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Neuromuscul Disord 2022 May 17. Epub 2022 May 17.

Department of Neurology, Tokushima University Hospital, Tokushima, Japan.

Eosinophilic fasciitis (EF) is a rare disorder characterized by muscle stiffness mimicking other neuromuscular diseases. The diagnosis of EF is made on the basis of typical skin lesions. We report a case of a 36-year-old male patient with suspected stiff-person syndrome (SPS), who presented with progressive limb muscle stiffness and limited mobility of both wrists without obvious skin changes. Read More

J Immunother Precis Oncol 2022 May 20;5(2):48-51. Epub 2022 Jan 20.

Department of Dermatology, University of Missouri, Columbia, MO, USA.

Immune checkpoint inhibitors (ICIs) have revolutionized cancer therapeutics. However, loss of physiologic tolerance in few cases has triggered rare and novel immune-related adverse events (irAEs). Eosinophilic fasciitis, an infrequently reported diffuse scleroderma-like entity, has been associated with ICI therapy. Read More

J Belg Soc Radiol 2022 24;106(1):48. Epub 2022 May 24.

Department of Radiology, AZ Sint Maarten, Mechelen, Belgium.

Eosinophilic fasciitis (EF) is a rare sclerodermiform disease characterized by upper- and lower-limbs oedema and hardness, which should be confirmed by skin biopsy and MRI in case of clinical suspicion. Read More

Z Rheumatol 2022 May 16. Epub 2022 May 16.

Medizinische Klinik III, Onkologie, Hämatologie, Rheumatologie und klinische Immunologie, Universitätsklinikum Bonn, Venusberg Campus 1, 53127, Bonn, Deutschland.

Eosinophilic fasciitis (EF, also known as Shulman syndrome) is an uncommon connective tissue disease characterized by inflammatory thickening of the fasciae as well as swelling and hardening of the skin. It mostly affects the lower extremities. Swollen and indurated skin, together with the groove sign, are typical clinical signs. Read More

Expert Rev Clin Immunol 2022 May 19:1-3. Epub 2022 May 19.

Bait Balev Nesher and the Ruth and Bruce Rappaport Faculty of Medicine, Technion, Israel Institute of Technology, Haifa, Israel.

Int J Dermatol 2022 May 15. Epub 2022 May 15.

Department of Dermatology, Dokkyo Medical University School of Medicine, Mibu, Tochigi, Japan.

Cureus 2022 Apr 6;14(4):e23896. Epub 2022 Apr 6.

Department of Neurology, Cheikh Khalifa International University Hospital, Faculty of Medicine, Mohammed VI University of Health Sciences (UM6SS), Casablanca, MAR.

Eosinophilic fasciitis (EF) or Shulman's fasciitis is a rare condition characterized by subcutaneous edematous induration sparing the face and distal extremities and progressing to skin sclerosis. Its association with other pathologies, notably hemopathies, is described in the literature, but its association with smoldering multiple myeloma remains very rare, especially in a younger subject. Read More

Intern Med 2022 Apr 30. Epub 2022 Apr 30.

Department of Hematology, Nagano Red Cross Hospital, Japan.

A 58-year-old man presented with painful edema of the extremities, and a diagnosis of eosinophilic fasciitis (EF) was confirmed. He also met the criteria for hypereosinophilic syndrome (HES), but there were no findings suggestive of malignancies or hematologic neoplasms despite a close examination. He was started on steroid therapy but subsequently developed severe liver dysfunction, hemophagocytic lymphohistiocytosis, hepatosplenomegaly, and renal involvement. Read More

JAAD Case Rep 2022 May 26;23:79-82. Epub 2022 Mar 26.

Department of Dermatology, University of Rochester Medical Center, Rochester, New York.

BMJ Case Rep 2022 Apr 6;15(4). Epub 2022 Apr 6.

Dermatology, St Vincent's Hospital Melbourne Pty Ltd, Fitzroy, Victoria, Australia.

Dystrophic calcinosis cutis can be associated with severe pain, decreased mobility, increased risk of infection and significantly decreased quality of life. We report a case of recalcitrant calcinosis cutis on the background of eosinophilic fasciitis, which achieved rapid reduction in calcium deposits following a novel injecting protocol of intralesional sodium thiosulfate. Read More

J Scleroderma Relat Disord 2021 Jun 26;6(2):211-213. Epub 2021 Mar 26.

Department of Dermatology, Venereology and Allergology, Medical University of Gdańsk, Gdańsk, Poland.

Eosinophilic fasciitis is a rare connective tissue disease of unknown etiology. Therapeutic options include high-dose corticosteroids and other immunosuppressive drugs. We present a typical eosinophilic fasciitis case, which did not respond to first-line treatment, but improved remarkably after infliximab administration. Read More

Rev Clin Esp (Barc) 2022 Mar 16. Epub 2022 Mar 16.

Servicio de Farmacia, Hospital Universitario Cabueñes, Gijón, Asturias, Spain.

JAAD Case Rep 2022 Apr 23;22:74-77. Epub 2022 Feb 23.

Corporal Michael J. Crescenz Veterans Affairs Medical Center, Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania.

Mediterr J Rheumatol 2021 Dec 27;32(4):376-377. Epub 2021 Dec 27.

7 Pulmonary Department and Central Bronchoscopic Unit "Christos Gagas" NHS, Athens Chest Hospital "Sotiria", Athens, Greece.

With the increasing number of indications for checkpoint inhibitor therapy in cancer patients, rheumatology specialists are often involved in the diagnosis and management of immune-related adverse events (irAEs). The most common rheumatic irAEs are arthritis, sicca syndrome, polymyalgia rheumatica, and myositis. Eosinophilic fasciitis, an already rare rheumatic disease, is a very unusual rheumatic irAE. Read More

Eur J Dermatol 2021 Dec;31(6):822-829

Department of Dermatology, Venereology, and Allergology, University Medical Center Göttingen, Germany, Robert Koch Str. 40, D-37075 Göttingen, Germany.

Background: Generalized morphea and eosinophilic fasciitis are difficult-to-treat inflammatory and sclerosing skin diseases. Few cases have been reported in which intravenous immunoglobulins were of benefit, possibly owing to their immunomodulatory and antifibrotic properties.

Objectives: We present three new patients with generalized morphea treated with intravenous immunoglobulins as well as a review of the literature. Read More

Acute Med 2021 ;20(4):282-285

MB BCh (Cantab), MA (Cambridge), BM (Oxon), PhD, FRCP (London) Consultant in Rheumatology, Department of Rheumatology, Addenbrooke's Hospital, Cambridge University Hospitals Trust, UK.

Bilateral lower limb swelling is a common clinical scenario with a wide differential diagnosis. We present a young man with gradually worsening bilateral leg swelling, who was diagnosed with eosinophilic fasciitis. A 20 year old Hispanic male presented with a six week history of bilateral lower limb pain and swelling, later involving the upper limbs, but sparing the hands, feet and face. Read More

Int J Rheum Dis 2022 Mar 13;25(3):364-366. Epub 2022 Jan 13.

Department of Dermatology, Mie University Graduate School of Medicine, Tsu, Japan.

We report a case of eosinophilic fasciitis triggered by strenuous physical activity, which did not relapse during the follow-up period. We ascertained that interleukin-33 (IL-33) was released from the vascular endothelial cells after intense exercise, inducing type 2 innate lymphocytes (ILC2) and causing fasciitis. A healthy woman experienced itching on both limbs a few hours after a game of drumming. Read More

BMJ Case Rep 2021 Dec 30;14(12). Epub 2021 Dec 30.

Department of Radiology, Hospital da Luz, Lisboa, Lisboa, Portugal.

Eosinophilic fasciitis (EF) is a rare subacute fibrosing disorder of unknown aetiology, characterised by thickening of the muscular fascia and subcutaneous tissue, leading to swelling of limbs and trunk and sparing fingers and toes. Eosinophilic infiltration and degranulation may prompt tissue damage and consequent fibrosis due to the accumulation of collagen and extracellular matrix proteins. MRI is the best imaging modality for diagnosis, depicting fascial thickening and enhancement. Read More

J Rheumatol 2022 05 15;49(5):546-547. Epub 2021 Dec 15.

Department of Rheumatology and Immunology, Zhuzhou Hospital Affiliated to Xiangya Medical College, Central South University, Zhuzhou.

Eur J Dermatol 2021 Dec;31(6):844-846

Department of Dermatology, Limoges University Hospital, Limoges, France.

Int J Dermatol 2022 Jun 8;61(6):718-726. Epub 2021 Dec 8.

Dermatology Unit and Skin Bank Unit, Department of Clinical, Surgical and Neuro-sciences, University of Siena, Italy.

Introduction: Eosinophilic fasciitis (EF) is a rare autoimmune disease causing progressive induration of dermal, hypodermal, and muscularis fascia. The exact pathogenesis is yet to be fully understood, and a validated therapy protocol still lacks. We here aimed to realize a clinical-functional characterization of these patients. Read More

Postepy Dermatol Alergol 2021 Oct 18;38(5):716-720. Epub 2021 May 18.

Department of Dermatology and Venereology, Poznan University of Medical Sciences, Poznan, Poland.

Morphea, also known as localized scleroderma, is a chronic, autoimmune disease of connective tissue. It is characterized by a typical clinical feature. In morphea, there is no Raynaud's phenomenon, no sclerodactyly or no ulcerations on the fingertips. Read More

Dermatol Online J 2021 Aug 15;27(8). Epub 2021 Aug 15.

Department of Dermatology, Venereology and Allergology, University Medical Center, Göttingen.

Eosinophilic fasciitis (EF) and generalized morphea (GM) are rare and difficult-to-treat sclerosing skin diseases which may occur in association with hematologic disorders. We present a 66-year-old man with EF and associated Waldenström macroglobulinemia who received combination therapy with rituximab (375mg/m2 every other week, gradually extended to every eight weeks), prednisolone (1.25-30mg/d), and methotrexate (7. Read More

World J Clin Cases 2021 Oct;9(29):8831-8838

Department of Thoracic Surgery, Shengjing Hospital of China Medical University, Shenyang 110004, Liaoning Province, China.

Background: Eosinophilic fasciitis (EF) is a rare disease characterized by inflammation of the fascia with immune system involvement. Failure to promptly diagnose and treat this disease can seriously affect the quality of life of patients. However, no clear and uniform criteria for diagnosis and treatment exist. Read More

Nature 2021 11 27;599(7886):667-672. Epub 2021 Oct 27.

Department of Dermatology, University of California at San Franscisco, San Francisco, CA, USA.

Inflammation early in life can prime the local immune milieu of peripheral tissues, which can cause lasting changes in immunological tone that confer disease protection or susceptibility. The cellular and molecular mechanisms that prompt changes in immune tone in many nonlymphoid tissues remain largely unknown. Here we find that time-limited neonatal inflammation induced by a transient reduction in neonatal regulatory T cells causes a dysregulation of subcutaneous tissue in mouse skin. Read More

JAMA Dermatol 2021 12;157(12):1508-1509

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

JAAD Case Rep 2021 Nov 29;17:49-51. Epub 2021 Sep 29.

Division of Dermatology, University of Washington, Seattle, Washington.

Am J Med 2022 02 22;135(2):187-190. Epub 2021 Sep 22.

Department of Internal Medicine and Infectious Diseases, Cliniques Universitaires Saint Luc, Catholic University of Louvain, Brussels, Belgium. Electronic address:

J Med Cases 2021 Jun 24;12(6):220-222. Epub 2021 Mar 24.

Department of Medicine, University of California, Riverside School of Medicine/Riverside Community Hospital, Riverside, CA, USA.

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. Read More

Naunyn Schmiedebergs Arch Pharmacol 2021 11 19;394(11):2233-2244. Epub 2021 Aug 19.

Institute of Pharmacology and Toxicology, Faculty of Medicine Carl Gustav Carus, Technische Universität Dresden, Fiedlerstraße 42, 01309, Dresden, Germany.

Skin fibrosis is a complex biological remodeling process occurring in disease like systemic sclerosis, morphea, or eosinophilic fasciitis. Since the knowledge about the underlying pathomechanisms is still incomplete, there is currently no therapy, which prevents or reverses skin fibrosis sufficiently. The present study investigates the role of polo-like kinase 2 (PLK2) and the pro-fibrotic cytokine osteopontin (OPN) in the pathogenesis of cutaneous fibrosis and demonstrates the antifibrotic effects of systemic mesalazine treatment in vivo. Read More