Combined therapeutic use of oral alitretinoin and narrowband ultraviolet-B therapy in the treatment of Hailey-Hailey disease

Abstract

Hailey-Hailey disease (HHD) is a chronic familial bullous disease characterized by recurrent blisters and erosions typically at friction-prone areas of the body accompanied by acantholysis upon histologic examination. There are a number of therapies used in the management of HHD. Its symptoms have been effectively treated with antimicrobial therapies, corticosteroids and other agents such as cyclosporine and prednisone. However, such treatments are not always effective. Therefore, there is a need for new treatments for the management of HHD. In this report, a patient with long-standing HHD responsive only to high levels of prednisone is described. After the successful tapering and cessation of oral prednisone the patient began a new combination therapy of complementary doses of oral alitretinoin, and narrowband UVB therapy, which yielded a favorable response within 2-3 weeks. After 6 weeks, a mono-therapy of daily (30 mg) oral alitretinoin was sufficient to maintain successful near-complete remission of the disease.

Recalcitrant vulvar Hailey-Hailey disease treated with alitretinoin and onabotulinumtoxinA: A case report

“…In the case of our patient, the only satisfying treatment was achieved with a combination of alitretinoin 10 mg daily and OnA injections every 9 months. Alitretinoin has been described in three other patients up to now by Sardy and Ruzicka, 6 and Vanderbeck et al 7 They were all treated with a dose of 30 mg and responded positively, although two of them needed a combination, one with oral prednisone and the other with narrowband ultraviolet B (UVB). One case was also successfully treated with etretinate, which is no longer available.…”

Management of familial benign chronic pemphigus

“…36 In a separate study, disease was controlled with oral prednisone during exacerbations. 38 However, her therapy was switched to a combination of oral alitretinoin (30 mg per day) and narrowband ultraviolet B light therapy twice weekly. Lesions near-cleared by 6 weeks, after which oral alitretinoin was used alone to maintain the improvement.…”

Identification of ATP2C1 mutations in the patients of Hailey-Hailey disease

“…The incidence of this disease is approximately 0.002% [1], and has no signi cant difference between men and women [2]. HHD is currently not curable, and symptomatic treatment is the main strategy taken so far to reduce symptoms and prevent disease recurrence [3][4][5][6], using cortisol to reduce in ammation [7], and using topical vitamins [8,9] to promote the differentiation of epidermal keratinocytes and regulate intercellular Ca 2+ concentrations [10,11].…”