Darier disease: A fold (intertriginous) dermatosis

Clin Dermatol. 2015 Jul-Aug;33(4):448-51. doi: 10.1016/j.clindermatol.2015.04.009. Epub 2015 Apr 9.

Abstract

Darier disease, also known as Darier-White disease, is characterized by yellow to brown, oily keratotic papules and plaques in the seborrheic areas of the face and chest. This disorder may show different clinical manifestations, such as palmoplantar pits and nail abnormalities. The trigger factors are mechanical trauma, heat, humidity, ultraviolet B, and pyogenic infections. The disease usually becomes apparent in the second decade of life. The ATP2 A2 (SERCA2) gene mutation was detected in all patients. Histopathologic changes include epidermal adhesion loss, acantholysis, abnormal keratinization, eosinophilic dyskeratotic cells in the spinous layer known as corps ronds, and the presence of grains in the stratum corneum. Although the treatment for Darier disease is unsatisfactory, some relief has been achieved with the use of corticosteroids and retinoids.

Publication types

  • Review

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Darier Disease / drug therapy
  • Darier Disease / epidemiology*
  • Darier Disease / genetics*
  • Darier Disease / physiopathology
  • Female
  • Genetic Predisposition to Disease / epidemiology*
  • Humans
  • Incidence
  • Intertrigo / diagnosis*
  • Intertrigo / epidemiology*
  • Intertrigo / therapy
  • Male
  • Mutation
  • Prognosis
  • Retinoids / therapeutic use
  • Risk Assessment
  • Sarcoplasmic Reticulum Calcium-Transporting ATPases / genetics*
  • Treatment Outcome

Substances

  • Adrenal Cortex Hormones
  • Retinoids
  • Sarcoplasmic Reticulum Calcium-Transporting ATPases
  • ATP2A2 protein, human