Darier's Disease (ungual findings)

Diagnosis: Darier's Disease (ungual findings)

Wedge-shaped onycholysis. Submitted by Dr. Alaa Saad to dermRounds Dermatology Network

Clinical Presentation

Wedge-shaped onycholysis. Submitted by Dr. Alaa Saad to dermRounds Dermatology Network

Clinical History

Keratotic papules in seborrheic areas worsened by heat, sweat, and sun. Family history. Nail changes. Secondary infections.

Treatment

Topical retinoids, emollients, sun protection. Systemic retinoids (acitretin, isotretinoin) for severe disease. Treat secondary infections promptly. Avoid triggers.

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: dermatology, kodachrome