Darier disease is a hereditary acantholytic dermatosis that presents as scaly crusted papules predominantly in seborrhoeic regions and skin folds. Hailey-Hailey disease (benign familial pemphigus) is a rare genetic skin disorder characterized by blistering. 

In this learning module, you will find a key point review on Darier disease and Hailey-Hailey disease focused on epidemiology, pathogenesis, clinical features, pathology, differential diagnosis and management. To test your knowledge, we refer you to the associated Quiz below.

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Sources

  1. Bolognia, J., Cerroni, L., & Schaffer, J. V. (2018). Dermatology. Philadelphia: Elsevier. 2-Wolverton, S. E. (2013). Comprehensive dermatologic drug therapy. Edinburgh: Saunders.

  2. Godic A, Miljkovic J, Kansky A, Vidmar G. Epidemiology of Darier’s Disease in Slovenia. Acta Dermatovenerol Alp Pannonica Adriat. 2005 Jun;14(2):43-8. PMID: 16001099.
  3. Jfri A, Litvinov IV, Netchiporouk E. Naltrexone for the Treatment of Darier and Hailey-Hailey Diseases. J Cutan Med Surg. 2019 Jul/Aug;23(4):453-454. PMID: 31337235.

  • Release Date: March 20 2023

  • Last Updated: March 20 2023

  • Time to complete: 30 minutes

  • Authors:

    • Dr. Elena Netchiporouk, MD, McGill University

    • Dr. Alireza Faghihi Kashani, MD, CISSS-AT

    • Dr. Lydia Ouchene, MD, McGill University,
    • Dr. Nickoo Merati, MD, McGill University
    • Dr. Rahul Nanda, MD, McGill University