Hailey–Hailey disease: Difference between revisions
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{{Infobox medical condition | |||
| name = Hailey–Hailey disease | |||
| image = [[File:Before_Magnesium_Chloride.jpg|left|thumb|Lesions of Hailey–Hailey disease]] | |||
| caption = Lesions of Hailey–Hailey disease | |||
| synonyms = [[Benign chronic pemphigus]] | |||
| pronounce = | |||
| specialty = [[Dermatology]] | |||
| symptoms = [[Blisters]], [[Erosions]], [[Itching]], [[Pain]] | |||
| complications = [[Secondary infection]] | |||
| onset = Usually in [[adulthood]] | |||
| duration = [[Chronic]] | |||
| causes = [[Genetic mutation]] in the [[ATP2C1]] gene | |||
| risks = [[Heat]], [[sweating]], [[friction]] | |||
| diagnosis = [[Clinical examination]], [[Skin biopsy]] | |||
| differential = [[Pemphigus vulgaris]], [[Intertrigo]], [[Candidiasis]] | |||
| treatment = [[Topical corticosteroids]], [[Antibiotics]], [[Laser therapy]] | |||
| medication = [[Corticosteroids]], [[Antibiotics]] | |||
| frequency = Rare | |||
| deaths = | |||
}} | |||
'''Hailey–Hailey disease''', also known as '''familial benign chronic pemphigus''', is a rare [[genetic disorder]] that affects the skin. It was first described by the Hailey brothers in 1939. The condition is characterized by recurrent blisters and erosions, primarily in areas where the skin folds, such as the armpits, groin, neck, and under the breasts. | |||
==Signs and Symptoms== | ==Signs and Symptoms== | ||
The primary symptoms of Hailey–Hailey disease include: | The primary symptoms of Hailey–Hailey disease include: | ||
| Line 8: | Line 28: | ||
* Crusting and oozing lesions | * Crusting and oozing lesions | ||
* Secondary bacterial infections | * Secondary bacterial infections | ||
These symptoms often worsen with heat, sweating, friction, and stress. The condition typically begins in adolescence or early adulthood and can vary in severity over time. | These symptoms often worsen with heat, sweating, friction, and stress. The condition typically begins in adolescence or early adulthood and can vary in severity over time. | ||
==Genetics== | ==Genetics== | ||
Hailey–Hailey disease is inherited in an [[autosomal dominant]] pattern, meaning only one copy of the altered gene is sufficient to cause the disorder. The disease is caused by mutations in the [[ATP2C1]] gene, which encodes a protein involved in calcium transport within cells. This mutation leads to impaired cell adhesion in the epidermis, resulting in the characteristic blisters and erosions. | Hailey–Hailey disease is inherited in an [[autosomal dominant]] pattern, meaning only one copy of the altered gene is sufficient to cause the disorder. The disease is caused by mutations in the [[ATP2C1]] gene, which encodes a protein involved in calcium transport within cells. This mutation leads to impaired cell adhesion in the epidermis, resulting in the characteristic blisters and erosions. | ||
==Diagnosis== | ==Diagnosis== | ||
Diagnosis of Hailey–Hailey disease is primarily based on clinical examination and patient history. A [[skin biopsy]] may be performed to confirm the diagnosis, revealing characteristic findings such as acantholysis, which is the loss of connections between skin cells. | Diagnosis of Hailey–Hailey disease is primarily based on clinical examination and patient history. A [[skin biopsy]] may be performed to confirm the diagnosis, revealing characteristic findings such as acantholysis, which is the loss of connections between skin cells. | ||
==Treatment== | ==Treatment== | ||
There is no cure for Hailey–Hailey disease, but various treatments can help manage symptoms. These include: | There is no cure for Hailey–Hailey disease, but various treatments can help manage symptoms. These include: | ||
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* Laser therapy to reduce lesions | * Laser therapy to reduce lesions | ||
* Botulinum toxin injections to reduce sweating | * Botulinum toxin injections to reduce sweating | ||
Patients are also advised to avoid triggers such as heat, friction, and stress, and to maintain good skin hygiene. | Patients are also advised to avoid triggers such as heat, friction, and stress, and to maintain good skin hygiene. | ||
==Prognosis== | ==Prognosis== | ||
The prognosis for individuals with Hailey–Hailey disease varies. While the condition is chronic and can be debilitating, it is not life-threatening. With appropriate management, many individuals can lead relatively normal lives. | The prognosis for individuals with Hailey–Hailey disease varies. While the condition is chronic and can be debilitating, it is not life-threatening. With appropriate management, many individuals can lead relatively normal lives. | ||
==See also== | |||
== | |||
* [[Genetic disorder]] | * [[Genetic disorder]] | ||
* [[Skin biopsy]] | * [[Skin biopsy]] | ||
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* [[Botulinum toxin]] | * [[Botulinum toxin]] | ||
[[Category:Genetic disorders]] | [[Category:Genetic disorders]] | ||
[[Category:Dermatology]] | [[Category:Dermatology]] | ||
[[Category:Rare diseases]] | [[Category:Rare diseases]] | ||
{{Genetic-disorder-stub}} | {{Genetic-disorder-stub}} | ||
Latest revision as of 03:50, 7 April 2025
Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD medical weight loss NYC and sleep center NYC
| Hailey–Hailey disease | |
|---|---|
 | |
| Synonyms | Benign chronic pemphigus |
| Pronounce | |
| Specialty | Dermatology |
| Symptoms | Blisters, Erosions, Itching, Pain |
| Complications | Secondary infection |
| Onset | Usually in adulthood |
| Duration | Chronic |
| Types | N/A |
| Causes | Genetic mutation in the ATP2C1 gene |
| Risks | Heat, sweating, friction |
| Diagnosis | Clinical examination, Skin biopsy |
| Differential diagnosis | Pemphigus vulgaris, Intertrigo, Candidiasis |
| Prevention | N/A |
| Treatment | Topical corticosteroids, Antibiotics, Laser therapy |
| Medication | Corticosteroids, Antibiotics |
| Prognosis | N/A |
| Frequency | Rare |
| Deaths | |
Hailey–Hailey disease, also known as familial benign chronic pemphigus, is a rare genetic disorder that affects the skin. It was first described by the Hailey brothers in 1939. The condition is characterized by recurrent blisters and erosions, primarily in areas where the skin folds, such as the armpits, groin, neck, and under the breasts.
Signs and Symptoms[edit]
The primary symptoms of Hailey–Hailey disease include:
- Painful blisters and erosions
- Red, inflamed skin
- Itching and burning sensations
- Crusting and oozing lesions
- Secondary bacterial infections
These symptoms often worsen with heat, sweating, friction, and stress. The condition typically begins in adolescence or early adulthood and can vary in severity over time.
Genetics[edit]
Hailey–Hailey disease is inherited in an autosomal dominant pattern, meaning only one copy of the altered gene is sufficient to cause the disorder. The disease is caused by mutations in the ATP2C1 gene, which encodes a protein involved in calcium transport within cells. This mutation leads to impaired cell adhesion in the epidermis, resulting in the characteristic blisters and erosions.
Diagnosis[edit]
Diagnosis of Hailey–Hailey disease is primarily based on clinical examination and patient history. A skin biopsy may be performed to confirm the diagnosis, revealing characteristic findings such as acantholysis, which is the loss of connections between skin cells.
Treatment[edit]
There is no cure for Hailey–Hailey disease, but various treatments can help manage symptoms. These include:
- Topical corticosteroids to reduce inflammation
- Antibiotics to treat secondary infections
- Antifungal creams if a fungal infection is present
- Oral retinoids in severe cases
- Laser therapy to reduce lesions
- Botulinum toxin injections to reduce sweating
Patients are also advised to avoid triggers such as heat, friction, and stress, and to maintain good skin hygiene.
Prognosis[edit]
The prognosis for individuals with Hailey–Hailey disease varies. While the condition is chronic and can be debilitating, it is not life-threatening. With appropriate management, many individuals can lead relatively normal lives.
See also[edit]
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