Darier and Hailey-Hailey disease: update 2021

Area of Science:

• Dermatology
• Genetics
• Calcium Channel Biology

Background:

• Darier disease and Hailey-Hailey disease are autosomal-dominant genodermatoses.
• Both conditions stem from defective ATPases in epidermal calcium channels, impairing keratinocyte adhesion.
• Despite shared pathogenesis, they exhibit distinct clinical features and therapeutic responses.

Purpose of the Study:

• To summarize current knowledge on Darier disease and Hailey-Hailey disease.
• To highlight differences in clinical presentation and treatment options.
• To incorporate recent advancements in understanding and managing these genodermatoses.

Main Methods:

• Review of current literature and clinical knowledge on Darier disease and Hailey-Hailey disease.
• Comparison of clinical manifestations, pathogenesis, and treatment strategies.
• Inclusion of recent therapeutic developments and evidence-based approaches.

Main Results:

• Darier disease presents with keratotic papules in seborrheic/intertriginous areas.
• Hailey-Hailey disease features intertriginous papulovesicles evolving into plaques with erosions and fissures.
• Quality of life is significantly impacted by symptoms like itching, pain, and malodor; treatment options remain limited.

Conclusions:

• Therapeutic options for Darier disease and Hailey-Hailey disease are limited, with antiseptics and topical corticosteroids as mainstays.
• Systemic treatments like acitretin (for Darier disease) and low-dose naltrexone (more effective in Hailey-Hailey disease) show variable efficacy.
• Ablative surgery and botulinum toxin injections offer potential long-term or temporary relief, respectively, particularly for Hailey-Hailey disease.