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Radiographics 2019 Jan-Feb;39(1):229-250

From the Departments of Radiology (M.N., E.J.I.C., M.R.P.) and Rheumatology (E.I., J.A.), Hospital Sant Joan de Déu, Passeig Sant Joan de Déu 2, 08950 Esplugues de Llobregat, Barcelona, Spain; Department of Medical Imaging, University of Toronto, Toronto, ON, Canada (M.N., O.M.N.); and Department of Diagnostic Imaging, The Hospital for Sick Children, Toronto, ON, Canada (M.N., O.M.N.).

Systemic connective tissue disorders are characterized by the presence of autoantibodies and multiorgan system involvement. Juvenile systemic lupus erythematosus with or without associated antiphospholipid syndrome; juvenile dermatomyositis; sclerodermiform syndromes, including systemic and localized sclerodermas and eosinophilic fasciitis; mixed connective tissue disease; and Sjögren syndrome are the disorders that affect children most frequently. Diagnosis is difficult, because the clinical presentation of patients is diverse, from mild to severe disease. Read More

Australas J Dermatol 2018 Dec 27. Epub 2018 Dec 27.

Department of Neurology, Renmin Hospital of Wuhan University, Wuhan, Hubei Province, China.

Eosinophilic fasciitis is a rare scleroderma-like syndrome with an unknown aetiology. The characteristics of this disorder include lymphoplasmacytic inflammation involving the subcutaneous fat septa and fascia. Eosinophilic myositis is diagnosed when inflammation extends into the muscles. Read More

BMJ Case Rep 2018 Dec 22;11(1). Epub 2018 Dec 22.

Department of Rheumatology, North Bristol NHS Trust, Bristol, UK.

Eosinophilic fasciitis (EF) is a syndrome of unknown aetiology characterised by progressive collagenous thickening of the subcutaneous fascia. Limb oedema can precede the skin thickening and induration classically associated with EF. We describe a case of EF in a 31-year-old woman who presented to her general practitioner with lower limb oedema and stiffness. Read More

J Thorac Oncol 2019 Jan;14(1):e13-e15

Department of Internal Medicine, South Hospital, Rennes, France; Rennes 1 University, Rennes, France.

Intern Med 2018 Oct 17. Epub 2018 Oct 17.

Third department of Internal Medicine, Nara Medical University, Japan.

Both autoimmune hepatitis (AIH) and eosinophilic fasciitis (EF) are known to be complicated by other autoimmune diseases. However, AIH complicated by EF has never been reported. We experienced a 58-year-old man with AIH complicated by EF. Read More

Drugs Context 2018 2;7:212529. Epub 2018 Oct 2.

Department of Dermatology, Wake Forest University School of Medicine, Winston-Salem, NC, USA.

Eosinophilic fasciitis is an uncommon connective tissue disorder that affects patients of all ages, resulting in significant morbidity. Systemic corticosteroids can induce remission of disease. However, there is no universally accepted treatment ladder for eosinophilic fasciitis. Read More

Z Rheumatol 2019 Feb;78(1):14-23

Klinik für Rheumatologie und klinische Immunologie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Deutschland.

Background: Systemic sclerosis (SSc) is characterized by heterogeneous clinical symptoms. Peripheral skin fibrosis can be a common symptom. Nevertheless, a variety of diseases with different etiologies are associated with a thickening of the skin and make the initial diagnosis of systemic sclerosis more difficult. Read More

Clin Exp Dermatol 2019 01 30;44(1):85-86. Epub 2018 Aug 30.

Department of Dermatology, Hospital Universitario La Paz, Paseo de la Castellana 261, Madrid, 28046, Spain.

Clin Case Rep 2018 Aug 4;6(8):1412-1417. Epub 2018 Jun 4.

Department of Rheumatology School of Medicine University of California, San Diego La Jolla CA USA.

Although eosinophilic fasciitis is known to be part of the deep morphea spectrum, this first report of the coexistence of granulomatous fasciitis and morphea profunda suggests that granulomatous fasciitis may also be a part of the spectrum of deep morphea. Read More

Clin Dermatol 2018 Jul - Aug;36(4):487-497. Epub 2018 Apr 10.

Department of Dermatology, Oregon Health and Science University, Portland, Oregon, USA.

Eosinophilic fasciitis (EF) is an uncommon connective tissue disease characterized by abrupt onset of edema, followed by progressive induration of primarily the distal extremities. Patients may exhibit inflammatory arthritis, joint contractures, decreased mobility, and nerve entrapment. Almost half of patients with EF may have coexisting morphea plaques. Read More

JAAD Case Rep 2018 Jun 30;4(5):443-445. Epub 2018 Apr 30.

The Department of Dermatology, Yale University, New Haven, Connecticut.

JAAD Case Rep 2018 Mar 4;4(2):175-178. Epub 2018 Feb 4.

Department of Dermatology, The University of Texas MD Anderson Cancer Center, Houston, Texas.

Generalized morphea is associated with epoxy resin vapors and is characterized by the development of lesions shortly after exposure. Morphea presenting along with eosinophilic fasciitis (EF), or morphea/EF overlap, is rare and an indicator of poor prognosis and resistance to treatment. Here we present a case of generalized morphea/EF overlap linked to epoxy exposure. Read More

Australas J Dermatol 2018 Nov 24;59(4):e302-e304. Epub 2018 May 24.

Department of Dermatology, Hospital 12 de Octubre and Institute i+12, Medical School, Universidad Complutense, Madrid, Spain.

JAAD Case Rep 2018 May 31;4(4):327-329. Epub 2018 Mar 31.

Department of Allergy and Immunology, Centre Hospitalier de l'Université de Montréal (CHUM), Montreal, Canada.

Muscle Nerve 2018 Aug 24;58(2):E15-E18. Epub 2018 May 24.

Department of Neurology, Wake Forest School of Medicine, Winston-Salem, North Carolina, USA.

J Investig Allergol Clin Immunol 2018 Apr;28(2):126-128

Department of Dermatology, Hospital Universitari Germans Trias i Pujol, Badalona, Universitat Autònoma de Barcelona, Spain.

Int J Dermatol 2018 06 6;57(6):743-745. Epub 2018 Apr 6.

Departament of Dermatology, University Hospital Complex of Santiago de Compostela, A Coruña, Spain.

Am J Dermatopathol 2018 Apr;40(4):e48-e49

Department of Dermatology and Cutaneous Surgery, University of South Florida, Tampa, FL.

Br J Dermatol 2018 Aug 7;179(2):516-517. Epub 2018 Jun 7.

Service de rhumatologie, Centre National de Référence des Maladies Systémiques et Auto-immunes rares, Université de Strasbourg, Strasbourg, France.

J Med Ultrason (2001) 2018 Oct 20;45(4):641-645. Epub 2018 Feb 20.

Department of Physical Medicine and Rehabilitation, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan, ROC.

Eosinophilic fasciitis (EF), a rare rheumatic disease, usually affects the limbs symmetrically and generally spares the hands and feet. Cases of unilateral hand involvement are rarely reported. Here, we report such a rare case. Read More

Int J Dermatol 2018 05 15;57(5):614-615. Epub 2018 Feb 15.

Department of Dermatology, Henry Ford Hospital, Detroit, MI, USA.

Isr Med Assoc J 2018 Feb;20(2):95-99

Department of Medicine, Hadassah-Hebrew University Medical Center, Ein Kerem Campus, Jerusalem, Israel.

Background: Eosinophilic fasciitis (EF) is a rare disease characterized by scleroderma-like skin, inflammation of deep muscle fascia, hypergammaglobulinemia, peripheral eosinophilia, and elevated erythrocyte sedimentation rate.

Objectives: To present our experience in diagnosis and treatment of seven biopsy-proven EF patients in a large tertiary medical center.

Methods: We screened all patients who were admitted to our tertiary medical center and diagnosed with EF by tissue biopsies from January 2000 to January 2016. Read More

Indian J Dermatol Venereol Leprol 2018;84(2):224-227

Department of Pathology, JIPMER, Puducherry, India.

Autoimmun Rev 2018 Mar 16;17(3):284-289. Epub 2018 Jan 16.

Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; Ella Lemelbaum Institute for Immuno-Oncology, Sheba Medical Center, Israel. Electronic address:

Background: The use of immune checkpoint inhibitors (ICI) has grown incessantly since they were first approved in 2014. These monoclonal antibodies inhibit T cell activation, yielding a dramatic tumor response with improved survival. However, immunotherapy is frequently hampered by immune adverse events (iAE) such as hypophysitis, colitis, hepatitis, pneumonitis and rash. Read More

Harefuah 2017 Dec;156(12):786-790

Sackler Faculty of Medicine, Tel Aviv University.

Introduction: Eosinophilic fasciitis (EF) is a rare connective tissue disease characterized by erythema, edema and myalgia with induration and thickening of the skin and soft tissue, especially subcutaneous fascia. Laboratory findings usually show peripheral eosinophilia, and full-thickness wedge biopsy is essential to establish the diagnosis. Corticosteroids are effective and remain the standard therapy for EF, although some patients may improve spontaneously. Read More

Clin Exp Dermatol 2018 Apr 26;43(3):306-310. Epub 2017 Dec 26.

Royal United Hospital Bath, Bath, Avon, UK.

In this small case series, all eight patients were women in their fifth and sixth decades. This is similar to the female predominance in morphoea and less in keeping with eosinophilic fasciitis (EF). All cases had diffuse induration of their limbs with both proximal and distal patterns of distribution, and five of the patients exhibited peau d'orange skin. Read More

Rheumatol Int 2018 Mar 15;38(3):525-529. Epub 2017 Dec 15.

Division of Rheumatology, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, São Paulo, SP, Brazil.

The authors describe the case of a 23-year-old woman who was referred to the rheumatologist due to symmetrical and progressive stiffness, induration, and swelling of arms and thighs at the 12th week of her first gestation. The characteristic clinical aspect of 'peau d'orange', associated to the histopathologic results of the deep biopsy of the skin confirmed the diagnosis of eosinophilic fasciitis. Treatment with oral prednisone, at an initial dose of 1 mg/kg/day, was effective and rapidly tapered to 10 mg/day till the birth of a healthy newborn. Read More

J Dermatol 2018 Aug 13;45(8):881-890. Epub 2017 Dec 13.

Department of Dermatology and Plastic Surgery, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan.

We established diagnostic criteria and severity classification of eosinophilic fasciitis because there is no established diagnostic criteria or widely accepted severity classification of the disease. Also, there has been no clinical guideline for eosinophilic fasciitis, so we established its clinical guideline ahead of all over the world. In particular, the clinical guideline was established by clinical questions based on evidence-based medicine according to the New Minds Clinical Practice Guideline Creation Manual (version 1. Read More

Curr Rheumatol Rep 2017 Nov 4;19(12):74. Epub 2017 Nov 4.

Department of Dermatology, Brigham and Women's Hospital, Boston, MA, USA.

Purpose Of Review: First recognized in 1974, eosinophilic fasciitis (EF) is a fibrosing disorder of the fascia with characteristic cutaneous and hematologic manifestations. This review discusses recent trends in the diagnosis and treatment of EF.

Recent Findings: Although fascial biopsy has classically been considered the gold standard for making a diagnosis of EF, radiologic imaging, particularly magnetic resonance imaging, has been increasingly used for both diagnosis and monitoring of treatment response. Read More

Reumatol Clin 2017 Oct 23. Epub 2017 Oct 23.

Department of Rheumatology, Hospital Universitario de Bellvitge-IDIBELL, Barcelona, Spain.

Ann Dermatol Venereol 2018 Jan 28;145(1):37-42. Epub 2017 Sep 28.

Service de dermatologie, hôpital Saint-Louis, 1, avenue Claude-Vellefaux, 75010 Paris, France; Université Paris-Diderot, Paris VII, 75010 Paris, France. Electronic address:

Background: Eosinophilic fasciitis (EF) is a rare condition characterized by swelling of the extremities, sclerodermatous evolution and frequent hypereosinophilia. Hematological disorders, including aplastic anemia, solid tumors and autoimmune diseases, may be associated with EF. EF is usually not associated with granulomatous diseases. Read More

Rheumatology (Oxford) 2017 Apr;56(4):628

Department of Internal Medicine, Autoimmune Diseases Unit, Vall d´Hebrón University Hospital, Barcelona, Spain.

Rev Med Interne 2017 Dec 1;38(12):840-843. Epub 2017 Sep 1.

Service de médecine interne, hôpital Nord, Aix-Marseille université, Assistance publique-Hôpitaux de Marseille (AP-HM), chemin des Bourrely, 13915 Marseille cedex 15, France. Electronic address:

Introduction: Eosinophilic fasciitis or Shulman's disease is characterized, in its typical form, by palpable thickening of the skin and soft tissues, blood hypereosinophilia and fascia lesions. We hereby report a case of eosinophilic fasciitis in which hypereosinophilia preceded for several months the clinical signs of fasciitis.

Case Report: A 64-year-old woman, with a history of Little's syndrome with motor disability, was admitted in internal medicine for eosinophilia. Read More

J Am Acad Dermatol 2018 Jan 31;78(1):121-128. Epub 2017 Aug 31.

Department of Dermatology, Mayo School of Graduate Medical Education, Mayo Clinic, Rochester, Minnesota; Department of Laboratory Medicine and Pathology, Mayo School of Graduate Medical Education, Mayo Clinic, Rochester, Minnesota. Electronic address:

Background: Eosinophilic fasciitis (EF) and morphea profunda (MP) are inflammatory and sclerosing disorders of the subcutis that can exhibit clinical and pathologic presentations that overlap.

Objective: To identify clinicopathologic features that can be used to distinguish EF from MP.

Methods: We performed a retrospective review of 16 patients with EF and 11 patients with MP. Read More

Ann Dermatol Venereol 2017 Nov 29;144(11):700-704. Epub 2017 Jul 29.

Service de dermatologie, CHU de Caen, avenue de la Côte-de-Nacre, CS 30001, 14033 Caen cedex 9, France.

Background: Connective tissue nevus (CTN) is a rare condition of the extracellular matrix components that generally presents as papulae of normal skin colour. This condition may be syndromic or sporadic.

Patients And Methods: We report herein two isolated cases of extensive and infiltrative CTN in children at risk for subsequent joint stiffening. Read More

J Am Acad Dermatol 2017 Sep 19;77(3):512-517.e5. Epub 2017 Jul 19.

Department of Dermatology, Radboud University, Nijmegen, The Netherlands; Radboud University Medical Centre, Radboud University, Nijmegen, The Netherlands.

Background: Eosinophilic fasciitis (EF) is a connective tissue disease with an unknown long-term course.

Objective: To evaluate presence and determinants of residual disease damage in patients with EF after long-term follow-up.

Methods: Patients with biopsy-proven EF were included for this cross-sectional study. Read More

Clin Rev Allergy Immunol 2017 Dec;53(3):306-336

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

Curr Rheumatol Rev 2018 Apr;14(1):22-27

Fortis Escorts Hospital, Jaipur, Rajasthan, India.

Background: 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.

Objective: 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

Skinmed 2017 1;15(2):139-140. Epub 2017 Apr 1.

Division of Dermatology, Departments of Medicine and Pediatrics, Cooper Medical School of Rowan University, Marlton, NJ;

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

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

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

Acta Reumatol Port 2017 Jul-Sep;42(3):271-272

Centro Hospitalar e Universitário de Coimbra.

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

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

J Clin Rheumatol 2017 Apr;23(3):169

From The Ronald O. Perelman Department of Dermatology, New York University Langone Medical Center, New York, NY.

J Eur Acad Dermatol Venereol 2017 Aug 27;31(8):e356-e358. Epub 2017 Feb 27.

Department of Dermatology, Henry Ford Hospital, Detroit, MI, 48202, USA.

Paediatr Int Child Health 2018 05 23;38(2):146-149. 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

Muscle Nerve 2017 09 27;56(3):525-529. Epub 2017 Mar 27.

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

Curr Rheumatol Rev 2017 ;13(2):113-120

Consultant Neurologist, Salford Royal NHS Foundation Trust, United Kingdom.

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