Darier's Disease (ungual findings)

Diagnosis: Darier's Disease (ungual findings)

Wedge-shaped onycholysis.

Clinical Presentation

Wedge-shaped onycholysis.

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, ungual, findings, alaa saad