Pediatric eosinophilic fasciitis: similarities and differences with adult forms

被引：0

作者：

Cesar, Thibaut ^{[1
]}

Giovannini, Diane ^{[2
,5
]}

Deroux, Alban ^{[3
]}

Bouillet, Laurence ^{[3
,5
]}

Nugues, Frederique ^{[4
]}

Carbasse, Aurelia ^{[6
]}

Kone-Paut, Isabelle ^{[7
,8
]}

Borocco, Charlotte ^{[7
,8
]}

Pietrement, Christine ^{[9
]}

Bader-Meunier, Brigitte ^{[10
,11
]}

Quartier, Pierre ^{[10
,11
]}

Laurent, Audrey ^{[12
]}

Giani, Teresa ^{[13
]}

Bruck, Normi ^{[14
]}

Calzada-Hernandez, Joan ^{[15
]}

Pagnier, Anne ^{[1
]}

Kevorkian-Verguet, Charlotte ^{[1
]}

GKJR

机构：

[1] Grenoble Alpes Univ Hosp, Dept Pediat, Grenoble, France

[2] Grenoble Alpes Univ Hosp, Pathol Dept, Grenoble, France

[3] Grenoble Alpes Univ Hosp, Internal Med Dept, Grenoble, France

[4] Grenoble Alpes Univ Hosp, Pediat Radiol, Grenoble, France

[5] Grenoble Alpes Univ, UMR 5525, TIMC IMAG, CNRS, La Tronche, France

[6] Montpellier Univ Hosp, Dept Pediat, Montpellier, France

[7] Paris Saclay Univ, Bicetre Hosp, Assistance Publ Hop Paris, Paris, France

[8] Bicetre Hosp, Assistance Publ Hop Paris, Dept Pediat Rheumatol, Paris, France

[9] Reims Univ Hosp, Dept Pediat, Reims, France

[10] Paris Cite Univ, Paris, France

[11] Necker Univ Hosp, Assistance Publ Hop Paris APHP, Pediat Hematol Immunol & Rheumatol Dept, Paris, France

[12] Hosp Civils Lyon, Dept Pediat Rheumatol, Lyon, France

[13] Anna Meyer Children Hosp, Dept Rheumatol, Florence, Italy

[14] Carl Gustav Carus Univ Hosp, Dept Pediat Rheumatol, Dresden, Germany

[15] Hosp San Joan Deu, Dept Pediat Rheumatol, Barcelona, Spain

来源：

EUROPEAN JOURNAL OF PEDIATRICS | 2025年 / 184卷 / 02期

关键词：

Pediatric eosinophilic fasciitis; Hypergammaglobulinemia; MRI; DIFFUSE FASCIITIS; SHULMAN DISEASE; LOCALIZED SCLERODERMA; CLINICAL SPECTRUM; DIAGNOSIS; SERIES; HYPERGAMMAGLOBULINEMIA; PRESENTATIONS; ABNORMALITIES; INVOLVEMENT;

D O I：

10.1007/s00431-025-05981-5

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

The purpose of this study was to identify pediatric eosinophilic fasciitis, which is an extremely rare condition, in order to describe their clinical, paraclinical, and therapeutic characteristics. We made a call for observations via societies for pediatric rheumatology in France and surrounding countries and collected clinical and paraclinical data of the cases fulfilling the diagnostic criteria. Eight patients under 18 years of age with confirmed eosinophilic fasciitis followed between April 2004 and July 2022 in France, Germany, Italy, and Spain were included. The median age of onset of symptoms was 8.7 years (range 3 to 12.6). All patients had skin and joint involvement at diagnosis. Eosinophilia was present at diagnosis in 5/8 patients and 5/7 patients presented hypergammaglobulinemia. All the patients had an MRI, and in 6, we observed thickened fascia with a T2 hypersignal. Five patients had undergone a full-thickness biopsy showing a polymorphic lymphoplasmacytic infiltrate of the fascia in all and the presence of eosinophils in 4 of them. All the patients were treated with corticosteroids with variable regimens and all received at least an immunosuppressant.Conclusion: To our knowledge, this is the largest pediatric series of eosinophilic fasciitis. The clinical and paraclinical presentation seems similar to that of adults except for a form that appears to be distinguished with isolated joint contractures, and hypergammaglobulinemia which appears to be more frequent in children. Dermatological and pathological expertise and MRI are key elements of the diagnosis. The most consensual treatment includes physiotherapy, prolonged corticosteroid therapy, and methotrexate as first-line therapy. What is Known center dot Eosinophilic fasciitis is a rare condition, especially in children with approximately 60 reported pediatric cases.center dot Some pediatric specificities tend to emerge from some reports.What is New center dot Possible isolated joints contractures and more frequent hypergammaglobulinemia seem to characterize pediatric eosinophilic fasciitis in comparison with adult forms.center dot The most frequently used first-line therapy combines physiotherapy, corticosteroids and methrotrexate.

引用

页数：11

共 72 条

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