Successful Treatment of a Widespread Pemphigus Chronicus Familiaris (Hailey-Hailey) By Erbium-YAG-Laser

Abstract

BACKGROUND: Familial chronic pemphigus or Hailey-Hailey disease (OMIM 169600) is a rare, autosomal dominant blistering skin disorder the genetic background are mutations of the ATP2 C1 gene. The treatment is challenging.

CASE REPORT: A 48-year-old Caucasian female patient presented to the department with a relapse of her Pemphigus chronicus familiaris (Hailey-Hailey). No other medical diseases were known. On examination, we observed an otherwise healthy woman with widespread erosive lesions on the neck, axillae, groins, submammary fold and anal fold. She reported burning sensations and an unpleasant odour. The diagnosis had been confirmed earlier by histopathology of a skin biopsy with acantholysis, and the relapsing and remitting course.  Family history was positive for father and brother. Since she had not responded well in the past to systemic retinoids and did not tolerate the adverse effects of these drugs, we suggested an ablative erbium-YAG laser treatment in general anaesthesia. Laser treatment was performed with the MCL 29 Dermablate (Asclepion Laser Technologies, Jena, Germany) on two occasions. We used a 5 mm focus, pulse energy of 1200 mJ at 8 Hz. The resulting superficial wounds were treated with an ointment containing fusidic acid 0.2% and betamethasone 0.1%. Wound healing was completed after 12 days. No adverse events were observed.

CONCLUSIONS: Ablative erbium-YAG therapy is an option for pemphigus chronicus familiaris, in particular in young women and patients who do not tolerate the adverse effects of retinoid therapy.