Dupilumab for Hailey-Hailey disease

AIM: Hailey-Hailey disease is a rare autosomal dominant blistering disease predominantly affecting the skinfolds. Due to the clinical heterogeneity and lack of guidelines, diagnosis and treatment is challenging. At present, there is an increasing use of biologicals in the treatment of HHD. However, their efficacy varies. METHODS: This case report describes the therapeutic effect of dupilumab in combination with benzathine benzylpenicillin in a 49-year-old woman with extensive therapy-resistant Hailey-Hailey disease. RESULTS: After a follow-up period of eight months, we observed a marked improvement of the Hailey-Hailey plaques, and the patient's quality of life improved. A minor relapse occurred after 15 months, possibly due to an emotionally stressful event for the patient. No adverse events were reported. CONCLUSION: Our case report illustrates the benefit of combining dupilumab and benzathine benzylpenicillin for extensive, therapy-resistant HHD. However, long-term complete remission remains challenging and sustained psychological support seems advised.