You use Therapeutics in Dermatology on a regular basis and you are pleased with it ?
Then help us improve it by

make a donation now!



MENU
Therapeutics in Dermatology
A reference textbook in dermatology

Darier disease

11 June 2012, by El FEKIH N. & KHARFI M. & KAMOUN M.R.

1 - BACKGROUND

Darier disease is a rare genodermatosis caused by abnormal adhesion between keratinocytes. It is transmitted in an autosomal dominant pattern, with almost total penetrance and variable expressivity. The disease is caused by a mutation in the ATP2A2 gene located on chromosome 12 q23-24.1 [1, 2, 3] that disrupts the regulation of intracellular calcium levels and affects the intercellular adhesion system and/or the organisation of the cytoskeleton. This abnormal keratinocyte adhesion is thought to be connected to abnormalities of the dendritic cells, which are involved in local immune response [4].

× N.B. : This limited content is for the general public. If you are a health professional, click here to register for free and gain access to a dedicated deeper content.
If you already have an account, log in!

Follow us

Newsletter

  Health professionals