressant failure. Read More

    Eosinophilic fasciitis after parasite infection.
    Reumatologia 2016 24;54(1):38-41. Epub 2016 Mar 24.
    Centro Hospitalar Baixo Vouga, Aveiro, Portugal.
    Eosinophilic fasciitis is a systemic inflammatory disease characterized by symmetrical swelling and skin induration of the distal portions of the arms and/or legs, evolving into a scleroderma-like appearance, accompanied by peripheral blood eosinophilia. It is a rare disease with a poorly understood etiology. Corticosteroid treatment remains the standard therapy, either taken alone or in association with an immunosuppressive drug. Read More

    Development of Eosinophilic Fasciitis during Infliximab Therapy for Psoriatic Arthritis.
    Case Rep Rheumatol 2016 11;2016:7906013. Epub 2016 May 11.
    Division of Rheumatology, Department of Medicine, Medical College of Wisconsin, 9200 W. Wisconsin Avenue, Milwaukee, WI 53226, USA.
    Eosinophilic fasciitis (EF) is a rare disorder involving chronic inflammation of the fascia and connective tissue surrounding muscles, nerves, and blood vessels. While its pathogenesis is not entirely understood, this disorder is thought to be autoimmune or allergic in nature. We present here a case of a 59-year-old male who developed peripheral eosinophilia and subsequent eosinophilic fasciitis during treatment with infliximab. Read More

    Juvenile eosinophilic fasciitis: three case reports with review of the literature.
    Clin Exp Rheumatol 2016 May-Jun;34(3):527-30. Epub 2016 May 30.
    UOC Pediatria II, Reumatologia, Istituto Giannina Gaslini, Genoa, Italy.
    Objectives: Eosinophilic fasciitis is an uncommon scleroderma-like disorder characterised by induration and thickening of skin and soft tissue, usually associated with peripheral eosinophilia, poorly characterised in childhood.

    Methods: We report 3 paediatric cases of eosinophilic fasciitis showing unusual clinical and histopathological features with a review of the literature.

    Results: All cases presented progressive motility impairment started from upper limbs with no skin abnormalities. Read More

    Correspondence: The association between morphea profunda and monoclonal gammopathy: A case series.
    Dermatol Online J 2016 Mar 16;22(3). Epub 2016 Mar 16.
    University of Wisconsin.
    It is known that eosinophilic fasciitis can be associated with monoclonal gammopathy. There is clinical similarity between eosinophilic fasciitis and morphea profunda, but it is unclear whether morphea profunda might be associated with monoclonal gammopathy. The temporal quantification of gammopathy in morphea profunda has not been well characterized. Read More

    [Scleroderma-like disorders].
    Rev Med Interne 2016 Sep 3;37(9):616-24. Epub 2016 Feb 3.
    Service de médecine interne, hôpital Robert-Bisson, 4, rue Roger-Aini, 14100 Lisieux, France.
    The finding of hardening and thickening of the skin is common and can be encountered in immune mediated, metabolic, neoplastic, toxic, genetic diseases, or associated with protein deposits. The lack of Raynaud's phenomenon, capillaroscopic abnormalities, or scleroderma-specific autoantibodies should question the diagnosis of scleroderma and trigger the search for a scleroderma-like disorder, for which treatment and prognosis differ. This article gives a review of these disorders and their main characteristics. Read More

    Eosinophilic Fasciitis and Acute Encephalopathy Toxicity from Pembrolizumab Treatment of a Patient with Metastatic Melanoma.
    Cancer Immunol Res 2016 Mar 28;4(3):175-8. Epub 2016 Jan 28.
    Princess Margaret Cancer Centre, Toronto, Ontario, Canada.
    Anti-PD-1 inhibitors have significant activity in metastatic melanoma. Responses often occur early and may be sustained. The optimal duration of treatment with these agents is unknown. Read More

    Atypical Presentation of Eosinophilic Fasciitis with Pitting Edema.
    Hawaii J Med Public Health 2015 Sep;74(9 Suppl 2):36-8
    John A. Burns School of Medicine, University of Hawai'i, Honolulu, HI (C-WC).
    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. Read More

    Atypical presentations of eosinophilic fasciitis.
    Indian J Dermatol Venereol Leprol 2016 Jan-Feb;82(1):47-52
    Department of Dermatology, Marmara University School of Medicine, Istanbul, Turkey.
    Eosinophilic fasciitis is an uncommon connective tissue disease that may mimic and overlap with other sclerosing disorders such as morphea and lichen sclerosus. Herein, we report four patients (two men and two women, aged 16-64 yeas) with eosinophilic fasciitis. There was overlap with both morphea and lichen sclerosus in 2 patients and with morphoea alone in 1 patient. Read More

    Severe eosinophilic fasciitis: comparison of treatment with D-penicillamine plus corticosteroids vs. corticosteroids alone.
    Scand J Rheumatol 2016 3;45(2):129-34. Epub 2015 Nov 3.
    b Jefferson Institute of Molecular Medicine and Scleroderma Center , Thomas Jefferson University , Philadelphia , PA , USA.
    Objectives: To compare the therapeutic effectiveness of corticosteroids (CS) alone vs. CS plus d-penicillamine (d-Pen) in severe eosinophilic fasciitis.

    Method: A long-term prospective non-randomized trial of d-Pen plus CS vs. Read More

    [Eosinophilic fasciitis (Shulman's disease): Diagnostic and therapeutic review].
    Rev Med Interne 2015 Nov 16;36(11):738-45. Epub 2015 Sep 16.
    Université Paris-Cité Sorbonne Paris-Diderot, 75013 Paris, France; Service de médecine interne, hôpital Lariboisière, AP-HP, 2, rue Ambroise-Paré, 75475 Paris cedex 10, France. Electronic address:
    Eosinophilic fasciitis (EF) is a rare connective tissue disease characterized by symmetrical and painful swelling with a progressive induration and thickening of the skin and soft tissues. The diagnosis of EF is often based on the association of characteristic skin or subcutaneous abnormalities and a thickened fascia with an inflammatory infiltration, mostly composed of lymphocytes and eosinophils. A peripheral eosinophilia is frequently present (60-90%) but is not mandatory for the EF diagnosis. Read More

    Scleredema Diabeticorum - A Case Report.
    Mymensingh Med J 2015 Jul;24(3):606-9
    Dr Md Nahiduzzamane Shazzad, Medical Officer, Department of Rheumatology, Bangabandhu Sheikh Mujib MedicalUniversity (BSMMU), Dhaka, Bangladesh.
    Buschke Scleredema is a rare connective tissue disorder of unknown aetiology and characterized by thickening of the dermis. It resembles systemic sclerosis, eosinophilic fasciitis and cutaneous amyloidosis. Scleredema may be associated with a history of an antecedent febrile illness, diabetes mellitus, or blood dyscrasia. Read More

    Finger stiffness or edema as presenting symptoms of eosinophilic fasciitis.
    Rheumatol Int 2015 Oct 7;35(10):1769-72. Epub 2015 Aug 7.
    Department of General Medicine, Chiba University Hospital, 1-8-1 Inohana, Chuo-ku, Chiba-shi, Chiba, 260-8677, Japan.
    To investigate the clinical features and finger symptoms of eosinophilic fasciitis (EF), we reviewed five patients with EF. The chief complaint was pain, edema and/or stiffness of the extremities. The distal extremities were affected in all patients, and there was also proximal involvement in one patient. Read More

    Eosinophilia in Rheumatologic/Vascular Disorders.
    Immunol Allergy Clin North Am 2015 Aug 17;35(3):453-76. Epub 2015 Jun 17.
    Department of Rheumatic and Immunologic Diseases, Center for Vasculitis Care and Research, Cleveland Clinic, 9500 Euclid Avenue, A50, Cleveland, OH 44195, USA. Electronic address:
    Peripheral and tissue eosinophilia can be a prominent feature of several unique rheumatologic and vascular diseases. These diseases span a wide range of clinical features, histologic findings, therapeutic approaches, and outcomes. Despite the rare nature of these entities--which makes large-scale studies challenging--knowledge has continued to grow regarding their epidemiology, pathophysiology, and management. Read More

    Efficacy of Tocilizumab in the treatment of Eosinophilic fasciitis: Report of one case.
    Joint Bone Spine 2015 Dec 7;82(6):460-1. Epub 2015 Jul 7.
    Clinical immunology and osteoarticular diseases therapeutic unit, Lapeyronie university hospital, 371, avenue du Doyen-Gaston-Giraud, 34295 Montpellier, France. Electronic address:
    A 43-year-old man was diagnosed with an Eosinophilic fasciitis with cutaneous and articular involvement. The patient experienced an early response with high-dose corticosteroids achieving a global remission of disease. Nevertheless, during the steroids tapering phase, he presented a new flare and subsequently developed a corticosteroid refractory disease. Read More

    Eosinophilic fasciitis associated with myositis.
    Case Rep Dermatol 2015 Jan-Apr;7(1):79-83. Epub 2015 Apr 30.
    Department of Dermatology, Gifu University Graduate School of Medicine, Gifu, Japan.
    Eosinophilic fasciitis is clinically characterized by symmetrical scleroderma-like indurations of the skin with pain. The histological features are fascial inflammation with lymphocytes and eosinophils as well as thickened and fibrotic fascia. Lymphocytic infiltration and degeneration of the underlying muscle are rarely observed. Read More

    [Eosinophilic fasciitis].
    Postepy Hig Med Dosw (Online) 2015 Jan 2;69:488-95. Epub 2015 Jan 2.
    Katedra i Klinika Reumatologii i Chorób Wewnętrznych Uniwersytetu Medycznego w Poznaniu.
    Eosinophilic fasciitis is a rare connective tissue disease with unclear etiology and pathogenesis. It is classified as a scleroderma-like syndrome. The disease is characterized by fibrosis of the skin and subcutaneous tissues with significant thickening of fascia. Read More

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