Benign Familial Pemphigus (Hailey-Hailey Disease)

Benign Familial Pemphigus (Hailey-Hailey Disease)

Hailey-Hailey disease (HHD) is a rare autosomal dominant genodermatoses caused by mutations in the ATP2C1 gene that encodes a calcium pump of the Golgi apparatus. HHD is characterized by ruptured vesicles and blisters that tend to form eroded, erythematous plaques with painful “rhagades” in flexural areas. Histologically, acantholysis throughout the epidermis is a hallmark of HHD. There is no curative treatment. In mild cases, management is based on intermittent topical anti-inflammatory treatments and antiseptics applications. This activity reviews the pathophysiology, clinical presentation, evaluation, and management of patients with HHD and highlights the role of the interprofessional team in improving care for patients with this condition.

  • Provider:StatPearls, LLC
  • Activity Link: https://www.statpearls.com/ArticleLibrary/viewarticle/146204
  • Start Date: 2023-09-01 05:00:00
  • End Date: 2023-09-01 05:00:00
  • Credit Details: AMA PRA Category 1 Credit™️: 1.5 hours
    Nursing: 1.5 hours
    Pharmacy: 1.5 hours
  • MOC Credit Details: ABS - 1.5 Point; Credit Type(s): Accredited CME (ABS)
    ABPATH - 1.5 Point; Credit Type(s): Lifelong Learning (ABPATH)
    ABIM - 1.5 Point; Credit Type(s): Medical Knowledge (ABIM)
    ABS - 1.5 Point; Credit Type(s): Self-Assessment (ABS)
    ABP - 1.5 Point; Credit Type(s): Lifelong Learning and Self-Assessment (ABP)
  • Commercial Support: No
  • Activity Type: Enduring Material
  • CME Finder Type: Online Learning
  • Fee to Participate: Variable
  • Measured Outcome: Learner Knowledge, Learner/Team Competence
  • Provider Ship: Directly Provided
  • Registration: Open to all
  • Specialty: Adolescent Medicine, Dermatopathology, General Pediatrics, General Surgery, Internal Medicine, Molecular Genetic Pathology
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