Acantholytic disorders: Update on pathophysiology, diagnosis, and management - PubMed
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- Acantholytic skin disorders involve impaired intercellular adhesion between epidermal keratinocytes without primary immunological etiology.
- Genetic acantholytic disorders include Darier disease (DD) and Hailey-Hailey disease (HHD), while Grover disease (GD) has an unknown pathogenesis.
- Diagnoses may be missed due to rarity, and there are no standardized treatment guidelines.
- Mild disease is typically treated with topical steroids and retinoids; severe cases may use oral retinoids like acitretin, though efficacy varies.
- Emerging treatments include biologics (dupilumab, TNF-α inhibitors), small molecular inhibitors (apremilast, JAK inhibitors), and low-dose naltrexone.
- Potential therapeutic strategies target IL-17/23, IL-4/13 axes, MAPK/ERK pathways (e.g., guselkumab), and SERCA activators for DD.
- This review aims to improve diagnosis and provide expert treatment recommendations based on emerging evidence.