HAILEY- HAILEY DISEASE

DERMATOLOGY LECTURE NOTES

DEBABRATA BANDYOPADHYAY
PROFESSOR & HEAD, DEPT. OF DERMATOLOGY,
R. G. KAR MEDICAL COLLEGE, CALCUTTA, INDIA
 


Hailey-Hailey disease (HHD), also known as familial benign chronic pemphigus, is a rare, autosomal dominantly inherited blistering disorder. Recurrent blisters and erosions affecting body folds characterize HHD. Mutation in a gene encoding an adenosine triphosphatase (ATPase) calcium transporter results in defect in adhesion of epidermal cells by unknown mechanisms resulting in blisters.
 

  

ETIOLOGY

ATP2C1 gene, located on chromosome 3 encodes an ATPase important in calcium transport. Mutations of this gene causes impairment of calcium pump leading to abnormal keratinocyte adhesion by ill-understood mechanisms. This leads to acantholysis and intraepidermal blister formation.  

Sunlight, friction, irritation, and inflammation as well as bacterial and yeast infection may induce acantholysis and blister formation.

 

CLINICAL FEATURES

  • Onset of disease typically occurs during the third or fourth decade of life.

  • In areas prone to friction, like the axillae, groin, perineum, and neck, small flaccid blisters and pustules are formed. These soon rupture to form crusted erosions that spread peripherally while the central area heals.  

  • Hypertrophic, raised, moist vegetations may form on body folds, notably the groins. These may develop painful fissures.  

  • Itching and pain are frequent complaints.

  • Mucosal lesions are rarely reported.

  • Longitudinal white bands in the nails are said to be characteristic.

  • Herpes simplex infections may cause exacerbation of lesions with much pain.

  • Contact dermatitis, both allergic and irritant, may occur on lesions from topical applications.

  • Carcinoma has rarely been reported.

 

DIFFERENTIAL DIAGNOSIS

  • Impetigo

  • Infective eczema

  • Ringworm

  • Candidiasis

  • Pemphigus vegetans

  • Darier’s disease

 
 

DIAGNOSIS

 
 

Skin biopsy of lesions show epidermal acantholytic blisters. Full-thickness acantholysis may give the characteristic appearance of ‘dilapidated brick walls’.
 

  
 

COURSE AND PROGNOSIS

  
  The course of Hailey-Hailey disease is a chronic one, with exacerbation and partial remissions. the severity of the disease may diminish with aging.
 		  
 

TREATMENT

  
 

  • Avoidance of friction and maceration at body folds by wearing loose, cool garments

  • Keeping flexural areas clean and dry.

  • Topical steroids, rarely systemic steroids.

  • Topical antibiotics like tetracycline, fusidic acid.

  • Long term systemic antibiotics: tetracycline, erythromycin

  • PUVA in rare cases have been beneficial

  • Miscellaneous therapies: dapsone, methotrexate, topical vitamin D3 analogue.

  • Surgical :full-thickness excision and grafting, CO2 laser vaporization, dermabrasion

 
 

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