Characteristics of Wells' syndrome are recurrent episodes of edema and erythema of sudden onset, often covering large areas of the skin. Microscopy shows marked eosinophilia and the presence of so-called flame figures. The flame figures have been considered to be either secondary to aggregates of expelled eosinophilic granules and disintergrating eosinophils, or foci of necrobiotic collagen. Our study indicates that the flame figure is secondary to disintegration of eosinophils and consists of aggregates of eosinophilic granules and nuclear fragments and not of necrobiotic collagen. We consider Wells' syndrome to be a distinctive clinical and histological reaction, which can be triggered by many different, mostly unknown factors.
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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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