Familial benign chronic pemphigus (Hailey-Hailey Disease): use of topical immunomodulators as a modern treatment option

Benign chronic familial pemphigus (Hailey-Hailey disease) is a rare autosomal dominant blistering skin disorder characterized by suprabasal cell separation (acantholysis) of the epidermis. The Hailey brothers first described it in 1939. Hailey-Hailey disease usually appears in the third or fourth de...

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Autores principales:	Tchernev,Georgi, Cardoso,José Carlos
Lenguaje:	English
Publicado:	Sociedad Médica de Santiago 2011
Materias:	Acantholysis Anti inflammatory agents non-steroideal Calcineurin
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872011000500011
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spelling	oai:scielo:S0034-988720110005000112011-09-16Familial benign chronic pemphigus (Hailey-Hailey Disease): use of topical immunomodulators as a modern treatment optionTchernev,GeorgiCardoso,José Carlos Acantholysis Anti inflammatory agents non-steroideal Calcineurin Benign chronic familial pemphigus (Hailey-Hailey disease) is a rare autosomal dominant blistering skin disorder characterized by suprabasal cell separation (acantholysis) of the epidermis. The Hailey brothers first described it in 1939. Hailey-Hailey disease usually appears in the third or fourth decade, although it can occur at any age. Heat, sweating and friction often exacerbates the disease, and most patients have worse symptoms during summer. It is characterized clinically by a recurrent eruption of vesicles and bullae at the sites of friction and intertriginous areas. We report a 51-year-old male presenting with grey-brown hyperkeratosis with partial papillomatosis and lichenification in the axillary and inguinal areas and infiltrated erythematous lesions in the infraorbitary region, on the side of the face. Biopsies obtained from inguinal and axillar areas revealed parakeratotic crusts overlying an acantholytic epidermis. A biopsy from one of the lesions from the infraorbital area showed a Jessner-Kanof lymphocytic infiltration. The patient was treated with antimicrobials and four days later, topical Pimecrolimus was started, leading to an improvement of the clinical picture. The efficacy of Pimecrolimus in our case suggests that cellular immunity couldplay a role in thepathogenesis of Hailey-Hailey disease.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.139 n.5 20112011-05-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872011000500011en10.4067/S0034-98872011000500011
institution	Scielo Chile
collection	Scielo Chile
language	English
topic	Acantholysis Anti inflammatory agents non-steroideal Calcineurin
spellingShingle	Acantholysis Anti inflammatory agents non-steroideal Calcineurin Tchernev,Georgi Cardoso,José Carlos Familial benign chronic pemphigus (Hailey-Hailey Disease): use of topical immunomodulators as a modern treatment option
description	Benign chronic familial pemphigus (Hailey-Hailey disease) is a rare autosomal dominant blistering skin disorder characterized by suprabasal cell separation (acantholysis) of the epidermis. The Hailey brothers first described it in 1939. Hailey-Hailey disease usually appears in the third or fourth decade, although it can occur at any age. Heat, sweating and friction often exacerbates the disease, and most patients have worse symptoms during summer. It is characterized clinically by a recurrent eruption of vesicles and bullae at the sites of friction and intertriginous areas. We report a 51-year-old male presenting with grey-brown hyperkeratosis with partial papillomatosis and lichenification in the axillary and inguinal areas and infiltrated erythematous lesions in the infraorbitary region, on the side of the face. Biopsies obtained from inguinal and axillar areas revealed parakeratotic crusts overlying an acantholytic epidermis. A biopsy from one of the lesions from the infraorbital area showed a Jessner-Kanof lymphocytic infiltration. The patient was treated with antimicrobials and four days later, topical Pimecrolimus was started, leading to an improvement of the clinical picture. The efficacy of Pimecrolimus in our case suggests that cellular immunity couldplay a role in thepathogenesis of Hailey-Hailey disease.
author	Tchernev,Georgi Cardoso,José Carlos
author_facet	Tchernev,Georgi Cardoso,José Carlos
author_sort	Tchernev,Georgi
title	Familial benign chronic pemphigus (Hailey-Hailey Disease): use of topical immunomodulators as a modern treatment option
title_short	Familial benign chronic pemphigus (Hailey-Hailey Disease): use of topical immunomodulators as a modern treatment option
title_full	Familial benign chronic pemphigus (Hailey-Hailey Disease): use of topical immunomodulators as a modern treatment option
title_fullStr	Familial benign chronic pemphigus (Hailey-Hailey Disease): use of topical immunomodulators as a modern treatment option
title_full_unstemmed	Familial benign chronic pemphigus (Hailey-Hailey Disease): use of topical immunomodulators as a modern treatment option
title_sort	familial benign chronic pemphigus (hailey-hailey disease): use of topical immunomodulators as a modern treatment option
publisher	Sociedad Médica de Santiago
publishDate	2011
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872011000500011
work_keys_str_mv	AT tchernevgeorgi familialbenignchronicpemphigushaileyhaileydiseaseuseoftopicalimmunomodulatorsasamoderntreatmentoption AT cardosojosecarlos familialbenignchronicpemphigushaileyhaileydiseaseuseoftopicalimmunomodulatorsasamoderntreatmentoption
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Familial benign chronic pemphigus (Hailey-Hailey Disease): use of topical immunomodulators as a modern treatment option

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