Darier Disease

Overview

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Is a:

Disease or Syndrome

Definition:

An autosomal dominantly inherited skin disorder characterized by warty malodorous papules that coalesce into plaques. It is caused by mutations in the atp2a2 gene encoding serca2 protein, one of the sarcoplasmic reticulum calcium-transporting atpases. The condition is similar, clinically and histologically, to benign familial pemphigus, another autosomal dominant skin disorder. Both diseases have defective calcium pumps (calcium-transporting atpases) and unstable desmosomal adhesion junctions (desmosomes) between keratinocytes. An alleleic variant of Darier's disease.

Synonyms:

Acantholytic Dyskeratotic Epidermal Nevi
Acantholytic Dyskeratotic Epidermal Nevus
Acrokeratosis Verruciformis
Acrokeratosis Verruciformis of Hopf
Darier White Disease
Darier's Disease
Darier-White Disease
Darier-White Diseases
Dariers Disease
Disease, Darier
Disease, Darier's
Disease, Darier-White
Disease, Hopf
Diseases, Darier-White
Diseases, Hopf
Hopf Acrokeratosis Verruciformis
Hopf Disease
Hopf Diseases
Keratosis Follicularis
Verruciformis, Acrokeratosis

Broader Topics:

Narrower Topics:

None

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