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Hailey - Hailey disease , also experience as genetic benign pemphigus , is a uncommon hereditaryskin diseasecharacterized by erosions and blisters on the hide , painful skin rash in the tegument folds like neck opening , armpits , groin , beneath the breast , and between the fundament . Patient can also have subaltern bacterial infection . symptom exasperate during summer from heat , friction and perspiration . The movement of Hailey - Hailey disease is mutation in the ATP2C1 gene . The symptoms of Hailey - Hailey disease are similar to pemphigus ; however , pemphigus is an autoimmune disease , whereas , Hailey - Hailey disease is not an autoimmune upset , but is a raregenetic disorder . examination reveals no autoantibodies in Hailey - Hailey disease .
There is no cure for this condition . Treatment is done to assuage the symptom and forestall further flare ups .
Causes of Hailey-Hailey Disease or Familial Benign Pemphigus
Hailey - Hailey Disease or Familial Benign Pemphigus is a rare tegument disorderliness . This is an familial condition . There are some rarified cases where this precondition may pass off without a crime syndicate account . Mutations in the ATP2C1 gene are identified as the effort behind this disease . This transmitted defect results in legal separation of the pelt cells from each other . Normal tegument cells are arranged compactly together ; but in Hailey - Hailey disease , due to the genetic defect , the cell separate and fall apart resulting in rashes and blistering .
Signs and Symptoms of Hailey-Hailey Disease or Familial Benign Pemphigus
Investigations for Hailey-Hailey Disease or Familial Benign Pemphigus
Physical examination and family history helps with the diagnosis . However , Hailey - Hailey Disease or Familial Benign Pemphigus can be mistake for other similar tegument circumstance , such as thrush , impetigo , tinea etc . Skin biopsy is done to confirm the diagnosis , as Hailey - Hailey disease has characteristic histology with mien of stratum of dislocated skin cellphone . The immunofluorescence test for antibody in Hailey - Hailey disease is also minus .
Treatment for Hailey-Hailey Disease or Familial Benign Pemphigus
There is no lasting remedy for this condition . handling is done to manage the symptoms and reduce further flare pass - ups . discussion depend on the rigourousness of the symptoms of the disease and includes :
Home Remedies for Hailey-Hailey Disease or Familial Benign Pemphigus
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