HONG KONG JOURNAL OF DERMATOLOGY & VENEREOLOGY, cilt.23, ss.71-75, 2015 (SCI İndekslerine Giren Dergi)
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.