Diseases

Darier Disease

An autosomal dominantly inherited skin disorder characterized by warty malodorous papules that coalesce into plaques. It is caused by mutations in the ATP2A2 gene encoding SERCA2 protein, one of the SARCOPLASMIC RETICULUM CALCIUM-TRANSPORTING ATPASES. The condition is similar, clinically and histologically, to BENIGN FAMILIAL PEMPHIGUS, another autosomal dominant skin disorder. Both diseases have defective calcium pumps (CALCIUM-TRANSPORTING ATPASES) and unstable desmosomal adhesion junctions (DESMOSOMES) between KERATINOCYTES.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

An autosomal dominantly inherited skin disorder characterized by warty malodorous papules that coalesce into plaques. It is caused by mutations in the ATP2A2 gene encoding SERCA2 protein, one of the SARCOPLASMIC RETICULUM CALCIUM-TRANSPORTING ATPASES. The condition is similar, clinically and histologically, to BENIGN FAMILIAL PEMPHIGUS, another autosomal dominant skin disorder. Both diseases have defective calcium pumps (CALCIUM-TRANSPORTING ATPASES) and unstable desmosomal adhesion junctions (DESMOSOMES) between KERATINOCYTES.

MeSH Record

Classification

Broader headings

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MeSH Record

Synonyms

20 entry terms
  • Darier's Disease
  • Darier-White Disease
  • Keratosis Follicularis
  • Darier White Disease
  • Darier-White Diseases
  • Dariers Disease
  • Disease, Darier
  • Disease, Darier's
  • Disease, Darier-White
  • Diseases, Darier-White
  • Acantholytic Dyskeratotic Epidermal Nevi
  • Acantholytic Dyskeratotic Epidermal Nevus
  • Acrokeratosis Verruciformis
  • Acrokeratosis Verruciformis of Hopf
  • Hopf Disease
  • Disease, Hopf
  • Diseases, Hopf
  • Hopf Acrokeratosis Verruciformis
  • Hopf Diseases
  • Verruciformis, Acrokeratosis

MeSH Record

Aspects Covered

34 allowable subheadings

Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.

MeSH Record

History Note

2009 (1966)

MeSH Hierarchy

Tree Numbers

  1. C16w.320w.850w.190w
  2. C17.800w.428w.275w
  3. C17.800w.827w.190w

AMA Style

References

  1. National Library of Medicine. Darier Disease. Medical Subject Headings (MeSH). 2026. Unique ID D007644. http://id.nlm.nih.gov/mesh/2026/D007644
  2. Darier Disease. In: Wikipedia. https://en.wikipedia.org/wiki/Darier%27s_disease
  3. Darier Disease. In: Wikidata. https://www.wikidata.org/wiki/Q580506