Sean Klepper M.D.
Artur Zembowicz M.D....
Also known as: keratosis follicularis
- 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.
- 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.