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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.
A form of Darier disease known as the linear or segmental form is characterized by blemishes on localized areas of the skin. The blemishes are not as widespread as they are in typical Darier disease.he linear form of Darier disease is caused by ATP2A2 gene mutations that are acquired during a person's lifetime and are present only in certain cells.