What is familial pemphigus?
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.
What is another name of familial benign pemphigus?
Description. Hailey-Hailey disease, also known as benign chronic pemphigus, is a rare skin condition that usually appears in early adulthood. The disorder is characterized by red, raw, and blistered areas of skin that occur most often in skin folds, such as the groin, armpits, neck, and under the breasts.
Is Hailey-Hailey disease genetic?
Hailey-Hailey disease is caused by a genetic change (mutation) in the ATP2C1 gene. The ATP2C1 gene contains instructions for creating (encoding) a protein that acts as a calcium and magnesium pump in the cells.
Is pemphigus autosomal recessive or dominant?
Familial benign pemphigus is a chronic autosomal dominant disorder with incomplete penetrance. Approximately two thirds of patients with familial benign pemphigus have a family history of the disorder. A history of multiple relapses and remissions is characteristic.
How do I get rid of Hailey-Hailey?
There is no cure for Hailey-Hailey disease since it is a genetic disorder. Treating patients for the symptoms does provide patients with relief and reduces the microbial infections. The first part of treatment is to avoid friction and sweating by wearing lightweight clothes.
How is Hailey-Hailey diagnosed?
The diagnosis of Hailey-Hailey disease can be confirmed by examining a sample of the skin (a biopsy) under the microscope. A test called direct immunofluorescence is also performed on a skin biopsy to confirm the diagnosis.
Why is it called Hailey-Hailey?
Hailey–Hailey disease, or familial benign chronic pemphigus or familial benign pemphigus, was originally described by the Hailey brothers (Hugh Edward and William Howard) in 1939….Hailey–Hailey disease.
| Hailey-Hailey disease | |
|---|---|
| Other names | Familial benign chronic pemphigus |
| Specialty | Medical genetics |
Does pemphigus run in families?
Abstract. Pemphigus vulgaris (PV) is a chronic autoimmune bullous disease of the skin and mucous membranes. Although there is some evidence pointing towards a genetic predisposition by some human leukocyte antigen (HLA) genes, familial occurrence of PV is very rare.
Is Pemphigus Foliaceus hereditary?
Pemphigus and pemphigoid are not genetic (hereditary) though there can be a genetic predisposition to develop the disease.
When does Hailey-Hailey disease start?
What does Hailey-Hailey disease look like? The condition usually first appears between the ages of 15 and 40 years, but it may begin at any age. The severity of the condition varies widely and is unpredictable.
Is familial benign pemphigus life threatening?
Familial benign pemphigus causes discomfort but is not life threatening. Benign familial pemphigus lesions often begin during the teenage years and manifest as itchy and malodorous plaques. Patients with familial benign pemphigus live long and productive lives. The skin disorder is more of a nuisance than a serious health threat.
What are the treatment options for benign familial chronic pemphigus?
Possible treatments include topical and oral therapy, light and laser therapy, and surgery with skin grafting. This review covers topical, systemic, laser, and surgical treatments for benign familial chronic pemphigus, with updates based on recent literature and case reports.
What is the history of familial benign pemphigus (Hailey-Hailey disease)?
Background. Familial benign pemphigus (Hailey-Hailey disease) originally was described by the Hailey brothers in 1939. [1] Familial benign pemphigus is a chronic autosomal dominant disorder with incomplete penetrance. Approximately two thirds of patients with familial benign pemphigus have a family history of the disorder.
When was pemphigus first discovered?
It is a rare hereditary blistering skin disease first described by the Hailey brothers in 1939. Who gets benign familial pemphigus? Benign familial pemphigus usually appears in the second to fourth decade, although it can occur at any age.