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Dr. Andrew Rynne
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Dr. Andrew Rynne

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Exp 50 years

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Article Home Skin Disorders Hailey Hailey Disease

Hailey Hailey Disease

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Hailey-Hailey disease, sometimes called ?familial benign chronic pemphigus? is a rare hereditary blistering skin disease. A history of multiple relapses and remissions is characteristic.

 

Causes

Hailey-Hailey disease, or familial benign pemphigus is an autosomal dominant disorder, is hypothesized to result from a genetic defect in a calcium pump protein - ATP2C1.

This causes the cells of the skin to not adhere together properly, causing the blisters and rashes.

Signs and symptoms

  • Vesicles and erythematous plaques with overlying crusts typically occur in the genital area, as well as the chest, neck, and axillary areas.
  • Common sites include the armpits, groins, and neck, under the breasts and between the buttocks.
  • Burning and itching is associated the eruption, and a malodorous discharge occurs in some cases as a result of secondary infection.
  • The lesions tend to come and go and leave no scars. As the lesions get bigger the centre clears leaving a typical ring shape.
  • Multiple asymptomatic longitudinal white bands or ridges on the fingernails
  • Heat, sweating and friction often exacerbates the disease, and most patients have worsening of symptoms during the summer months

Tests and diagnosis

Diagnosis by skin biopsy

Treatment

  • Avoiding triggering factors such as sunburn, sweating and friction helps to control the disease
  • Topical corticosteroid creams are effective in treating lesions and preventing exacerbations
  • Combination corticosteroid/ antibiotic creams are helpful, as treating the secondary infection is important
  • Prolonged courses of oral antibiotics such as tetracycline may be useful
  • A number of other oral medications (retinoid, cyclosporine, dapsone, and methotrexate) have been reported in single cases as partially effective
  • Wet compresses to dry up oozing patches
  • Botulinum toxin to reduce sweating or hyperhidrosis
  • Phototherapy (ultraviolet light A and B) has also been used.
  • Lasers have been reported to be useful in one study, vaporizing the affected skin.
  • In severe cases surgery can be performed to remove the affected skin. Skin grafts are usually necessary to repair the wounds.

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