Successful Treatment of Refractory Hailey-Hailey Disease with Dupilumab: A Case Report
نویسندگان
چکیده
Hailey-Hailey disease (HHD) is an autosomal dominant blistering dermatosis with incomplete penetrance caused by ATP2C1 gene mutation. Currently, there no cure for HHD; however therapeutic options aim to minimize the exacerbating factors and manage patients’ symptoms. A 58-year-old male presented a 10-year history of biopsy-proven HHD. He was seen consistently over course nine years multiple flares year consisting pruritic painful intertriginous plaques involvement chest, upper arms, back. His recalcitrant topicals, oral topical antibiotics, phototherapy, systemic corticosteroids. started on dupilumab noticed significant improvement. We present case HHD various modalities treatment. Our description rapid improvement suggests role Th2 signaling in pathophysiology propose that works due skin barrier dysfunction, similar atopic dermatitis, consider whether inflammation plays earlier disease. Although used off-label purpose, this case, further studies should assess clinical response safety patients treated dupilumab.
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ژورنال
عنوان ژورنال: Skin
سال: 2023
ISSN: ['2574-1624']
DOI: https://doi.org/10.25251/skin.7.2.15