Hailey Hailey Disease

Common Name(s)

Hailey Hailey Disease, Familial benign pemphigus, Hailey-Hailey Disease

Hailey-Hailey disease is an inherited condition characterized by recurrent blisters seen primarily on the neck, groin, and axillae (armpit). Precipitating factors include heat, sweating, skin infection, and ultraviolet radiation. The disorder often becomes apparent after puberty, usually by the third or fourth decade, but symptoms can develop at any age.  Hailey-Hailey disease occurs due to a mutation in a specific gene (ATP2C1) that creates a protein that is essential for skin health. There is no cure for this condition. Treatment is aimed at reducing symptoms and preventing flare-ups. This condition is inherited in an autosomal dominant fashion.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Hailey Hailey Disease" for support, advocacy or research.

Hailey-Hailey Disease Society

Our primary mission is the dissemination of free, correct and useful information and support for HHD patients and the health care community. We support an informational website and forum and manage the Yahoo Group, TalkHHD.

Last Updated: 8 Mar 2013

View Details

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
Top

How do you compare to others with this condition?

Privately answer questions about your health. Let resources, you select, come to you.

Anonymously share and see how your answers compare with others with this condition while privately providing key pieces of information to medical researchers, disease advocacy groups, and others ONLY YOU select to help speed up cures and better alternatives.

 
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Hailey Hailey Disease" for support, advocacy or research.

Hailey-Hailey Disease Society

Our primary mission is the dissemination of free, correct and useful information and support for HHD patients and the health care community. We support an informational website and forum and manage the Yahoo Group, TalkHHD.

http://haileyhailey.com

Last Updated: 8 Mar 2013

View Details

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
 
 
Top

Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Hailey Hailey Disease" returned 44 free, full-text research articles on human participants. First 3 results:

A family with atypical Hailey Hailey disease--is there more to the underlying genetics than ATP2C1?
 

Author(s): Nina van Beek, Aikaterini Patsatsi, Yask Gupta, Steffen Möller, Miriam Freitag, Susanne Lemcke, Andreas Recke, Detlef Zillikens, Enno Schmidt, Saleh Ibrahim

Journal:

 

The autosomal dominant Hailey Hailey disease (HHD) is caused by mutations in the ATP2C1 gene encoding for human secretory pathway Ca2+/Mn2+ ATPase protein (hSPCA1) in the Golgi apparatus. Clinically, HHD presents with erosions and hyperkeratosis predominantly in the intertrigines. ...

Last Updated: 3 Apr 2015

Go To URL
Identification of several mutations in ATP2C1 in Lebanese families: insight into the pathogenesis of Hailey-Hailey disease.
 

Author(s): Waed Btadini, Ossama K Abou Hassan, Dana Saadeh, Ossama Abbas, Farah Ballout, Abdul-Ghani Kibbi, Ghassan Dbaibo, Nadine Darwiche, Georges Nemer, Mazen Kurban

Journal:

 

Hailey-Hailey disease (HHD) is an inherited blistering dermatosis characterized by recurrent erosions and erythematous plaques that generally manifest in intertriginous areas. Genetically, HHD is an autosomal dominant disease, resulting from heterozygous mutations in ATP2C1, which ...

Last Updated: 9 Feb 2015

Go To URL
Hailey-Hailey disease with skin lesions at unusual sites and a good response to acitretin.
 

Author(s): Biju Vasudevan, Rajesh Verma, Sonia Badwal, Shekar Neema, Debdeep Mitra, T Sethumadhavan

Journal: Indian J Dermatol Venereol Leprol. ;81(1):88-91.

 

Last Updated: 8 Jan 2015

Go To URL

Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Hailey Hailey Disease" returned 4 free, full-text review articles on human participants. First 3 results:

Two novel ATP2C1 mutations in patients with Hailey-Hailey disease and a literature review of sequence variants reported in the Chinese population.
 

Author(s): L Meng, Y Gu, X F Du, M H Shao, L L Zhang, G L Zhang, X L Wang

Journal:

 

Hailey-Hailey disease (HHD) is an autosomal dominant disorder in which the ATP2C1 gene has been implicated. Many mutations of this gene have been detected in HHD patients. To analyze such mutations in HHD and summarize all those identified in Chinese patients with this disease, we ...

Last Updated: 19 Jan 2016

Go To URL
Using yeast to model calcium-related diseases: example of the Hailey-Hailey disease.
 

Author(s): Cécile Voisset, Néstor García-Rodríguez, April Birkmire, Marc Blondel, Ralf Erik Wellinger

Journal: Biochim. Biophys. Acta. 2014 Oct;1843(10):2315-21.

 

Cross-complementation studies offer the possibility to overcome limitations imposed by the inherent complexity of multicellular organisms in the study of human diseases, by taking advantage of simpler model organisms like the budding yeast Saccharomyces cerevisiae. This review deals ...

Last Updated: 1 Aug 2014

Go To URL
Hailey-Hailey disease as an orthodisease of PMR1 deficiency in Saccharomyces cerevisiae.
 

Author(s): Richard Kellermayer

Journal: FEBS Lett.. 2005 Apr;579(10):2021-5.

 

The term orthodisease has recently been introduced to define human disorders in which the pathogenic gene has orthologs in model organism genomes. Here, we describe Hailey-Hailey disease (HHD), a blistering skin disorder caused by haploinsufficiency of ATP2C1 as an orthodisease from ...

Last Updated: 6 Apr 2005

Go To URL
 
 
Top

Symptoms, Diagnosis, and Treatment

There are currently no related results available in Genetics Home Reference.

There are currently no related results available in GeneReviews.

 
 
Top

Clinical Trial Information This information is provided by ClinicalTrials.gov

There are currently no open clinical trials for this condition.