Darier's disease (keratosis follicularis) is caused by mutation in ATP2A2 gene encoding SERCA2 (sarco/endoplasmic reticulum Ca2+-ATPase). The hallmark histological feature is:
- A Subcorneal blistering with dyskeratosis
- B Corps ronds in spinous layer and grains in granular layer with suprabasal acantholytic clefts ✓
- C Hypergranulosis with saw-tooth rete ridges
- D Munro microabscesses with parakeratosis
Explanation
Darier's disease shows three key histological features: (1) suprabasal acantholytic clefts (lacunae), (2) corps ronds — large dyskeratotic cells with pyknotic nuclei in the spinous layer, and (3) grains — small parakeratotic dyskeratotic cells in the granular/corneal layer. The SERCA2 mutation impairs calcium pump function, disrupting desmosome assembly and keratinocyte cohesion. Clinically, greasy crusted papules in sebaceous distribution occur; Nikolsky sign is negative.
Reference: Neena Khanna Illustrated Synopsis of Dermatology & STD, 6th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.