Treatment of Hailey–Hailey disease with narrowband phototherapy and acitretin: A case report

Abstract

The Hailey–Hailey disease, or familial benign chronic pemphigus, is an autosomal dominant genodermatosis affecting mainly intertriginous areas. It manifests itself in painful blisters, erosions, and cracks and has a chronic course with frequent flares, significantly impacting patients’ quality of life. Presently, there is no cure, but multiple treatment modalities are available. Most evidence supports treatment with topical steroids and antimicrobials. Treatment of recalcitrant disease has been shown to benefit from the addition of oral antibiotics, Naltrexone, systemic retinoids, botulinum toxin A injections, laser treatment, and surgical excision. We describe a case of refractory Hailey–Hailey disease for which most of the abovementioned options failed, but which demonstrated significant improvement following a combination of oral acitretin and narrowband ultraviolet-B phototherapy. To achieve remission, the patient received 30 sessions three times per week with the increment of 20 mJ/cm2 per session and oral acitretin 25 mg PO daily.