Hailey-Hailey disease

ICD-10 | Q82.8
ICD-9 | 757.39
OMIM | 169600
DiseasesDB | 29321
MeSH | D016506

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## Contents

* 1 Overview
* 2 Signs and symptoms
* 3 Causes
* 4 Treatment
* 5 References
* 6 Controversy
* 7 External links

## Overview[edit | edit source]

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.

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[edit | edit source]

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[edit | edit source]

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[edit | edit source]

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[edit | edit source]

1. ↑ Template:WhoNamedIt
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. (2000). "Mutations in ATP2C1, encoding a calcium pump, cause Hailey-Hailey disease". Nat. Genet. 24 (1): 61–5. doi:10.1038/71701. PMID 10615129. Unknown parameter `|month=` ignored (help)CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)

## Controversy[edit | edit source]

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[edit | edit source]

* Hailey-Hailey Disease Society

* v
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Diseases of the skin and subcutaneous tissue (integumentary system) (L, 680-709)

Infections|

Template:Navbox subgroup

Bullous disorders|

acantholysis (Pemphigus, Transient acantholytic dermatosis) * Pemphigoid (Bullous, Cicatricial, Gestational) * Dermatitis herpetiformis

Inflammatory|

Template:Navbox subgroup

Radiation-related disorders|

Sunburn * actinic rays (Actinic keratosis, Actinic cheilitis) * Polymorphous light eruption (Acne aestivalis) * Radiodermatitis * Erythema ab igne

Pigmentation disorder|

hypopigmentation (Albinism, Vitiligo) * hyperpigmentation (Melasma, Freckle, Café au lait spot, Lentigo/Liver spot, Acanthosis nigricans, Acral acanthotic anomaly)

Other skin|

keratosis/hyperkeratosis (Seborrheic keratosis, Callus) * other epidermal thickening (Ichthyosis acquisita, Palmoplantar keratoderma)

skin ulcer (Pyoderma gangrenosum, Bedsore)

Cutaneous Markers of Internal Malignancy (Florid cutaneous papillomatosis, acanthosis nigricans, sign of Leser-Trelat)

atrophic (Lichen sclerosus, Acrodermatitis chronica atrophicans)

necrobiosis (Granuloma annulare, Necrobiosis lipoidica) * other granuloma (Granuloma faciale, Pyogenic granuloma)

cutaneous vasculitis (Livedoid vasculitis, Erythema elevatum diutinum)

Connective tissues|

collagen disease: Keloid
localized connective tissue disorders: Lupus erythematosus (Discoid lupus erythematosus, Subacute cutaneous lupus erythematosus) * Scleroderma/Morphea * Linear scleroderma * Calcinosis cutis * Sclerodactyly * Ainhum

see also congenital, neoplasia

Template:Congenital malformations and deformations of integument

de:Morbus Hailey-Hailey nl:Familiaire benigne chronische pemphigus

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