Muscle Nerve 2018 Apr 20. Epub 2018 Apr 20.

Department of Neurology, Wake Forest School of Medicine, Winston-Salem, NC, USA, 27157.

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 Apr 6. Epub 2018 Apr 6.

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

Br J Dermatol 2018 Mar 11. Epub 2018 Mar 11.

Service de rhumatologie, Centre National de Référence des Maladies Systémiques et Auto-immunes rares, Université de Strasbourg, INSERM UMR-S 1109, F-67000, Strasbourg, France.

Our knowledge of Eosinophilic fasciitis (EF), also known as Shulman's syndrome, is limited, and its prevalence has not been estimated so far. We conducted a regional survey which aimed at estimating the prevalence of EF in Alsace, a Region in the North-East of France. We retrospectively collected EF cases from the first of January 1983 to the 30 of March 2015 among Alsace residents aged >18 years, then performed a capture-recapture analysis with the prevalent cases in 2015. Read More

J Med Ultrason (2001) 2018 Feb 20. 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 May 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.

Arthritis Care Res (Hoboken) 2018 Jan 30. Epub 2018 Jan 30.

Division of Rheumatology, Washington University in St. Louis School of Medicine, MO.

One month prior to rheumatology evaluation he was admitted to the hospital for progressive worsening of shortness of breath on exertion, epistaxis, and gingival bleeding. He had fatigue, poor appetite, and an unintentional weight loss of about 30 pounds over the preceding two years. He denied symptoms of chest pain, orthopnea, paroxysmal nocturnal dyspnea, or leg swelling. Read More

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 2017 Dec 13. 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 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

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 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

Muscle Nerve 2017 Sep 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

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

Ir Med J 2016 Apr 11;109(4):399. Epub 2016 Apr 11.

Neurology Department, Cork University Hospital, Wilton, Cork.

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

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

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

Intern Med 2016;55(16):2321-2. Epub 2016 Aug 15.

Department of Allergy and Clinical immunology, Chiba University Hospital, Japan.

Ann Emerg Med 2016 Aug;68(2):e57-8

Immuno-Rheumatology Center, St. Luke's International Hospital, St. Luke's International University, Tokyo, Japan.

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

J Eur Acad Dermatol Venereol 2017 Feb 12;31(2):e101-e102. Epub 2016 Jul 12.

Dermatology Department, Saint-Louis Hospital, Paris, France.

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

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

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

Eur J Dermatol 2016 Jun;26(3):319-20

Department of Dermatology, Shiga University of Medical Science, Setatsukinowa, Otsu, Shiga 520-2192, Japan.

MMW Fortschr Med 2015 Nov;157(19)