Eosinophilic fasciitis: demographics, disease pattern and response to treatment: report of 12 cases and review of the literature
Identifieur interne : 001A06 ( Main/Exploration ); précédent : 001A05; suivant : 001A07Eosinophilic fasciitis: demographics, disease pattern and response to treatment: report of 12 cases and review of the literature
Auteurs : Lindsay Bischoff [États-Unis] ; Chris T. Derk [États-Unis]Source :
- International Journal of Dermatology [ 0011-9059 ] ; 2008-01.
English descriptors
- Teeft :
- Biopsy, Bischoff, Carpal tunnel syndrome, Clinical spectrum, Contracture, Corticosteroid, Cutaneous, Cutaneous manifestations, Derk, Dermatology, Dermatology bischoff, Diffuse fasciitis, Eosinophil, Eosinophilic, Eosinophilic fasciitis, Extracutaneous, Extracutaneous manifestations, Family history, Fasciitis, Full thickness skin, Hydroxychloroquine, Induration, Inflammatory, Inflammatory arthritis, International journal, International society, Joint contractures, Laboratory results, Monoclonal gammopathy, Months prednisone, Muscle biopsy, Other diseases, Other studies, Pack year history, Peau, Peripheral eosinophilia, Rare disease, Scleroderma, Scleroderma center, Scleroderma clinic, Serum protein electrophoresis, Skin induration, Skin involvement, Syndrome, Upper extremity involvement.
Abstract
Background Eosinophilic fasciitis is a rare scleroderma‐like illness. The clinical spectrum of the disease has evolved since its initial description. Methods We identified all patients diagnosed with eosinophilic fasciitis over the past 10 years at our scleroderma clinic. Demographics, disease pattern, serologies, tissue pathology and reponse to treatment were all recorded. Results Twelve patients with eosinophilic fasciitis were identified in our clinic over the past 10 years. The mean age at diagnosis was 49.8 ± 9.8 years, with nine female and three male patients. The first symptoms were noticed at an average of 8.8 ± 6.1 months before diagnosis. The mean initial absolute peripheral blood eosinophil count was 1188 ± 1059 cells/L. Two patients had a monoclonal gammopathy, and two had positive ANA titers. All patients received corticosteroids, 10 of whom received the equivalent dose of > 20 mg/day of prednisone for more than a month. Five patients received hydroxychloroquine, two received methotrexate, one received cyclosporine, one received topical tacrolimus, and one received sulfasalazine. At a mean follow up of 17.6 months (range 2–94 months), 8 patients had a good response to treatment, 2 patients had no effect, and 2 patients had a poor response to treatment. Conclusion High dose corticosteroid treatment lasting longer than a month with or without an immunosuppressive agent helped most patients with eosinophilic fasciitis, best results seen in those patients who were initiated treatment early on after their first symptoms.
Url:
DOI: 10.1111/j.1365-4632.2007.03544.x
Affiliations:
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Derk</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>From the Jefferson Medical College, Philadelphia PA, and Division of Rheumatology, Thomas Jefferson University, Philadelphia, PA 19107</wicri:regionArea><placeName><region type="state">Pennsylvanie</region></placeName></affiliation></author></analytic><monogr></monogr><series><title level="j" type="main">International Journal of Dermatology</title><title level="j" type="alt">INTERNATIONAL JOURNAL OF DERMATOLOGY</title><idno type="ISSN">0011-9059</idno><idno type="eISSN">1365-4632</idno><imprint><biblScope unit="vol">47</biblScope><biblScope unit="issue">1</biblScope><biblScope unit="page" from="29">29</biblScope><biblScope unit="page" to="35">35</biblScope><biblScope unit="page-count">7</biblScope><publisher>Blackwell Publishing Ltd</publisher><pubPlace>Oxford, UK</pubPlace><date type="published" when="2008-01">2008-01</date></imprint><idno type="ISSN">0011-9059</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0011-9059</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="Teeft" xml:lang="en"><term>Biopsy</term><term>Bischoff</term><term>Carpal tunnel syndrome</term><term>Clinical spectrum</term><term>Contracture</term><term>Corticosteroid</term><term>Cutaneous</term><term>Cutaneous manifestations</term><term>Derk</term><term>Dermatology</term><term>Dermatology bischoff</term><term>Diffuse fasciitis</term><term>Eosinophil</term><term>Eosinophilic</term><term>Eosinophilic fasciitis</term><term>Extracutaneous</term><term>Extracutaneous manifestations</term><term>Family history</term><term>Fasciitis</term><term>Full thickness skin</term><term>Hydroxychloroquine</term><term>Induration</term><term>Inflammatory</term><term>Inflammatory arthritis</term><term>International journal</term><term>International society</term><term>Joint contractures</term><term>Laboratory results</term><term>Monoclonal gammopathy</term><term>Months prednisone</term><term>Muscle biopsy</term><term>Other diseases</term><term>Other studies</term><term>Pack year history</term><term>Peau</term><term>Peripheral eosinophilia</term><term>Rare disease</term><term>Scleroderma</term><term>Scleroderma center</term><term>Scleroderma clinic</term><term>Serum protein electrophoresis</term><term>Skin induration</term><term>Skin involvement</term><term>Syndrome</term><term>Upper extremity involvement</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Background Eosinophilic fasciitis is a rare scleroderma‐like illness. The clinical spectrum of the disease has evolved since its initial description. Methods We identified all patients diagnosed with eosinophilic fasciitis over the past 10 years at our scleroderma clinic. Demographics, disease pattern, serologies, tissue pathology and reponse to treatment were all recorded. Results Twelve patients with eosinophilic fasciitis were identified in our clinic over the past 10 years. The mean age at diagnosis was 49.8 ± 9.8 years, with nine female and three male patients. The first symptoms were noticed at an average of 8.8 ± 6.1 months before diagnosis. The mean initial absolute peripheral blood eosinophil count was 1188 ± 1059 cells/L. Two patients had a monoclonal gammopathy, and two had positive ANA titers. All patients received corticosteroids, 10 of whom received the equivalent dose of > 20 mg/day of prednisone for more than a month. Five patients received hydroxychloroquine, two received methotrexate, one received cyclosporine, one received topical tacrolimus, and one received sulfasalazine. At a mean follow up of 17.6 months (range 2–94 months), 8 patients had a good response to treatment, 2 patients had no effect, and 2 patients had a poor response to treatment. Conclusion High dose corticosteroid treatment lasting longer than a month with or without an immunosuppressive agent helped most patients with eosinophilic fasciitis, best results seen in those patients who were initiated treatment early on after their first symptoms.</div></front></TEI><affiliations><list><country><li>États-Unis</li></country><region><li>Pennsylvanie</li></region></list><tree><country name="États-Unis"><region name="Pennsylvanie"><name sortKey="Bischoff, Lindsay" sort="Bischoff, Lindsay" uniqKey="Bischoff L" first="Lindsay" last="Bischoff">Lindsay Bischoff</name></region><name sortKey="Derk, Chris T" sort="Derk, Chris T" uniqKey="Derk C" first="Chris T." last="Derk">Chris T. Derk</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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