Skip to main content
Log in

Pemphigus chronicus benignus familiaris (Morbus Hailey-Hailey)

Erfolgreiche Behandlung mittels CO2-Laser

Familial benign chronic pemphigus (Hailey-Hailey disease)

Successful treatment with carbon dioxide laser

  • Kurzkasuistiken
  • Published:
Der Hautarzt Aims and scope Submit manuscript

Zusammenfassung

Morbus Hailey-Hailey ist eine seltene hereditäre Erkrankung mit einem autosomal-dominanten Erbgang. Wir präsentieren den Fall einer 56-jährigen Patientin, die sich mit einem seit 15 Jahren bestehenden, chronisch rezidivierenden und therapieresistenten M. Hailey-Hailey in unserer Klinik vorstellte. Aufgrund des frustranen Verlaufs unter konservativen Therapiemaßnahmen führten wir eine ablative CO2-Laserbehandlung der Hautläsionen in Intubationsnarkose durch. Im Rahmen der klinischen Kontrollen nach 6 Wochen und nach einem halben Jahr zeigte sich eine komplette und anhaltende Remission der Hautveränderungen in den behandelten Arealen. Nach unseren Erfahrungen stellt die Ablation mittels CO2-Laser eine effektive und nebenwirkungsarme Option bei der Behandlung eines therapieresistenten M. Hailey-Hailey dar.

Abstract

Hailey-Hailey disease is a rare, autosomal dominantly inherited genodermatosis, which manifests with recurrent intraepidermal blistering and erythematous hyperkeratotic patches in intertriginous areas. Conventional therapeutic approaches include topical and systemic steroids, antibiotic agents, as well as oral retinoids. Alternative treatments include surgical interventions such as excision, dermabrasion and laser ablation. A 56-year-old woman presented with 15-year history of severe therapy-resistant Hailey-Hailey disease. We performed laser ablation with carbon dioxide laser under general anesthesia. Six weeks after treatment complete remission within ablated areas was achieved. Six-month follow-up showed no relapse. In our opinion carbon dioxide laser ablation represents an effective and safe therapeutic option for Hailey-Hailey disease.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Abb. 1
Abb. 2

Literatur

  1. Bianchi L, Chimenti MS, Giunta A (2004) Treatment of Hailey-Hailey disease with topical calcitriol. J Am Acad Dermatol 51:475–476

    Article  PubMed  Google Scholar 

  2. Bel B, Jeudy G, Vabres P (2010) Dermoscopy of longitudinal leukonychia in Hailey-Hailey disease. Arch Dermatol 146(10):1204

    Article  PubMed  Google Scholar 

  3. Berger EM, Galadari HI, Gottlieb AB (2007) Case reports: successful treatment of Hailey-Hailey disease with acitretin. Drugs Dermatol 6:734–736

    Google Scholar 

  4. Burge SM (1992) Hailey-Hailey disease: the clinical features, response to treatment and prognosis. Br J Dermatol 126:275–282

    Article  PubMed  CAS  Google Scholar 

  5. Hamm H, Metze D, Bröcker EB (1994) Hailey-Hailey disease: eradication by dermabrasion. Arch Dermatol 130(9):1143–1149

    Article  PubMed  CAS  Google Scholar 

  6. Hu Z, Bonifas JM, Beech J et al (2000) Mutations in ATP2C1, encoding a calcium pump, cause Hailey-Hailey disease. Nat Genet 24:61–65

    Article  PubMed  CAS  Google Scholar 

  7. Kartamaa M, Reitamo S (1992) Familial benign chronic pemphigus (Hailey-Hailey disease): treatment with carbon dioxide laser vaporization. Arch Dermatol 128:646–648

    Article  PubMed  CAS  Google Scholar 

  8. Koeyers WJ, Van Der Geer S, Krekels G (2008) Botulinum toxin type A as an adjuvant treatment modality for extensive Hailey-Hailey disease. J Dermatol Treat 19(4):251–254

    Article  CAS  Google Scholar 

  9. Rabeni EJ, Cunningham NM (2002) Effective treatment of Hailey-Hailey disease with topical tacrolimus. J Am Acad Dermatol 47:797–798

    Article  PubMed  Google Scholar 

  10. Ruocco E, Brunetti G, Del Vecchio M, Ruocco V (2011) The practical use of cytology for diagnosis in dermatology. J Eur Acad Dermatol Venereol 25(2):125–129

    Article  PubMed  CAS  Google Scholar 

  11. Vilarinho C, Ventura F, Brito C (2010) Methotrexate for refractory Hailey-Hailey disease. J Eur Acad Dermatol Venereol 24(1):106

    Article  PubMed  CAS  Google Scholar 

  12. Zhang ZZ, Liang YH, Quan C et al (2008) Three novel ATP2C1 mutations in Chinese patients with Hailey-Hailey disease. Br J Dermatol 158:831–833

    Article  PubMed  CAS  Google Scholar 

Download references

Interessenkonflikt

Der korrespondierende Autor gibt an, dass kein Interessenkonflikt besteht.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to E. Diedrichson.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Kukova, G., Homey, B., Bruch-Gerharz, D. et al. Pemphigus chronicus benignus familiaris (Morbus Hailey-Hailey). Hautarzt 62, 258–261 (2011). https://doi.org/10.1007/s00105-011-2151-8

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00105-011-2151-8

Schlüsselwörter

Keywords

Navigation