Familial Benign Pemphigus

Professional Reference articles are written by UK doctors and are based on research evidence, UK and European Guidelines. They are designed for health professionals to use, so you may find the language more technical than the condition leaflets.

This page has been archived. It has not been updated since 26/10/2010. External links and references may no longer work.

Synonyms: familial benign chronic pemphigus, Hailey-Hailey disease

This is a rare inherited autosomal dominant skin disorder, caused by a genetic mutation in the ATP2C1 gene located on chromosome 3.[1] Occasionally sporadic cases do occur. There is a defect in keratinocyte cohesion, first described in 1939 by dermatologist brothers Hailey and Hailey.[2]

It may start in teenage years but it most commonly presents in the third and fourth decades. Vesicular or bullous rashes and erosions develop first in skin folds (axilla, groin, genitalia and under the breasts).[3] The lesions heal without scarring.

If the lesions persist they may become thickened, with maceration, itching and painful cracks. This can lead to secondary bacterial or candidal infection and malodour.

The trunk and neck can also be affected, with lesions provoked by friction, sun exposure, heat, and trauma.[1]

Fingernails may show white, longitudinal bands. Pits may occur on the palms.

Skin biopsy may be required, although appearance and family history is usually enough. The histology is characteristic.[3]

Supportive:

  • Avoid trigger factors - sunburn, friction and sweating; wear soft and absorbent clothing; avoid obesity.

Topical treatments:

Systemic treatments:

  • Prolonged courses of oral antibiotics (eg tetracycline or erythromycin) may help.
  • Immunosuppressants have been used, eg retinoids, ciclosporin, dapsone, methotrexate, systemic steroids and alefacept. However, no clinical trials have been performed.

Other treatments:

  • Phototherapy.
  • Low-dose botulinum toxin - to reduce sweating.
  • Carbon dioxide laser ablation[9] - although problems with hypertrophic scars have been reported.
  • Photodynamic therapy ± other treatments - with varying success.[10, 11]
  • In severe cases, affected areas can be removed surgically, but skin grafts may be required to close the skin deficit and scarring may be a problem.
  • Radiotherapy has been reported as successful in local disease control, although it does not seem to influence the natural course of the disease.
  • For most patients the condition is a 'nuisance' rather than a serious problem.
  • May have long remissions.
  • May improve with age.

Did you find this information useful?

Further reading & references

  1. Benign chronic pemphigus (Hailey-Hailey disease), Online Mendelian Inheritance in Man (OMIM)
  2. Michel B; "Familial benign chronic pemphigus" by Hailey and Hailey, April 1939. Commentary: Hailey-Hailey disease, familial benign chronic pemphigus. Arch Dermatol. 1982 Oct 118(10):774-83.
  3. Hailey-Hailey Disease, DermNet NZ; with images
  4. Helm TN et al; Familial Benign Pemphigus (Hailey-Hailey Disease), eMedicine, Mar 2010
  5. Warycha M, Patel R, Meehan S, et al; Familial benign chronic pemphigus (Hailey-Hailey disease). Dermatol Online J. 2009 Aug 15 15(8):15.
  6. Langenberg A, Berger TG, Cardelli M, et al; Genital benign chronic pemphigus (Hailey-Hailey disease) presenting as J Am Acad Dermatol. 1992 Jun 26(6):951-5.
  7. Tang MB, Tan ES; Hailey-Hailey disease: Effective treatment with topical cadexomer iodine. J Dermatolog Treat. 2010 Aug 1.
  8. Dammak A, Camus M, Anyfantakis V, et al; Successful treatment of Hailey-Hailey disease with topical 5-fluorouracil. Br J Dermatol. 2009 Oct 161(4):967-8. Epub 2009 Aug 7.
  9. Touma DJ, Krauss M, Feingold DS, et al; Benign familial pemphigus (Hailey-Hailey disease). Treatment with the pulsed Dermatol Surg. 1998 Dec 24(12):1411-4.
  10. Ruiz-Rodriguez R, Alvarez JG, Jaen P, et al; Photodynamic therapy with 5-aminolevulinic acid for recalcitrant familial benign J Am Acad Dermatol. 2002 Nov 47(5):740-2.
  11. Fernandez Guarino M, Ryan AM, Harto A, et al; Experience with photodynamic therapy in Hailey-Hailey disease. J Dermatolog Treat. 2008 19(5):288-90.
  12. Lee GH, Kim YM, Lee SY, et al; A case of eczema herpeticum with hailey-hailey disease. Ann Dermatol. 2009 Aug 21(3):311-4. Epub 2009 Aug 31.
  13. von Felbert V, Hampl M, Talhari C, et al; Squamous cell carcinoma arising from a localized vulval lesion of Hailey-Hailey Am J Obstet Gynecol. 2010 Sep 203(3):e5-7.
Author:
Dr Naomi Hartree
Document ID:
4104 (v23)
Last Checked:
26 October 2010
Next Review:
25 October 2015

Disclaimer: This article is for information only and should not be used for the diagnosis or treatment of medical conditions. Patient Platform Limited has used all reasonable care in compiling the information but make no warranty as to its accuracy. Consult a doctor or other health care professional for diagnosis and treatment of medical conditions. For details see our conditions.