Dupilumab for Hailey-Hailey disease.

Area of Science:

• Dermatology
• Rare Diseases
• Autoimmune Blistering Disorders

Background:

• Hailey-Hailey disease (HHD) is a rare, autosomal dominant blistering condition affecting skin folds.
• Clinical heterogeneity and lack of treatment guidelines complicate HHD diagnosis and management.
• Biological therapies are increasingly used for HHD, but their efficacy is variable.

Purpose of the Study:

• To report the therapeutic effect of a combination treatment for extensive, therapy-resistant Hailey-Hailey disease.
• To evaluate the efficacy and safety of dupilumab and benzathine benzylpenicillin in a HHD patient.

Main Methods:

• A case report detailing the treatment of a 49-year-old woman with severe, therapy-resistant HHD.
• Combination therapy with dupilumab and benzathine benzylpenicillin was administered.
• Patient was followed for 15 months post-treatment.

Main Results:

• Marked improvement in HHD plaques and patient quality of life observed after an eight-month follow-up.
• A minor relapse occurred at 15 months, potentially linked to emotional stress.
• No adverse events were reported during the treatment period.

Conclusions:

• Combining dupilumab and benzathine benzylpenicillin shows benefit for extensive, therapy-resistant HHD.
• Achieving long-term complete remission in HHD remains challenging.
• Sustained psychological support may be beneficial for HHD patients.