Hailey-Hailey Disease or Familial Benign Pemphigus

Hailey-Hailey disease, also known as familial benign pemphigus, is a rare hereditary skin disease characterized by erosions and blisters on the skin, painful rash in the skin folds like neck, armpits, groin, beneath the breasts, and between the buttocks. Patient can also have secondary bacterial infection. Symptoms worsen during summer from heat, friction and sweating. The cause of Hailey-Hailey disease is mutations 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 disorder, but is a rare genetic disorder. Testing reveals no autoantibodies in Hailey-Hailey disease.

Hailey-Hailey Disease or Familial Benign Pemphigus

There is no cure for this condition. Treatment is done to alleviate the symptoms and prevent further flare ups.

Causes of Hailey-Hailey Disease or Familial Benign Pemphigus

Hailey-Hailey Disease or Familial Benign Pemphigus is a rare skin disorder. This is an inherited condition. There are some rare cases where this condition may occur without a family history. Mutations in the ATP2C1 gene are identified as the cause behind this disease. This genetic defect results in separation of the skin cells from each other. Normal skin cells are arranged compactly together; but in Hailey-Hailey disease, due to the genetic defect, the cells separate and fall apart resulting in rashes and blistering.

Signs and Symptoms of Hailey-Hailey Disease or Familial Benign Pemphigus

  • Hailey-Hailey Disease or Familial Benign Pemphigus often affects individuals aged in their 30s and 40s.
  • It starts with an erosive and painful skin rash occurring in the skin folds, such as armpits, neck, groins, and under the breasts etc.
  • The blistering lesions tend to come and go with no scarring.
  • The lesions slowly increase in size and the centre clears up resulting in a distinctive ring shape.
  • Old lesions become thick and the skin separates resulting in painful cracks.
  • Patient often has secondary bacterial infection, which can cause foul smell.
  • Patient may also have white stripes on the fingernails and indentations in the palms of the hands.
  • Factors which worsen this condition include sweating, heat and friction.

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 mistaken for other similar skin conditions, such as thrush, impetigo, tinea etc. Skin biopsy is done to confirm the diagnosis, as Hailey-Hailey disease has characteristic histology with presence of layers of separated skin cells. The immunofluorescence test for antibodies in Hailey-Hailey disease is also negative.

Treatment for Hailey-Hailey Disease or Familial Benign Pemphigus

There is no permanent cure for this condition. Treatment is done to manage the symptoms and reduce further flare-ups. Treatment depends on the severity of the symptoms of the disease and includes:

  • Topical antibiotics and corticosteroid creams are prescribed for mild cases. They help in relieving the symptoms.
  • Oral antibiotics or stronger corticosteroid creams are prescribed in severe cases.
  • Carbon dioxide laser treatment may be done for severe cases.
  • Botulinum toxin can also be prescribed to reduce sweating.
  • Phototherapy using UV (ultraviolet) light also helps.
  • Oral antivirals (acyclovir) are given if the patient has herpes virus infection.
  • Various other oral medications, such as dapsone, ciclosporin, retinoids, and methotrexate have also found to be partially effective; however, research is still being done on them.
  • Surgery is done for extremely severe conditions where the affected skin is removed and skin grafts are placed over the wounds for healing.

Home Remedies for Hailey-Hailey Disease or Familial Benign Pemphigus

  • Patients are told to avoid "triggers," such as sweating, friction and sunburn.
  • They should also keep the affected regions clean and dry.
  • Always use sunscreen before stepping out.
  • Always wear loose fitting clothes.
  • Apply a moisturizer after bathing.
  • Try to avoid excessive heat, as this can worsen and cause more outbreaks.
  • Application of cool compresses also help in relieving the symptoms.
  • Keeping the affected regions clean and dry is important to avoid secondary bacterial, viral and fungal infections.

Written, Edited or Reviewed By:

, MD, FFARCSI

Last Modified On: June 7, 2014

Pain Assist Inc.

Pramod Kerkar
  Note: Information provided is not a substitute for physician, hospital or any form of medical care. Examination and Investigation is necessary for correct diagnosis.

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