Dupilumab, a Potential Novel Treatment for Hailey-Hailey Disease

Area of Science:

• Dermatology
• Genetics
• Immunology

Background:

• Hailey-Hailey disease (HHD) is a rare, inherited skin disorder causing chronic blistering and affecting keratinocyte adhesion.
• Current management of HHD presents challenges due to its recurrent nature and lack of targeted therapies.

Purpose of the Study:

• To review existing literature on dupilumab for refractory HHD.
• To present new clinical experience with dupilumab in HHD treatment.
• To discuss dupilumab's mechanism of action and future treatment strategies for HHD.

Main Methods:

• A comprehensive literature search was performed using keywords related to HHD and dupilumab.
• Databases searched included Medline, Cochrane Library, and EMBASE up to September 2024.
• A case of recalcitrant HHD treated with dupilumab was added to the reviewed data.

Main Results:

• Six published studies involving 11 HHD patients treated with dupilumab showed significant clinical improvement.
• Sustained disease remission was observed in 64% of patients over 5 to 24 months.
• No adverse effects were reported with dupilumab treatment in the reviewed cases.

Conclusions:

• Dupilumab emerges as a safe and effective therapeutic option for severe, refractory Hailey-Hailey disease.
• Further research is needed to establish long-term efficacy and optimal dosing regimens for dupilumab in HHD.