What is it about?
Darier's disease (DD) is an autosomal dominant disorder that is characterized clinically by multiple keratotic papules involving mainly the seborrheic areas and histologically by acantholysis and dyskeratosis. In contrast, segmental DD is characterized by localization of keratotic papules in a linear or zosteriform pattern following the Lines of Blaschko More Details in a unilateral fashion.
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Why is it important?
Localized DD is considered a genetic mosaic of generalized DD resulting from a post-zygotic somatic mutation in early embryogenesis. The zosteriform pattern described in localized DD would actually exhibit a Blaschkoid rather than a dermatomal distribution.
Perspectives
Type 1 segmental DD, characterized by the occurrence of both zosteriform and linear patterns involving multiple segments is an extremely rare presentation and hence, is highlighted.
Dr Arunprasath Palanisamy
Read the Original
This page is a summary of: Type one segmental Darier′s disease, Indian Journal of Dermatopathology and Diagnostic Dermatology, January 2015, Medknow,
DOI: 10.4103/2349-6029.173414.
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