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Inflamm Bowel Dis 2020 Jun 12. Epub 2020 Jun 12.

Department of Gastroenterology, Venizeleio General Hospital, Heraklion, Greece.

Clin Dermatol 2020 Mar - Apr;38(2):235-249. Epub 2019 Oct 24.

Department of Rheumatology and Immunology, University of Pécs Clinical Center, Pecs, Hungary. Electronic address:

Sclerodermalike syndromes (SLSs) comprise diseases with mucin deposition (eg, scleromyxedema, scleredema), with eosinophilia (eg, eosinophilic fasciitis), metabolic or biochemical abnormalities (eg, nephrogenic systemic fibrosis), or endocrine disorders (eg, POEMS syndrome, or polyneuropathy, organomegaly, endocrinopathy, monoclonal lymphoproliferative disorder, and hypothyroidism). Chronic graft-versus-host disease may also show sclerodermalike skin changes. Inherited progeria syndromes with early aging (eg, Werner syndrome) and a heterogeneous group of hereditary disorders with either skin thickening (eg, stiff skin syndrome) or atrophy and tightening (eg, acrogeria) can also imitate classic systemic sclerosis (SSc). Read More

Dermatol Ther 2020 May 22:e13641. Epub 2020 May 22.

Department of Rheumatology, PLA General Hospital, Beijing, China.

Eosinophilic fasciitis (EF) is a rare connective tissue disease characterized by increased peripheral blood eosinophils and diffuse fasciitis, generalized morphea (GM) is a subtype of localized scleroderma, and IgA nephropathy is a chronic glomerulonephritis caused by abnormal deposition of IgA in the mesangial area of the glomeruli. We describe a 49-year-old male patient with hard skin, cutaneous hyperpigmentation, and proteinuria. The patient had suffered from a long disease course of hard skin, while urine protein was newly detected. Read More

Am J Clin Dermatol 2020 May 11. Epub 2020 May 11.

Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Eosinophilic dermatoses encompass a broad spectrum of diseases of different etiologies hallmarked by eosinophilic infiltration of the skin and/or mucous membranes, with or without associated blood eosinophilia. The wide range of dermatological manifestations of this spectrum, including nodules and plaques, pustules, blisters, ulcers, and urticarial lesions, is reflected in a non-univocal classification system. We identified six groups of eosinophilic dermatoses based on the predominant anatomic level of involvement: (1) epidermal; (2) of the dermal-epidermal junction; (3) dermal; (4) of the hypodermis and muscle fascia; (5) of the pilosebaceous unit; and (6) vascular/perivascular. Read More

Cureus 2020 Apr 6;12(4):e7562. Epub 2020 Apr 6.

Department of Dermatology, University of Nevada Las Vegas School of Medicine, Las Vegas, USA.

Cutaneous sclerosis occurs in association with a variety of systemic diseases, including hematologic malignancy, plasma cell dyscrasias, solid organ tumors, and other systemic autoimmune conditions. Herein, we present a unique case of morphea/lichen sclerosus overlap arising in association with aplastic anemia. To expand upon this rare case, we also review the literature surrounding paraneoplastic sclerosing skin disorders. Read More

Eur J Case Rep Intern Med 2020 2;7(4):001485. Epub 2020 Mar 2.

Internal Medicine Department, Centro Hospitalar Universitário do Porto, Porto, Portugal.

Fasciitis with eosinophilia (FE) is a rare connective tissue disease. Due to its rarity, large-scale studies are lacking, which makes its treatment challenging. Systemic corticosteroids (SCSs) are the cornerstone of treatment; however, additional immunosuppressive drugs (ISDs) are frequently necessary (usually methotrexate). Read More

Cureus 2020 Mar 1;12(3):e7155. Epub 2020 Mar 1.

Dermatology, San Diego Family Dermatology, National City, USA.

Statin medications [3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase inhibitors] are generally used to treat hypercholesterolemia. Lichenoid drug eruptions are a potential cutaneous side effect of medications including antibiotics, antimalarials, and statins. This drug eruption can mimic features of idiopathic lichen planus in clinical presentation and pathology. Read More

Clin Exp Rheumatol 2020 Apr 3. Epub 2020 Apr 3.

Department of Rheumatology, Ghent University Hospital, Ghent, Belgium.

Dermatol Online J 2020 Feb 15;26(2). Epub 2020 Feb 15.

Department of Dermatology, Fukushima Medical University, Fukushima.

A 60-year old man developed skin hardening and edema on his extremities. Although he had been treated with oral prednisolone at another hospital, skin stiffness relapsed during tapering of prednisolone. At the initial visit to our department, physical examination showed skin hardening of the extremities and also symmetric erythematous macules on the back. Read More

JAMA Dermatol 2020 Apr 1. Epub 2020 Apr 1.

Division of Rheumatology, Department of Internal Medicine, Okinawa Chubu Hospital, Uruma City, Okinawa, Japan.

JAMA Dermatol 2020 Apr 1. Epub 2020 Apr 1.

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

Pediatr Dermatol 2020 May 20;37(3):572-573. Epub 2020 Mar 20.

Departments of Dermatology and Pediatrics, University of Wisconsin, Madison, Wisconsin.

Lipoatrophic panniculitis (LP) is a rare childhood panniculitis characterized by sclerotic, atrophic plaques on the extremities. We present a case of LP diagnosed during the inflammatory phase that was difficult to distinguish clinically from eosinophilic fasciitis. This report adds to the limited phenotypic spectrum of LP by differentiating the clinical features of disease activity from disease damage and highlighting the importance of biopsy in establishing a diagnosis. Read More

BMJ Mil Health 2020 Mar 5. Epub 2020 Mar 5.

Department of Infection, Royal Free Hospital, London, UK.

We present the case of a Nepalese British soldier with peripheral oedema and a significantly raised eosinophil count. After extensive investigation looking for a parasitic cause of his illness, he was diagnosed with eosinophilic fasciitis, a connective tissue disorder, often triggered by heavy exertion and responsiveness to immunosuppression. In a military setting, in which clinicians are likely to encounter patients who have spent time in tropical areas, it is important to still consider non-infectious causes of eosinophilia. Read More

J Thorac Oncol 2020 02;15(2):e29-e30

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

Scand J Rheumatol 2020 May 20;49(3):251-252. Epub 2020 Feb 20.

Department of Rheumatology, Odense University Hospital, Odense, Denmark.

Rheumatol Adv Pract 2020 28;4(1):rkaa001. Epub 2020 Jan 28.

Service de Médecine Interne, Centre Hospitalier Universitaire de Toulouse, Institut Universitaire du Cancer Toulouse Oncopole.

Cureus 2020 Jan 7;12(1):e6581. Epub 2020 Jan 7.

Rheumatology, Xuanwu Hospital, Beijing, CHN.

Eosinophilic fasciitis (EF) is an uncommon disease of unknown etiology and is characterized by inflammation and thickening of the muscular fascia and subcutaneous tissue. The patients often have peripheral eosinophilia, increased erythrocyte sedimentation rate, and hypergammaglobulinemia. In EF, the skin of the hands and feet are generally spared. Read More

Oncologist 2020 Feb 15;25(2):140-149. Epub 2019 Oct 15.

Department of Medicine, Hospital for Special Surgery, New York, New York, USA.

Background: Checkpoint inhibitor therapy is widely known to cause a number of immune-related adverse events. One rare adverse effect that is emerging is eosinophilic fasciitis, a fibrosing disorder causing inflammatory infiltration of subcutaneous fascia. It is characterized clinically by edema and subsequent induration and tightening of the skin and subcutaneous tissues. Read More

J Hand Surg Asian Pac Vol 2020 Mar;25(1):129-132

Victorian Hand Surgery Associates, Hand Unit, St. Vincent's Hospital Melbourne, Fitzroy, Australia.

Eosinophilic fasciitis (EF) is a rare form of fibrosing disorder associated with peripheral eosinophilia with scleroderma-like skin induration and fasciitis in the extremities resulting in painful swelling, erythema and progressive contracture. We present a case report of EF and a literature review to raise awareness of this unusual condition and also highlight key features in its management. Read More

Clin Rheumatol 2020 May 23;39(5):1693-1698. Epub 2020 Jan 23.

Department of Rheumatology, Parc de Salut Mar/Hospital del Mar, Passeig Marítim 25-29, 08003, Barcelona, Spain.

Eosinophilic fasciitis (EF) is a rare disorder involving chronic inflammation of the fascia and connective tissue of unknown aetiology and poorly understood pathogenesis. We present the case of a 60-year-old man diagnosed with eosinophilic fasciitis with extensive cutaneous involvement and severe functional repercussion, which appeared weeks after suffering from pneumonia due to Legionella pneumophila. The patient did not experience any clinical response with high-dose corticosteroids, subcutaneous methotrexate, and intravenous immunoglobulins. Read More

Clin Med (Lond) 2020 Jan;20(1):105-106

Imperial College Healthcare NHS Trust, London, UK

A 28-year-old man presented with a sudden onset of left leg pain and swelling. There was no history of preceding trauma. Initial blood tests were unremarkable, ultrasound imaging showed extensive subcutaneous oedema. Read More

Harefuah 2020 Jan;159(1):34-37

Department of Dermatology, Emek Medical Center, Afula, Israel.

Aims: This study aims to critically review the pros and cons of biological drugs as treatments and triggers of eosinophilic dermatoses.

Background: Eosinophilic dermatoses syndromes are rare diseases with a prominent eosinophilic infiltration mechanism. These syndromes have several known treatments with limited success. Read More

Open Access Maced J Med Sci 2019 Sep 15;7(18):2964-2968. Epub 2019 May 15.

University of Rome Guglielmo Marconi Rome, Italy.

Background: Eosinophilic fasciitis is a rare fibrosing disorder of muscle fascia with rapid onset of erythema, induration, oedema and tenderness affecting extremities bilaterally.

Case Report: We report three cases of eosinophilic fasciitis in 3 females aged 64, 65 and 73 years, in two of them in association with morphea. They fulfilled the proposed diagnostic criteria. Read More

J Am Acad Dermatol 2019 Dec 14. Epub 2019 Dec 14.

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

Clin Exp Rheumatol 2020 May-Jun;38(3):567-568. Epub 2019 Nov 18.

Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.

Int J Rheum Dis 2020 Feb 6;23(2):233-239. Epub 2019 Dec 6.

Section of Rheumatology, Allergy and Immunology, Yale University School of Medicine, New Haven, CT, USA.

Aim: Eosinophilic fasciitis (EF) is a rare, fibrosing disorder of skin and subcutaneous tissue. This study was undertaken to describe its clinical and laboratory features and identify prognostic factors associated with outcome.

Methods: We conducted a retrospective review of all EF patients evaluated at our institution from 1 January1997 to 30 December 2016. Read More

Oman Med J 2019 Nov;34(6):564-567

Department of Medicine, Rheumatology Unit, Royal Hospital, Muscat, Oman.

Eosinophilic fasciitis (EF) is a rare systemic inflammatory disease with an unknown etiology. Making a diagnosis in such a case is always a challenge as it is a rare disease and mimics scleroderma and scleroderma-like syndrome but should be kept in mind as it carries a high mortality. Furthermore, it is a treatable disease. Read More

J Clin Rheumatol 2019 Oct 29. Epub 2019 Oct 29.

The Ronald O. Perelman Department of Dermatology New York University School of Medicine New York, NY

Oncologist 2019 Oct 15. Epub 2019 Oct 15.

Department of Medicine, Hospital for Special Surgery, New York New York, USA.

Background: Checkpoint inhibitor therapy is widely known to cause a number of immune-related adverse events. One rare adverse effect that is emerging is eosinophilic fasciitis, a fibrosing disorder causing inflammatory infiltration of subcutaneous fascia. It is characterized clinically by edema and subsequent induration and tightening of the skin and subcutaneous tissues. Read More

Joint Bone Spine 2020 Mar 25;87(2):185-186. Epub 2019 Sep 25.

Service de médecine interne, centre hospitalo universitaire de Caen, avenue de la Côte-de-Nacre, 14033 Caen cedex 9, France. Electronic address:

J Dtsch Dermatol Ges 2019 10 27;17(10):1039-1051. Epub 2019 Sep 27.

Department of Dermatology, University Hospital, Jena, Germany.

Eosinophilic dermatoses are a heterogeneous group of diseases, characterized by an eosinophil-rich infiltrate and/or degranulation of eosinophils. Blood eosinophilia may be an associated feature. Typical, albeit not specific histological findings include 'flame figures', which are caused by the accumulation of cationic proteins released by eosinophils and subsequent collagen denaturation. Read More

Eur J Rheumatol 2020 01 5;7(1):52. Epub 2019 Sep 5.

Department of Rheumatology, All India Institute of Medical Sciences, New Delhi, India.

Dermatol Online J 2019 Aug 15;25(8). Epub 2019 Aug 15.

Department of Dermatology, University of California Davis Medical Center, Sacramento, CA Georgetown University School of Medicine, Washington, DC.

Eosinophilic fasciitis is a rare connective tissue disorder characterized by inflammation of the fascia that leads to painful, indurated skin. Because of its variable clinical presentation and overlap with conditions, such as morphea, the diagnosis of eosinophilic fasciitis can be challenging and relies on clinical presentation, histopathologic and laboratory analysis, and response to therapy. Herein, we present an unusual, solitary, isolated plaque with pathologic features and response to therapy most consistent with eosinophilic fasciitis. Read More

J Dermatol 2019 Dec 17;46(12):e474-e475. Epub 2019 Sep 17.

Department of Dermatology, Sapporo Medical University School of Medicine, Sapporo, Japan.

Clin Exp Rheumatol 2019 Jul-Aug;37 Suppl 119(4):149-150. Epub 2019 Sep 3.

Institute for Autoimmune Systemic and Neurological Diseases, Athens; Academy of Athens, and Department of Pathophysiology, Medical School, National and Kapodistrian University of Athens, Greece.

J Family Med Prim Care 2019 Jun;8(6):2158-2160

Department of Pathology, Kerala Institute of Medical Sciences, Trivandrum, Kerala, India.

Eosinophilic fasciitis (EF, also called Shulman syndrome) is a rare connective tissue disorder with poorly understood pathogenesis and unknown etiology. EF is characterized initially by limb or trunk erythema and edema and later by collagenous thickening of the subcutaneous fascia. Here, we present the case of a 16-year-old boy who presented with typical clinical features of EF with a history of typical trigger factor - preceding strenuous physical activity and had a rarer atypical association of hypercalcemia and raised angiotensin converting enzyme (ACE) levels. Read More

J Allergy Clin Immunol Pract 2019 Nov - Dec;7(8):2848-2849.e1. Epub 2019 Jul 13.

Internal Medicine Department, Hôpital de la Croix-Rousse, Lyon, France; Faculty of Medicine Lyon Sud Charles Mérieux, Université Claude Bernard Lyon 1, Oullins CEDEX, Lyon, France; Hospices Civils de Lyon, Lyon, France; University of Lyon, Université Claude Bernard Lyon 1, Lyon, France.

Internist (Berl) 2019 Aug;60(8):783-791

Hautklinik, Universitätsklinikum Erlangen, Ulmenweg 18, 91054, Erlangen, Deutschland.

Sclerosing skin manifestations are more a symptom than a diagnosis and must therefore be meticulously clarified. Systemic scleroderma as a multiorgan disease must be distinguished from localized scleroderma or morphea because in addition to a different clinical picture they have a different prognosis and necessitate other therapeutic procedures. Rare sclerosing skin diseases with implications for internal medicine are eosinophilic fasciitis, Buschke's scleredema adultorum, scleromyxedema and nephrogenic systemic fibrosis. Read More

Reumatologia 2019 29;57(2):85-90. Epub 2019 Apr 29.

Medicine Department, University Hospital Center of Porto, Portugal.

Objectives: Eosinophilic fasciitis (EF) was described in 1974 by Shulman as a rare fibrosing connective tissue disease of unknown etiology. An undetermined trigger is thought to lead to the degranulation of eosinophils that interact with fibroblasts and express fibrogenic cytokines including the transforming factor of tumor growth a and b and interleukins 1 and 6. The purpose of this study was to summarize seven cases of EF in a central hospital. Read More

Rheumatology (Oxford) 2019 10;58(10):1875-1877

Department of Dermatology, Universitätsklinikum Erlangen, Friedrich-Alexander-University Erlangen-Nuremberg (FAU), Erlangen, Germany.

Dermatol Ther 2019 07 25;32(4):e12926. Epub 2019 Apr 25.

Dermatology Department, Hospital General Universitario de Valencia, Valencia, Spain.

Eosinophilic fasciitis (EF) is an uncommon fibrosing disease of the fascia with characteristic cutaneous and hematologic manifestations. Although EF is most commonly treated with corticosteroids at the beginning, a considerable number of patients show an inadequate response and hence various therapeutic strategies have been tried, including extracorporeal photopheresis (ECP). We describe the case of a 66-year-old woman with steroid-resistant EF that improved significantly after ECP was added to her treatment regimen. Read More

J Autoimmun 2019 07 18;101:86-93. Epub 2019 Apr 18.

Department of Rheumatology and Clinical Immunology, University Medical Centre Utrecht, Utrecht, the Netherlands; Laboratory of Translational Immunology, University Medical Centre Utrecht, Utrecht, the Netherlands.

Background: Localized Scleroderma (LoS) encompasses a group of idiopathic skin conditions characterized by (sub)cutaneous inflammation and subsequent development of fibrosis. Currently, lack of accurate tools enabling disease activity assessment leads to suboptimal treatment approaches.

Objective: To investigate serum concentrations of cytokines and chemokines implicated in inflammation and angiogenesis in LoS and explore their potential to be utilized as biomarker of disease activity. Read More

Rev Assoc Med Bras (1992) 2019 Mar 11;65(3):326-329. Epub 2019 Apr 11.

Internal Medicine Service, Hospital de Braga, Braga, Portugal.

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. Read More

Z Rheumatol 2019 May;78(4):322-332

Klinik für Innere Medizin I, Sektion Rheumatologie, Exzellenzzentrum Entzündungsmedizin, Universitätsklinikum Schleswig-Holstein, Campus Kiel, Arnold-Heller-Str. 3, 24105, Kiel, Deutschland.

Among the eosinophilic diseases treated by rheumatologists other than eosinophilic granulomatosis with polyangiitis, there are further organ-related and systemic diseases with hypereosinophilia. Only the exact differential diagnostic demarcation of the diseases enables a pathogenetic oriented treatment. This article focuses on the hypereosinophilic syndromes. Read More

Allergol Int 2019 Oct 22;68(4):437-439. Epub 2019 Mar 22.

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

Eosinophilic fasciitis is a disease originally proposed as "diffuse fasciitis with eosinophilia" by Shulman in 1974. The patients with this disease often have history of strenuous exercise or labor a few days to 1-2 weeks before the onset. The chief symptoms are symmetrical, full-circumference swelling and plate-like hardness of the distal limbs. Read More

Mayo Clin Proc 2019 04 6;94(4):704-708. Epub 2019 Feb 6.

Advisor to residents and Consultant in General Internal Medicine, Mayo Clinic, Rochester, MN. Electronic address:

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 2019 Aug 27;60(3):e227-e231. 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 01;14(1):e13-e15

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