Tratamiento de la enfermedad de Hailey-Hailey con láser de dióxido de carbono: tratamiento en 8 pacientes / Carbon Dioxide Laser Treatment in Hailey-Hailey Disease: A Series of 8 Patients

Introducción y objetivos: La enfermedad de Hailey-Hailey (EHH) o pénfigo familiar benigno es un rara genodermatosis ampollosa con herencia autosómica dominante. Cursa con erosiones cutáneas, localizadas principalmente en los grandes pliegues, que se sobreinfectan y pueden ser muy dolorosas e incapacitantes para los pacientes. Es una afección difícil de tratar, y aunque se han usado múltiples tratamientos tópicos y sistémicos para controlar los brotes de la enfermedad, ninguno de ellos consigue remisiones a medio-largo plazo. Pacientes y método: Realizamos un estudio retrospectivo de una serie de 8 casos de EHH tratados con láser CO2 en nuestro centro desde 1999 a 2011. Los pacientes tratados tenían una media de edad de 50,7 años, siendo 4 de ellos hombres y 4 mujeres. El seguimiento de los pacientes osciló entre uno y 12 años. Resultados: De los 8 pacientes tratados 6 obtuvieron unos resultados satisfactorios (4 pacientes con mejoría clínica>75% y 2 pacientes entre 50-75%). Esta eficacia se mantuvo a lo largo del tiempo. Los peores resultados se observaron en los pacientes tratados con potencias más bajas. Las zonas con recidiva de la enfermedad después del tratamiento se trataron con una segunda sesión de láser con buena respuesta. No observamos efectos adversos, salvo ligeros cambios texturales y de la pigmentación. Conclusiones: El láser de CO2 es un tratamiento eficaz y seguro a medio-largo plazo en los casos de EHH sintomáticos y refractarios a tratamientos convencionales (AU)

Introduction and objectives: Hailey-Hailey disease (HHD), or benign familial pemphigus, is arare autosomal dominant genodermatosis characterized by the formation of blisters. Eruptionsare usually located in large skin folds, and when blisters become infected the condition can bevery painful and disabling. HHD is difficult to treat. Many topical and systemic treatments have been used to bring exacerbations under control, but none have achieved medium- to long-term remission. Patients and methods: Retrospective study of 8 patients with HHD treated with carbon dioxide laser therapy in our hospital between 1999 and 2011. The patients’ mean age was 50.7 years. The 4 men and 4 women were followed for between 1 and 12 years. Results: Satisfactory outcomes were achieved for 6 of the patients. Clinical improvement was observed in more than 75% of the affected area in 4 patients and in 50% to 75% of the area in 2 patients. The effect of treatments was maintained over time. The poorest outcomes were observed in patients treated at lower potencies. When blistering recurred after treatment, a second laser session achieved a good response. We observed no adverse events other than slight changes in skin texture and pigmentation. Conclusions: Carbon dioxide laser therapy was safe and effective in producing medium- to long-term improvement in HHD symptoms that were refractory to conventional treatments (AU)

Carbon dioxide laser treatment in Hailey-Hailey disease: a series of 8 patients.

BACKGROUND: Hailey-Hailey disease (HHD), or benign familial pemphigus, is a rare autosomal dominant genodermatosis characterized by the formation of blisters. Eruptions are usually located in large skin folds, and when blisters become infected the condition can be very painful and disabling. HHD is difficult to treat. Many topical and systemic treatments have been used to bring exacerbations under control, but none have achieved medium- to long-term remission. PATIENTS AND METHODS: Retrospective study of 8 patients with HHD treated with carbon dioxide laser therapy in our hospital between 1999 and 2011. The patients' mean age was 50.7 years. The 4 men and 4 women were followed for between 1 and 12 years. RESULTS: Satisfactory outcomes were achieved for 6 of the patients. Clinical improvement was observed in more than 75% of the affected area in 4 patients and in 50% to 75% of the area in 2 patients. The effect of treatments was maintained over time. The poorest outcomes were observed in patients treated at lower potencies. When blistering recurred after treatment, a second laser session achieved a good response. We observed no adverse events other than slight changes in skin texture and pigmentation. CONCLUSIONS: Carbon dioxide laser therapy was safe and effective in producing medium- to long-term improvement in HHD symptoms that were refractory to conventional treatments.