Dermatology · Genodermatoses and Rare Disorders

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
Correct answer: B. Corps ronds in spinous layer and grains in granular layer with suprabasal acantholytic clefts

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.

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