Academic Journal

The Spectrum of Eosinophilic Cellulitis (Wells' Syndrome): New Insights Based on 114 Cases.

Λεπτομέρειες βιβλιογραφικής εγγραφής
Τίτλος: The Spectrum of Eosinophilic Cellulitis (Wells' Syndrome): New Insights Based on 114 Cases.
Συγγραφείς: Castro A; Department of Internal Medicine, Hôpital André Mignot, Le Chesnay-Rocquencourt, France; Université de Versailles Saint Quentin-en-Yvelines, National Reference Center for Hypereosinophilic Syndromes, Department of Internal Medicine, Clinical Immunology and Hematology, Hôpital Foch, Suresnes, France., Osio A; Department of Pathology, APHP Hôpital Saint Louis, Université Paris Cité, INSERM U1342, Paris, France., Landais C; Delegation of Clinical Research and Innovation, Hôpital Foch, Suresnes, France., Shourick J; Epidemiology Department, Toulouse University Hospital, Toulouse, France., Aouba A; Department of Internal Medicine, University Hospital Caen-Normandie, Caen, France., Aubert H; Department of Dermatology, CHU Nantes, Nantes, France., Bernier M; Department of Pathology, Foch Hospital, Suresnes, France., Bogiatzi S; Department of Pathology, APHP Hôpital Saint Louis, Université Paris Cité, INSERM U1342, Paris, France., Bohelay G; Department of Dermatology, Avicenne University Hospital, AP-HP, Sorbonne-Paris-Nord University, Bobigny, France., Charvet E; Department of Dermatology, Hôpital Saint Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France., Chastagner M; Department of Dermatology, Hospices Civils de Lyon, Edouard Herriot Hospital, Lyon, France., Chosidow O; Department of ENT, Facial Dermatosis Clinic, Pitié-Salpêtrière University Hospital, Paris, France., Comoz F; Department of Pathology, CHU de Caen, Caen, France., Delmotte A; Department of Dermatology, Hôpital Saint Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France., Denis-Musquer M; Department of Pathology, Hôpital-Dieu, Nantes, France., Descamps V; Department of Dermatology, Hôpital Bichat, AP-HP, Paris, France., Deschamps L; Pathology Department, AP-HP, Bichat-Claude Bernard Hospital, Paris, France., Dezoteux F; CHU Lille, Univ. Lille, Service de Dermatologie, U1286 Inserm INFINITE Institute for Translational Research in Inflammation, Lille, France., Dupin N; Dermatology Department, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France., Emile JF; Pathology Unit, Ambroise Paré Hospital, APHP, Université de Versailles SQY, Boulogne-Billancourt, France., Gibier JB; Department of Pathology, Lille University Hospital, Lille, France., Grolleau C; Department of Dermatology, Hôpital Saint Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France., Habougit C; Pathology Department, University Hospital of Saint-Etienne, Saint-Etienne, France., Jachiet M; Department of Dermatology, Hôpital Saint Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France., Jullie ML; Pathology Department, Bordeaux University Hospital, Haut-Lévêque Hospital, Pessac, France., Kahn JE; Internal Medicine Department, Ambroise-Paré University Hospital, AP-HP Paris Saclay, Boulogne-Billancourt, France., Kanitakis J; Department of Dermatology, Hospices Civils de Lyon, Edouard Herriot Hospital, Lyon, France., Killian M; Department of Internal Medicine, CHU Saint-Etienne, Saint-Etienne, France., Lefèvre G; Department of Internal Medicine and Clinical Immunology, CHU Lille, Lille, France., Livideanu CB; Department of Dermatology, Hôpital Larrey, Toulouse University Hospital, Toulouse, France., Moguelet P; Pathology Department, Hôpital Tenon, Assistance Publique-Hôpitaux de Paris, Paris, France., Ortonne N; Pathology Department, Henri Mondor Hospital, AP-HP, Créteil, France., Paul C; Department of Dermatology, CHU and Toulouse III University, Toulouse, France., Perrot JL; Department of Dermatology, University Hospital of Saint-Etienne, Saint-Etienne, France., Poisot T; Paris Cité University, INSERM U1342, Research Institute of Saint-Louis, Paris, France., Rakotoarivelo HN; Department of Internal Medicine, CH de Colmar, Colmar, France., Seneschal J; Department of Dermatology and Pediatric Dermatology, National Reference Center for Rare Skin Disorders, Hôpital Saint-André, Bordeaux, France., Sohier P; Pathology Department, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France., Villani AP; Department of Dermatology, Edouard Herriot Hospital, Hospices Civils de Lyon, Claude Bernard Lyon I University, Lyon, France., Bouaziz JD; Department of Dermatology, Hôpital Saint Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France; Paris Cité University, INSERM U1342, Research Institute of Saint-Louis, Paris, France., Staumont-Sallé D; Department of Dermatology, Lille University Hospital, U1286 Inserm INFINITE, Lille University, Lille, France., Battistella M; Department of Pathology, APHP Hôpital Saint Louis, Université Paris Cité, INSERM U1342, Paris, France., Chasset F; Department of Dermatology, Hôpital Tenon, Assistance Publique - Hôpitaux de Paris, Sorbonne Université, Faculté de médecine, INSERM U1135, CIMI, Paris, France., Groh M; Université de Versailles Saint Quentin-en-Yvelines, National Reference Center for Hypereosinophilic Syndromes, Department of Internal Medicine, Clinical Immunology and Hematology, Hôpital Foch, Suresnes, France; Department of Internal Medicine, CH de Colmar, Colmar, France. Electronic address: m.groh@hopital-foch.com.
Συλλογικό Έργο: CEREO (French National Reference Center for Hypereosinophilic Syndromes) and EMSED (Study Group of Systemic Diseases in Dermatology) networks
Πηγή: The journal of allergy and clinical immunology. In practice [J Allergy Clin Immunol Pract] 2026 Oct; Vol. 14 (10), pp. 2368-2378.e4. Date of Electronic Publication: 2026 Aug 04.
Τύπος έκδοσης: Journal Article; Multicenter Study
Γλώσσα: English
Στοιχεία περιοδικού: Publisher: Elsevier Inc Country of Publication: United States NLM ID: 101597220 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 2213-2201 (Electronic) NLM ISO Abbreviation: J Allergy Clin Immunol Pract Subsets: MEDLINE
Imprint Name(s): Original Publication: New York, NY : Elsevier Inc., [2013]-
Ιατρικοί όροι (MeSH): Cellulitis*/drug therapy , Cellulitis*/pathology , Cellulitis*/diagnosis , Eosinophilia*/drug therapy , Eosinophilia*/pathology , Eosinophilia*/diagnosis, Adrenal Cortex Hormones/therapeutic use ; Humans ; Female ; Middle Aged ; Retrospective Studies ; Male ; Adult ; Aged ; Risk Factors ; Young Adult
Περίληψη: Background: Eosinophilic cellulitis (EC), also known as Wells' syndrome, is a rare, poorly studied inflammatory dermatosis.
Objective: To describe the clinical and histopathological spectrum, associated conditions, outcomes of EC, and treatment response rates.
Methods: Retrospective multicenter study including patients with biopsy-proven EC. Risk factors for unfavorable clinical course (defined as recurrence or persistent disease >6 months) were assessed using univariable and multivariable logistic regression models.
Results: A total of 114 patients (53% females; median age, 52 years) were included. The most frequent clinical presentation was papulonodular (52%), with lesions predominantly on the legs (69%). Disabling pruritus was common (83%), whereas systemic symptoms were rare. Histological findings did not differ between patients with or without triggering factors or associated diseases, or between those with or without blood eosinophilia. Triggering factors and associated conditions were identified in 30 (26%) and 24 (21%) patients, respectively. Arthropod bites or stings were the most common triggering factor, whereas hematological malignancies, antineutrophil cytoplasmic antibody-negative eosinophilic granulomatosis with polyangiitis, and idiopathic hypereosinophilic syndrome were the most frequently associated conditions. Most frequently used treatments were topical (54%) and oral corticosteroids (35%), with complete response rates of 67% and 82%, respectively. Conventional synthetic disease-modifying antirheumatic drugs showed moderate efficacy (complete response ≤31%), whereas biologics targeting type 2 inflammation yielded promising results. In multivariable analysis, lesion size higher than 5 cm was the only variable independently associated with unfavorable clinical course (odds ratio, 3.79; 95% CI 1.42-10.10; P = .008).
Conclusions: EC likely represents a disease spectrum encompassing conditions leading to eosinophil-mediated dermal toxicity. Lesion size larger than 5 cm is associated with unfavorable clinical course. Although corticosteroids achieve high remission rates, biologics targeting type 2 inflammation appear promising second-line options warranting further evaluation.
(Copyright © 2026 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)
Contributed Indexing: Keywords: Eosinophilic cellulitis; Eosinophilic granulomatosis with polyangiitis; Hypereosinophilic syndrome; IL-5; Janus kinase inhibitors; Type 2 inflammation
Substance Nomenclature: 0 (Adrenal Cortex Hormones)
SCR Disease Name: Wells syndrome
Entry Date(s): Date Created: 20260804 Date Completed: 20261007 Latest Revision: 20261007
Update Code: 20261008
DOI: 10.1016/j.jaip.2026.07.033
PMID: 42551694
Βάση Δεδομένων: MEDLINE
Περιγραφή
ISSN:2213-2201
DOI:10.1016/j.jaip.2026.07.033