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Eosinophilic fasciitis

Aseem Sharma, Rahul Ray, Jandhyala Sridhar, Arti Trehan, Manish Khandare
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. A well-defined hypopigmented indurated plaque was present over the left iliac region. Histopathology and imaging studies confirmed the diagnosis of eosinophilic fasciitis and lichen sclerosus...
September 2016: Indian Dermatology Online Journal
A Pituch-Noworolska, H Mach-Tomalska, A Szaflarska, D Adamek
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. Autoimmune diseases as co-existing diseases in XLA are noted in a few patients presenting symptoms associated with arthritis, scleroderma and myositis...
June 2016: Polish Journal of Pathology: Official Journal of the Polish Society of Pathologists
Jorre S Mertens, Manon C Zweers, Wietske Kievit, Hanneke K A Knaapen, Martijn Gerritsen, Timothy R D J Radstake, Frank H J van den Hoogen, Marjonne C W Creemers, Elke M G J de Jong
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. Design, Setting, and Participants: For this prospective single-arm study, we recruited 12 patients diagnosed with biopsy specimen-proven EF between 2006 and 2009 from the Department of Dermatology and Rheumatology at the Radboud University Medical Centre...
August 17, 2016: JAMA Dermatology
Koji Sasaki, Uday Popat, Preetesh Jain, Tapan Kadia, Krina Patel, Keyur Patel, Nitin Jain, Koichi Takahashi, Ken Young, Roberto N Miranda, Thein H Oo, Huifang Lu, Naveen Pemmaraju
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.
August 2016: Clinical Case Reports
Ryota Kurimoto, Kei Ikeda, Daiki Nakagomi, Hiroshi Nakajima
No abstract text is available yet for this article.
2016: Internal Medicine
Marta Oliveira, Fabia Patinha, Antonio Marinho
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. This paper presents a case of a male patient with palpebral edema and marked eosinophilia, diagnosed with intestinal parasitic infection in October 2006...
2016: Reumatologia
Richard Hariman, Payal Patel, Jennifer Strouse, Michael P Collins, Ann Rosenthal
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. To our knowledge, eosinophilic fasciitis has not been previously described in patients during treatment with an inhibitor of tumor necrosis factor α...
2016: Case Reports in Rheumatology
Riccardo Papa, Paolo Nozza, Claudio Granata, Roberta Caorsi, Marco Gattorno, Alberto Martini, Paolo Picco
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...
May 2016: Clinical and Experimental Rheumatology
Justin Endo, Nicole Strickland, Simer Grewal, Travis Vandergriff, Thomas Keenan, B Jack Longley, Heidi Jacobe
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. We describe four patients with morphea profunda that were associated with monoclonal gammopathy. Three were associated with monoclonal IgG protein and one with IgM...
2016: Dermatology Online Journal
Shino Minami, Noriki Fujimoto, Toshifumi Takahashi, Takeshi Nakanishi, Toshihiro Tanaka
No abstract text is available yet for this article.
June 1, 2016: European Journal of Dermatology: EJD
Vikash S Oza, Rabina Walsh, Jeffrey North, Timothy G Berger, Jenny E Murase
No abstract text is available yet for this article.
April 2016: JAMA Dermatology
Leila Khoja, Catherine Maurice, MaryAnne Chappell, Leslie MacMillan, Ayman S Al-Habeeb, Nada Al-Faraidy, Marcus O Butler, Patrik Rogalla, Warren Mason, Anthony M Joshua, David Hogg
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. Here, we report the case of a 51-year-old woman treated with pembrolizumab, as part of the Keynote-001 trial, as first-line treatment for metastatic disease. She experienced a complete response after 13.8 months of treatment with no adverse events. One month after the last drug infusion and 18 months from starting treatment, the patient presented with eosinophilic fasciitis...
March 2016: Cancer Immunology Research
Chih-Wei Chang, Matthew S Lau
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. This is a case of eosinophilic fasciitis presenting in atypical fashion with pitting peripheral edema in addition to the classic symptoms...
September 2015: Hawai'i Journal of Medicine & Public Health: a Journal of Asia Pacific Medicine & Public Health
Tulin Ergun, Dilek Seckin, Andac Salman, Esra Sarac Ocak, Ayse Deniz Yucelten, Haner Direskeneli, Cuyan Demirkesen, Gazanfer Ekinci, Mahmut Bayik
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. Magnetic resonance imaging (MRI) was used for diagnosis in three patients and for assessing treatment response in one patient. Eosinophilic fasciitis may co-exist with morhoea and lichen sclerosus...
December 14, 2015: Indian Journal of Dermatology, Venereology and Leprology
Natalie A Wright, Daniel R Mazori, Mital Patel, Joseph F Merola, Alisa N Femia, Ruth Ann Vleugels
No abstract text is available yet for this article.
January 2016: JAMA Dermatology
F A Mendoza, R Bai, A G Kebede, S A Jimenez
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. CS alone in patients with severe eosinophilic fasciitis, defined as clinically apparent cutaneous fibrotic involvement affecting more than 15% body surface area (BSA) or more than 10% BSA with joint flexion contractures. RESULTS: Sixteen patients with severe eosinophilic fasciitis entered the study...
2016: Scandinavian Journal of Rheumatology
D Sène
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. At the onset, the morphological diagnosis might be helped by a muscle magnetic resonance imaging, which typically may evidence an increased signal intensity within the fascia and marked fascia enhancement after gadolinium administration at the acute phase of the disease...
November 2015: La Revue de Médecine Interne
C Bachmeyer, P Moguelet, C Rein, R Girot
No abstract text is available yet for this article.
June 2016: Clinical and Experimental Dermatology
M N Shazzad, A K Azad, S J Abdal, R Afrose, M M Rahman, S A Haq
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. It may be classified into three clinical groups, each has a different history, course, and prognosis. Histology of skin is characterized by thickened dermis and increased spaces between large collagen bundles due to increased deposition of mucopolysaccharide in the dermis...
July 2015: Mymensingh Medical Journal: MMJ
Sumantro Mondal, Rudra Prosad Goswami, Debanjali Sinha, Alakendu Ghosh
No abstract text is available yet for this article.
November 2015: Rheumatology
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