Hailey-Hailey disease

Hailey-Hailey Disease (HHD) is a rare inherited skin disorder due to disturbed cell adhesion (acantholysis) in the upper layers of the epidermis. Hailey-Hailey disease usually develops in adolescence with a peak of onset between the second and fourth decade of life. Skin lesions begin with transient blistering, rapidly evolving into crusted erosions and scaling, fissured plaques. Primarily affected are the large skin folds (e.g. armpits, neck) and intertriginous areas (e.g. under the breasts, groin). The skin problems are exacerbated by trauma and irritation, for example due to heat, sweating, and friction. The disorder follows a chronic, recurrent course, often leading to discomfort, pain, and limitation of physical activity.

Tests Available

Forms and Documents

Test Details

  • Confirmation of the clinical diagnosis
  • Identification of at-risk family members, including pre-symptomatic testing
  • Prenatal diagnosis


3 weeks
2-5 mL Blood - Lavender Top Tube
Buccal Swabs

*Reporting times are typical, but could be extended in situations outside GeneDx's reasonable control.


  • 757.39 Other Accessory skin tags, congenital, Congenital scar, Epidermolysis bullosa, Keratoderma (congenital)
  • 694.5 Pemphigoid Benign pemphigus NOS, Bullous pemphigoid, Herpes circinatus bullosus, Senile dermatitis herpetiformis
For price inquiries please email zebras@genedx.com

**The CPT codes provided are based on AMA guidelines and are for informational purposes only. CPT coding is the sole responsibility of the billing party. Please direct any questions regarding coding to the payer being billed.


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  3. Hamada T et al. J Dermatol Sci. 2008 Jul;51(1):31-6.
  4. Foggia and Hovnanian Am J Med Genet (Part C) 131C: 20-31, 2004.
  5. Dobson-Stone C. et al. J Invest Dermatol 118:338-343, 2002.
  6. Ikeda S. et al. J Invest Dermatol 117:1654-1656, 2001.
  7. Sudbrak R. et al. Hum Mol Genet 9:1131-1140, 2000.
  8. Hu Z. et al. Nat Genet 24:61-65, 2000.