Hailey-Hailey Disease - Western SummaryWestern Medicine Summary

Western Medicine

Western Summary

Hailey-Hailey disease, also called familial benign chronic pemphigus, is a rare genetic skin condition.

Symptoms

Hailey-Hailey disease begins as a painful rash with cracks, taking place in skin folds such as the armpits, neck, under the breasts, and groin. The rash may come and go without leaving scars, but bacterial and yeast infections may occur, resulting in a bad odor. The disease can occur at any age but typically appears in people between the ages of 30-50. 

Causes

Hailey-Hailey is an inherited disorder caused by a genetic mutation in a protein called SPCA1. This protein pumps calcium and manganese ions at the cellular level to help skin cells (keratinocytes) stick together. When this pump does not work correctly, the skin breaks down easily, leading to lesions in areas of friction (such as the skin folds). 

 

Treatments

Unfortunately, there is no cure for Hailey-Hailey disease. Treatments are aimed to decrease symptoms and prevent worsening. It is important to avoid things that can trigger a flare such as heat, sweating, friction, and sunburns. It is also important to wear loose clothing.

Some of the medications used to treat Hailey-Haily include:

  • Topical steroids to treat inflamed lesions and prevent flares
  • Topical antibiotic and antifungal medications to treat and prevent infections
  • Benzoyl peroxide washes or creams to treat and prevent infections

Botulinum toxin

Botulinum toxin injections may work by decreasing sweating but the true manner by which they work is not clear. 

 

Phototherapy

Phototherapy is where the skin is intentionally exposed to light (ultraviolet, visible, or infrared) light to improve a medical condition. In the case of Hailey-Hailey, narrowband UVB has been reported to be helpful in isolated case reports.[1,2]

 

Photodynamic therapy

Photodynamic therapy involves the topical application of aminolevulinic acid and then allowing the skin cells to convert that medication to protoporphiryn IX, which is senstive to blue and red light. The results with photodynamic therapy are mixed with some medical reports showing improvement[3-5] while others do not.[6]

 

Surgery

The affected skin may be removed surgically in severe cases. 

 

  1. Hamada T, Umemura H, Aoyama Y, et al. Successful therapeutic use of targeted narrow-band ultraviolet B therapy for refractory Hailey-Hailey disease. Acta Derm Venereol.2013;93(1):110-111; PMID: 22735754.
  2. Mizuno K, Hamada T, Hashimoto T, et al. Successful treatment with narrow-band UVB therapy for a case of generalized Hailey-Hailey disease with a novel splice-site mutation in ATP2C1 gene. Dermatol Ther.2014;27(4):233-235; PMID: 24962025.
  3. Lobato-Berezo A, Imbernon-Moya A, Aguilar-Martinez A. Refractory Hailey-Hailey Disease That Responded Well to Photodynamic Therapy. Actas Dermosifiliogr.2015;106(10):852-854; PMID: 26387451.
  4. Ruiz-Rodriguez R, Alvarez JG, Jaen P, et al. Photodynamic therapy with 5-aminolevulinic acid for recalcitrant familial benign pemphigus (Hailey-Hailey disease). J Am Acad Dermatol.2002;47(5):740-742; PMID: 12399767.
  5. Yan XX, Tian HQ, Wang CL, et al. Successful treatment of hailey-hailey disease with aminolevulinic Acid photodynamic therapy. Ann Dermatol.2015;27(2):222-223; PMID: 25834370.
  6. Fernandez Guarino M, Ryan AM, Harto A, et al. Experience with photodynamic therapy in Hailey-Hailey disease. J Dermatolog Treat.2008;19(5):288-290; PMID: 18608733.