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/cm 2 per session and oral acitretin 25 mg PO daily.

Botulinum toxin in treating Hailey‐Hailey disease: A systematic review

“…At present, there is no cure for Hailey-Hailey disease, but multiple treatment options are available to improve symptoms. 49 However, these treatment options may have adverse effects. Topical application of steroids on the intertriginous areas could cause atrophy, streaks, and telangiectasias.…”

“…At present, there is no cure for Hailey‐Hailey disease, but multiple treatment options are available to improve symptoms 49 . However, these treatment options may have adverse effects.…”

Recurrent Ulcerations in an 84-Year-Old Male Diagnosed with Hailey-Hailey Disease

“…Hailey-Hailey disease (HHD), also known as familial benign chronic pemphigus, is a rare autosomal dominant skin disorder [ 1 , 2 ]. It is caused by a mutation in the ATP2C1 gene, which encodes for intracellular calcium pumps that alter the homeostasis of this ion [ 3 , 4 , 5 ].…”

“…It is caused by a mutation in the ATP2C1 gene, which encodes for intracellular calcium pumps that alter the homeostasis of this ion [ 3 , 4 , 5 ]. Excessive sweating, heat, and trauma to the skin may exacerbate HHD and cause separation of keratinocytes, especially in friction-prone areas [ 1 , 3 ]. Therapeutic options range from topical to systemic corticosteroids and antibiotics, among others [ 1 ].…”

“…Excessive sweating, heat, and trauma to the skin may exacerbate HHD and cause separation of keratinocytes, especially in friction-prone areas [ 1 , 3 ]. Therapeutic options range from topical to systemic corticosteroids and antibiotics, among others [ 1 ].…”