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Darier's sign is a change observed after stroking the skin of a person with systemic mastocytosis or urticaria pigmentosa.In general, the skin becomes swollen, itchy and red.arier's sign is a change observed after stroking the skin of a person with systemic mastocytosis or urticaria pigmentosa. In general, the skin becomes swollen, itchy and red.
Darier's disease (DAR), also known as Darier disease, DarierâWhite disease, Dyskeratosis follicularis and Keratosis follicularis, is an autosomal dominant disorder discovered by French dermatologist Ferdinand-Jean Darier.Darier's is characterized by dark crusty patches on the skin, sometimes containing pus.he symptoms of the disease are thought to be caused by an abnormality in the desmosome-keratin filament complex leading to a breakdown in cell adhesion. Worldwide prevalence is estimated as between 1: 30,000 and 1: 100,000.