Darier's disease

    Article Contributors: 
    Sean Klepper M.D.
    Artur Zembowicz M.D....

    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.
    External Links:

    Cases associated with this book:

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

    Conference: DermatopathologyConsultations.com Teaching Collection