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Dermatology
Darier Disease and Hailey–Hailey Disease
Built from Dermatology, 5th Edition
What’s inside
4 sections · 74 slides
Overview
- What this topic covers
Darier Disease
Keratotic papules in a seborrheic distribution, from a faulty calcium pump
- Darier disease: key features
- History of Darier disease
- Epidemiology of Darier disease
- The calcium pump behind Darier disease
- From low calcium to acantholysis and dyskeratosis
- Onset and distribution of Darier disease
- Truncal and facial involvement
- Facial and hairline involvement in Darier disease
- Distribution patterns: three related disorders
- Comparative distribution of Darier, Hailey–Hailey, and Grover disease
- Guttate leukoderma in Darier disease
- Intertriginous Darier disease in the groin and abdominal fold
- Intertriginous involvement and malodor
- Acral and palmoplantar findings
- Palmar keratotic papules and depressions
- Nail changes in Darier disease
- Oral mucosal involvement
- Symptoms and disease course
- What makes Darier disease flare
- Infectious complications
- Kaposi varicelliform eruption (KVE)
- Salivary glands, nerves, and mood
- Other associations
- Acral hemorrhagic type
- Less common presentations of Darier disease
- Less common presentations of Darier disease (continued)
- Segmental (mosaic) forms of Darier disease
- Pathology: acantholysis and dyskeratosis
- Darier disease versus Grover disease on biopsy
- Acrokeratosis verruciformis of Hopf
- Other conditions in the differential diagnosis
- Treatment: general skin care
- Topical and intralesional therapy
- Systemic therapy for Darier disease
- Surgical therapy for focal disease
Hailey–Hailey Disease
Blistering and erosions of the skin folds, from a Golgi calcium pump defect
- Hailey–Hailey disease: key features
- History of Hailey–Hailey disease
- Epidemiology of Hailey–Hailey disease
- The calcium pump behind Hailey–Hailey disease
- Darier disease and Hailey–Hailey disease side by side
- Onset and typical distribution
- From blisters to circinate plaques
- Nails and mucosa in Hailey–Hailey disease
- Symptoms, triggers, and course
- Infectious and other complications
- Segmental forms of Hailey–Hailey disease
- Pathology: the “dilapidated brick wall”
- Differential diagnosis of Hailey–Hailey disease
- Treatment: general measures
- Topical therapy for Hailey–Hailey disease
- Surgical and physical therapy
- Systemic therapy for Hailey–Hailey disease
Additional Clinical Photographs
Further examples of Darier disease and Hailey–Hailey disease
- Red-brown keratotic papules on the abdomen
- Confluent chest and neck papules with a central scar
- Nail erythronychia and free-edge notching
- Inguinal and scrotal erosions in Hailey–Hailey disease
- Erosive plaques with crusting in Hailey–Hailey disease
- Key takeaways
- References
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- References (continued)
- Dermatology, 5th Edition (2-Volume Set)