Unlabelled: Wells syndrome or eosinophilic cellulitis is a rare and relapsing skin disease which lacks systemic involvement. A skin biopsy is needed to establish a diagnosis. Several precipitating factors have been proposed but no proven causative link has been found. On the other hand, Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA), an auto-immune disease, is associated with multiorgan, including cutaneous manifestations. We report a case with overlapping features of Wells and Churg-Strauss syndrome, suggesting that these syndromes could be part of the same nosological entity.
Learning Points: Biopsies are essential to establish a diagnosis in unusual cases of cellulitis.COVID-19 vaccination is a putative trigger for Wells syndrome.There is significant overlap between Wells and Churg-Strauss syndromes.
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http://dx.doi.org/10.12890/2023_003954 | DOI Listing |
J Exp Med
February 2025
Laboratory of Immunogenetics of Pediatric Autoimmune Diseases, INSERM UMR 1163, Imagine Institute, University Paris Cité, Paris, France.
IKKα, encoded by CHUK, is crucial in the non-canonical NF-κB pathway and part of the IKK complex activating the canonical pathway alongside IKKβ. The absence of IKKα causes fetal encasement syndrome in humans, fatal in utero, while an impaired IKKα-NIK interaction was reported in a single patient and causes combined immunodeficiency. Here, we describe compound heterozygous variants in the kinase domain of IKKα in a female patient with hypogammaglobulinemia, recurrent lung infections, and Hay-Wells syndrome-like features.
View Article and Find Full Text PDFCureus
October 2024
Pathology, Lakeshore Hospital and Research Centre, Ernakulam, IND.
Eosinophilic cellulitis, also known as Wells syndrome, presents a wide range of morphological spectrum, from pruritic erythematous papules, nodules, and pustules to urticarial and bullous lesions. This is a rare dermatological condition and is known to develop after treatment of hematological malignancy. Here, we report a case of Wells syndrome that was the initial presentation of lymphoma, preceding all other symptoms by six months.
View Article and Find Full Text PDFJAAD Case Rep
November 2024
Division of Allergy, Pulmonary and Critical Care Medicine, Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee.
Allergol Select
October 2024
Center for Child and Adolescent Health, Helios Hospital Krefeld, Academic Hospital of RWTH Aachen, Krefeld.
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