Darier's Disease (keratosis follicularis)

Diagnosis: Darier's Disease (keratosis follicularis)

Keratotic papules on the neck and upper chest.

Clinical Presentation

Keratotic papules on the neck and upper chest.

Clinical History

Submitted by Alaa Saad. Originally posted September 17, 2010.

Treatment

See case discussion.

Differential Diagnosis

• Seborrheic dermatitis • Acne vulgaris • Hailey-Hailey disease • Grover disease • Pemphigus foliaceus • Confluent and reticulated papillomatosis

Key Learnings

• Autosomal dominant — mutation in ATP2A2 (SERCA2 pump) → defective calcium signaling → impaired desmosomal adhesion • Greasy, crusted, keratotic papules in seborrheic distribution (chest, back, scalp, forehead) • Nail changes: alternating red and white longitudinal bands, V-shaped nicking at free edge • Palmar pits are a subtle but specific finding • Heat, sweat, UV exposure, and friction worsen the condition • Secondary bacterial (S. aureus), viral (HSV — Kaposi varicelliform eruption), and fungal infections are common complications • Histology: corps ronds (dyskeratotic cells with clear halo) and corps grains — pathognomonic • Acrokeratosis verruciformis of Hopf may be a variant (same gene mutation)

Tags: darier039s, disease, keratosis, follicularis, alaa saad