Rapid and long‐lasting remission of refractory Hailey‐Hailey disease by IL‐13 inhibition with tralokinumab
Year: 2026
Type: article
Abstract: Dear Editors,Hailey-Hailey disease (HHD; OMIM 169600), also known as pemphigus chronicus benignus familiaris, is a rare, autosomal dominant, chronic genodermatosis that usually manifests for the first time in early adulthood with complete penetrance but variable expressivity.It affects the intertriginous areas, especially the axillae, groin and, in women, the submammary regions, where erosive, macerated, fissured, erythematous plaques develop.The associated pain and frequent foetor often lead to social isolation, which further impairs the quality of life of patients.1 Mechanical irritation, sweating, UV exposure and microbial and viral infections can aggravate the disease or can trigger a relapse.The disease is caused by a mutation in the calcium pump-encoding ATP2C1 gene that leads to disturbances in intracellular calcium homeostasis and thus to impaired suprabasal keratinocyte adhesion and acantholysis.2 We report on a 68-year-old female patient with a positive family history (father and both uncle and grandmother on the father's side were affected) who had suffered from histologically confirmed Hailey-Hailey disease for 38 years, and who did not respond or only poorly responded to numerous therapy attempts but became symptom-free under treatment with the anti-interleukin (IL)-13 antibody tralokinumab.The skin lesions had previously persisted in the axillae, submammary and inguinal areas (Figure 1a, b) and regularly worsened during the warm season due to sweating, causing the patient to wear her underwear inside out to avoid additional mechanical irritation from seams.In addition, during periods of deterioration, she refrained from sports
DOI: 10.1111/ddg.70023x
Institution: University Hospital of Basel
Language: German
Subfield: Genetics
Domain: Life Sciences
Open Access status: hybrid
Estimated APC paid: $4,480
OpenAlex ID: W7122711679