Pénfigo benigno familiar, un padecimiento poco reconocido.

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Bibliographic Details
Title:	Pénfigo benigno familiar, un padecimiento poco reconocido.
Alternate Title:	Benign familial pemphigus, a misrecognized entity.
Authors:	Guevara-Hernández, Cindy¹ cindyguevara85@gmail.com, Jaramillo-Manzur, Silvia Cristina¹, Landeta-Sa, Ana Paula², Castillo, Rodrigo Hernández³, Arellano-Mendoza, María Ivonne⁴
Source:	Dermatología Revista Mexicana. Mar-Abr2020, Vol. 64 Issue 2, p195-200. 6p.
Abstract (English):	BACKGROUND: Benign familial pemphigus, also known as Hailey-Hailey disease, is a chronic, autosomal dominant dermatosis that usually presents with a benign evolution. It affects friction or sweating areas, such as groins and armpits. It is characterized by erythematous plaques with erosions, fissures and crusting, genital lesions usually look warty and disappear leaving postinflammatory hyperpigmentation. It is a clinical diagnosis; however, a biopsy should be performed to confirm it. The management of Hailey-Hailey disease is a challenge, since there is no curative treatment, topical steroids and antibiotics are the first line treatment. CLINICAL CASE: A 32-year-old female patient with a maternal branch family history of bullous dermatosis, who presented plaques with multiple papular-like lesions, exulcerations and hypopigmentation associated with burning pain in the genital area. The biopsy confirmed the diagnosis of Hailey-Hailey disease. CONCLUSIONS: Hailey-Hailey disease is a rare and little-known condition. It substantially affects the daily life of the patients, so it should be a comprehensive management and individually assess the best treatment, according to the need of each patient. [ABSTRACT FROM AUTHOR]
Abstract (Spanish):	ANTECEDENTES: El pénfigo benigno familiar, también conocido como enfermedad de Hailey-Hailey, es una dermatosis crónica, de herencia autosómica dominante que generalmente tiene curso benigno. Suele afectar zonas de fricción o sudoración como las ingles y las axilas. Se caracteriza por placas eritematosas maceradas con erosiones, fisuras y formación de costras, las lesiones genitales suelen tener aspecto verrugoso y suelen desaparecer con hiperpigmentación posinflamatoria. El diagnóstico de la enfermedad es clínico; sin embargo, debe realizarse biopsia para confirmarlo. El manejo de la enfermedad de Hailey-Hailey es un desafío porque no existe ningún tratamiento curativo; sin embargo, los esteroides y antibióticos tópicos son el tratamiento de primera línea. CASO CLÍNICO: Paciente femenina de 32 años de edad con antecedentes familiares en la rama materna de dermatosis ampollosa, que tuvo placas con múltiples lesiones de aspecto papular, exulceraciones e hipopigmentación asociadas con dolor ardoroso en la zona genital. La biopsia confirmó el diagnóstico de enfermedad de Hailey-Hailey. CONCLUSIONES: La enfermedad de Hailey-Hailey es un padecimiento raro y poco conocido. Afecta sustancialmente la vida cotidiana de los pacientes, por lo que debe darse un manejo integral y valorar individualmente el mejor tratamiento, según la necesidad de cada paciente. [ABSTRACT FROM AUTHOR]
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Database:	MedicLatina

Description
Abstract:	BACKGROUND: Benign familial pemphigus, also known as Hailey-Hailey disease, is a chronic, autosomal dominant dermatosis that usually presents with a benign evolution. It affects friction or sweating areas, such as groins and armpits. It is characterized by erythematous plaques with erosions, fissures and crusting, genital lesions usually look warty and disappear leaving postinflammatory hyperpigmentation. It is a clinical diagnosis; however, a biopsy should be performed to confirm it. The management of Hailey-Hailey disease is a challenge, since there is no curative treatment, topical steroids and antibiotics are the first line treatment. CLINICAL CASE: A 32-year-old female patient with a maternal branch family history of bullous dermatosis, who presented plaques with multiple papular-like lesions, exulcerations and hypopigmentation associated with burning pain in the genital area. The biopsy confirmed the diagnosis of Hailey-Hailey disease. CONCLUSIONS: Hailey-Hailey disease is a rare and little-known condition. It substantially affects the daily life of the patients, so it should be a comprehensive management and individually assess the best treatment, according to the need of each patient. [ABSTRACT FROM AUTHOR]
ISSN:	01854038