Hailey–Hailey disease (HHD), or benign familial chronic pemphigus, is a rare autosomal-dominant genodermatosis caused by mutations in the ATP2C1 gene, which encodes the calcium/ manganese (Ca2+/Mn2+) ATPase hSPCA1 that regulates Ca2+ homeostasis in the Golgi apparatus. Mutations in this gene disrupt desmosome formation and keratinocyte adhesion, leading to epidermal acantholysis and recurrent vesicopustular and erosive lesions in intertriginous areas. HHD follows a chronic relapsing course, with flares triggered by heat, friction, or infections, markedly impairing quality of life. Therapeutic management is challenging, and standardized treatment guidelines are lacking [1]. Emerging evidence suggests a potential role for oral magnesium chloride (MgCl2) in disease control
Targeting Intracellular Calcium Dysregulation in Hailey–Hailey Disease: A Case‐Based Rationale for Oral Magnesium Chloride Therapy / Corio, Andrea; Zampino, Maria Rosaria; Di Meo, Nicola; Zalaudek, Iris. - In: INTERNATIONAL JOURNAL OF DERMATOLOGY. - ISSN 0011-9059. - (2026), pp. 1-2. [10.1111/ijd.70294]
Targeting Intracellular Calcium Dysregulation in Hailey–Hailey Disease: A Case‐Based Rationale for Oral Magnesium Chloride Therapy
Corio, Andrea;di Meo, Nicola;Zalaudek, Iris
2026-01-01
Abstract
Hailey–Hailey disease (HHD), or benign familial chronic pemphigus, is a rare autosomal-dominant genodermatosis caused by mutations in the ATP2C1 gene, which encodes the calcium/ manganese (Ca2+/Mn2+) ATPase hSPCA1 that regulates Ca2+ homeostasis in the Golgi apparatus. Mutations in this gene disrupt desmosome formation and keratinocyte adhesion, leading to epidermal acantholysis and recurrent vesicopustular and erosive lesions in intertriginous areas. HHD follows a chronic relapsing course, with flares triggered by heat, friction, or infections, markedly impairing quality of life. Therapeutic management is challenging, and standardized treatment guidelines are lacking [1]. Emerging evidence suggests a potential role for oral magnesium chloride (MgCl2) in disease controlPubblicazioni consigliate
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