Skin-nontumor / Clinical dermatology
Blistering disorders
Darier's disease

Author: Mowafak Hamodat, M.D., MB.CH.B, MSc., FRCPC (see Authors page)

Revised: 13 June 2016, last major update July 2011

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: Darier's disease [title] skin

Cite this page: Darier's disease. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/skinnontumordariersdisease.html. Accessed December 4th, 2016.
Definition / General
  • Rare autosomal dominant genodermatoses with symmetric red-brown keratotic papules in seborrheic distribution, due to suprabasal dyskeratotic cells with large nuclei and a paranuclear halo
Terminology
  • Also called keratosis follicularis, Darier-White disease
Etiology
Clinical Features
  • Uncommon
  • Initial cutaneous findings usually develop after adolescence and before age 30; affects males and females equally
  • Symmetrical distribution of red-brown keratotic papules, may be unilateral or localized, may be verrucoid if close together
  • Greasy, malodorous papules and plaques on seborrheic areas and in flexures (trunk, axillae, neck)
  • Punctate keratoses or pits on palms and soles
  • Nails show subungual hyperkeratosis, fragility and splintering, with white and red alternating longitudinal bands and triangular nicking of the distal nail plate
  • May involve oral mucosa
Case Reports
Treatment
  • Oral or topical retinoids, topical or systemic corticosteroids, possibly surgery (eMedicine)
Clinical Images

Images hosted on PathOut servers:

Courtesy of Mark R. Wick, M.D.



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Keratotic papules

Micro Description
  • Acantholytic dyskeratosis
  • Suprabasal clefts contain dyskeratotic, basophilic cells with large nuclei and a paranuclear halo
  • Called grains if present in stratum corneum and corps ronds if present in granular layer
  • Also prominent irregular acanthosis and papillomatosis
  • May have prominent upper dermis infiltrates of lymphocytes
Micro Images

Images hosted on PathOut servers:

Courtesy of Mark R. Wick, M.D.

Case of the Week #155



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Characteristic acantholytic dyskeratosis

Acantholysis, dyskeratosis and chronic inflammatory dermal infiltrate, H&E

Symmetric brown papules on the trunk and on the scalp, H&E

Differential Diagnosis