Also known as: keratosis follicularis

Clinical Features:

• Autosomal dominant disorder characterized by scaly papules and plaques on the head, trunk and groin
• Nail lesions, palmar pits, white oral plaques and acrokeratosis verruciformis on the dorsa of the hands and feet are also seen.

Histologic Features:

• Acantholytic dyskeratosis with a suprabasal split
• "Corps ronds" (round dyskeratotic granular cells) and "grains" (spindle-shaped dyskeratotic parakeratotic cells) often seen within the acantholytic areas
• Acanthosis and parakeratosis
• Dermal papillae lined by basal cells protrude upward into the clefts formed by the acantholysis, forming "villi"
• Immunofluorescence shows granular C3 along the basement membrane zone.

Cases associated with this book:

Darier's disease
Author: Artur Zembowicz M.D. Ph.D.

Conference: DermatopathologyConsultations.com Teaching Collection