Hailey-Hailey disease (syn. familial benign chronic pemphigus)

Hailey-Hailey disease is a rare, autosomal dominant, intraepidermal, blistering condition. The Hailey brothers first described it in 1939.

This chapter is set out as follows:

• Hailey-Hailey disease is inherited as an autosomal dominant condition
• Occasionally sporadic cases arise without a family history

• It usually appears in the third or fourth decade, although can arise at any age
• Heat, sweating and friction often exacerbate the condition, and most patients have more troublesome symptoms during the summer months

Distribution

• ​Common sites are those exposed to friction such as the neck, axilla, under the breasts, the groins and inbetween the buttocks

Morphology

• Typically begins with painful erosions 
• Lesions extend peripherally, healing in the centre without scarring 
• If the lesions are present for some time they may become thickened and malodorous with soft vegetations and fissures

Other cutaneous features

• Longitudinal white bands on the fingernails, and pits on the palms are sometimes seen
• Secondary bacterial and viral infection are common, especially with herpes simplex

• Hailey-Hailey disease is usually diagnosed by its appearance and family history, although it is often mistaken for other skin conditions such as candida intertrigo or eczema 

• Histology of lesional skin from HHD demonstrates widespread partial loss of cohesion between suprabasal keratinocytes, or acantholysis, said to resemble a ‘dilapidated brick wall’. Acantholytic clefts and bullae form suprabasally, and may contain floating clusters of loosely coherent cells (reference: Rook's Textbook of Dermatology)

 Step 1: general measures

• Provide a patient information leaflet 
• Unfortunately there is no cure for Hailey-Hailey disease, treatment is aimed at reducing symptoms and preventing flares
• Avoid triggers such as sunburn, sweating and friction
• Wear loose fitting cool clothing and wear absorbent pads
• Weight loss, if appropriate, may help 
• Antiseptic washes, such as the Dermol ® range, may help reduce secondary infection  
• If there is a significant exacerbation send swabs for bacteriology and virology (herpes simplex) and treat accordingly

Step 2: topical steroids 

• ​Combination treatments of corticosteroids with antibacterial and / or antifungal treatments may be effective eg Trimovate ® cream or Lotriderm ® cream  

Step 3: systemic antibiotics 

• Tetracycline's (lymecycline 408 mg OD or doxycycline 100 mg OD) and erythromycin / clarithromycin have anti-inflammatory properties in the skin, and can be given for trial periods of three months. If beneficial they can be used in the longer-term  

Step 4: treatments provided by specialists 

• Naltrexone
• Recent studies have found Naltrexone to be effective 
• The initial starting dose is 1.5 mg OD; gradually increasing by 1.5 mg every 1-2 weeks until 4.5 mg OD is reached depending on tolerance / response. Currently, there is no reliable evidence that doses higher than 4.5 mg OD are likely to be of additional benefit 
• The time to assess effectiveness is 4-6 months, although some may see improvement within several weeks
• LFT need to be checked at baseline and then after 8-12 weeks. If there are no significant issues these do not need repeating 

• Other treatments
  • There is very limited evidence for calcipotriol cream, tacrolimus ointment, oral retinoids and ciclosporin
  • The evidence is equally uncertain for PUVA phototherapy, cryosurgery, laser, photodynamic therapy and surgical excision  
  • Botulinum toxin may help by reducing sweating

Prognosis 

• Many patients have long remissions and an improvement with age