Hailey-Hailey disease

Jump to: navigation, search
Hailey-Hailey disease
ICD-10 Q82.8
ICD-9 757.39
OMIM 169600
DiseasesDB 29321
MeSH D016506

WikiDoc Resources for

Hailey-Hailey disease

Articles

Most recent articles on Hailey-Hailey disease

Most cited articles on Hailey-Hailey disease

Review articles on Hailey-Hailey disease

Articles on Hailey-Hailey disease in N Eng J Med, Lancet, BMJ

Media

Powerpoint slides on Hailey-Hailey disease

Images of Hailey-Hailey disease

Photos of Hailey-Hailey disease

Podcasts & MP3s on Hailey-Hailey disease

Videos on Hailey-Hailey disease

Evidence Based Medicine

Cochrane Collaboration on Hailey-Hailey disease

Bandolier on Hailey-Hailey disease

TRIP on Hailey-Hailey disease

Clinical Trials

Ongoing Trials on Hailey-Hailey disease at Clinical Trials.gov

Trial results on Hailey-Hailey disease

Clinical Trials on Hailey-Hailey disease at Google

Guidelines / Policies / Govt

US National Guidelines Clearinghouse on Hailey-Hailey disease

NICE Guidance on Hailey-Hailey disease

NHS PRODIGY Guidance

FDA on Hailey-Hailey disease

CDC on Hailey-Hailey disease

Books

Books on Hailey-Hailey disease

News

Hailey-Hailey disease in the news

Be alerted to news on Hailey-Hailey disease

News trends on Hailey-Hailey disease

Commentary

Blogs on Hailey-Hailey disease

Definitions

Definitions of Hailey-Hailey disease

Patient Resources / Community

Patient resources on Hailey-Hailey disease

Discussion groups on Hailey-Hailey disease

Patient Handouts on Hailey-Hailey disease

Directions to Hospitals Treating Hailey-Hailey disease

Risk calculators and risk factors for Hailey-Hailey disease

Healthcare Provider Resources

Symptoms of Hailey-Hailey disease

Causes & Risk Factors for Hailey-Hailey disease

Diagnostic studies for Hailey-Hailey disease

Treatment of Hailey-Hailey disease

Continuing Medical Education (CME)

CME Programs on Hailey-Hailey disease

International

Hailey-Hailey disease en Espanol

Hailey-Hailey disease en Francais

Business

Hailey-Hailey disease in the Marketplace

Patents on Hailey-Hailey disease

Experimental / Informatics

List of terms related to Hailey-Hailey disease


Hailey-Hailey disease, or familial benign pemphigus, was originally described by the Hailey brothers in 1939.[1][2] It is a genetic disorder that causes blisters to form on the skin.

Overview

Familial benign pemphigus originally was described by the Hailey brothers in 1939, and is commonly called Hailey Hailey disease.

It is characterized by outbreaks of rashes and blisters in the skin, usually in the folds of the skins, but also often over large areas of the body. The painful blisters break and sometimes become infected and raw, with new blisters forming over raw skin in a sometimes seemingly unending cycle of outbreaks.

The cause of the disease is a defect in the calcium pump ATP2C1 gene which is located on chromosome 3. This causes the cells of the skin to not adhere together properly, causing the blisters and rashes. There is no cure.

Topical steroid preparations have some effect during outbreaks, but do not stop them. Secondary bacterial, fungal and/or viral infections are common and may exacerbate an outbreak. Avoiding heat and friction of affected areas and keeping the area clean and dry work about as well.

Some have found relief in laser resurfacing that burns off the top layer of the epidermis, allowing healthy non-affected skin to regrow in its place. Reducing sweat production is helpful in many cases. Some patients have found that outbreaks are triggered by certain foods, hormone cycles and stress.

Unfortunately, there is not much that really helps when the outbreaks come, except to suffer through them, "suffer" being the key word here, since the outbreaks are often extremely painful.

Signs and symptoms

It is characterized by outbreaks of rashes and blisters in the skin, usually in the folds of the skins, but also often over large areas of the body. The painful blisters break and sometimes become infected and raw, with new blisters forming over raw skin in a sometimes seemingly unending cycle of outbreaks.

Causes

The cause of the disease is a defect in the calcium pump ATP2C1[3] gene which is located on chromosome 3. This causes the cells of the skin to not adhere together properly, causing the blisters and rashes. There is no cure.

Treatment

Topical steroid preparations have some effect during outbreaks, but do not stop them. Secondary bacterial, fungal and/or viral infections are common and may exacerbate an outbreak. Avoiding heat and friction of affected areas and keeping the area clean and dry work about as well.

Some have found relief in laser resurfacing that burns off the top layer of the epidermis, allowing healthy non-affected skin to regrow in its place. Reducing sweat production is helpful in many cases. Some patients have found that outbreaks are triggered by certain foods, hormone cycles and stress.

References

  1. synd/2081 at Who Named It
  2. H. Hailey, H. Hailey. Familial benign chronic pemphigus. Report of 13 cases in four generations of a family and report of 9 additional cases in 4 generations of a family. Archives of Dermatology and Syphilology, Chicago, 1939, 39: 679-685.
  3. Hu Z, Bonifas JM, Beech J, et al (January 2000). "Mutations in ATP2C1, encoding a calcium pump, cause Hailey-Hailey disease". Nat. Genet. 24 (1): 61–5. doi:10.1038/71701. PMID 10615129.

Controversy

Pemphigus is not Benign Familial Pemphigus, usually called Hailey-Hailey disease. Some medical books refer to Hailey-Hailey Disease as 'Benign Familial Pemphigus' but it is not true pemphigus. Hailey-Hailey is not an autoimmune disease as is pemphigus, it is genetic. There are some similarities in the blisters, but Hailey-Hailey is not caused by an auto-antibody, as in PV. For more information Click here

External links

de:Morbus Hailey-Hailey

nl:Familiaire benigne chronische pemphigus


Navigation WikiDoc | WikiPatient | Up To Date Pages | Recently Edited Pages | Recently Added Pictures

Table of Contents In Alphabetical Order | By Individual Diseases | Signs and Symptoms | Physical Examination | Lab Tests | Drugs

Editor Tools Become an Editor | Editors Help Menu | Create a Page | Edit a Page | Upload a Picture or File | Printable version | Permanent link | Maintain Pages | What Pages Link Here
There is no pharmaceutical or device industry support for this site and we need your viewer supported Donations | Editorial Board | Governance | Licensing | Disclaimers | Avoid Plagiarism | Policies
Linked-in.jpg