Successful Treatment of Hailey-Hailey Disease with Systemic Cyclosporine: Case Report

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Şahn G., Şentürk N., Şahin S. K.

Turkiye Klinikleri Dermatoloji, vol.27, no.2, pp.101-105, 2017 (Scopus) identifier

  • Publication Type: Article / Article
  • Volume: 27 Issue: 2
  • Publication Date: 2017
  • Doi Number: 10.5336/dermato.2016-53980
  • Journal Name: Turkiye Klinikleri Dermatoloji
  • Journal Indexes: Scopus
  • Page Numbers: pp.101-105
  • Keywords: Benign familial, Cyclosporine, Pemphigus, Therapeutics
  • Ondokuz Mayıs University Affiliated: Yes


Hailey-Hailey disease (HHD) is a rare disease that affects the adhesion of epidermal keratinocytes. It is characterized by erosions and crustrations primarily in intertriginous areas. Treatment options include topical antibiotics, antifungals, corticosteroids, dapsone, photo chemotherapy, systemic retinoids, cyclosporine, methotrexate and photodynamic therapy. Here we report a 56 years old female with refractory HHD that is succesfully treated with cyclosporine. The patient had been treated with topical steroids, antifungals, antimicrobials and topical calcineurin inhibitors with limited success. Systemic treatment with methotrexate (15 mg weekly) and acitretin (25 mg daily) were also unsuccessful. Cyclosporine was started 2.5 mg/kg/day and resulted in almost complete clearance of lesions after a month. The dose of cyclosporine was reduced gradually and stopped at the end of sixth months. Topical antibiotics and topical tacrolimus were used for minor recurrences. Short course of cyclosporine can be an alternative for refractory HHD.