Type 1 segmental Darier's disease: report of two cases with favourable response to topical retinoids


Bakar O. , Durmaz E. , Sezer E., Cetin E. D.

HONG KONG JOURNAL OF DERMATOLOGY & VENEREOLOGY, vol.23, no.2, pp.71-75, 2015 (Journal Indexed in SCI) identifier identifier

  • Publication Type: Article / Article
  • Volume: 23 Issue: 2
  • Publication Date: 2015
  • Title of Journal : HONG KONG JOURNAL OF DERMATOLOGY & VENEREOLOGY
  • Page Numbers: pp.71-75

Abstract

Darier's disease is a rare late-onset genetic disorder of keratinisation. Mosaic forms of the disease characterised by localised and unilateral keratotic papules carrying post-zygotic ATP2A2 mutation in affected areas have been documented. Segmental forms of Darier's disease are classified into two clinical subtypes: type 1 manifesting with distinct lesions on a background of normal appearing skin and type 2 with well-defined areas of Darier's disease occurring on a background of less severe non-mosaic phenotype. Herein we describe two cases of type 1 segmental Darier's disease with favourable response to topical retinoids.