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Darier's disease (DD) is a rare, genetic skin disorder. It is an autosomal dominant disorder, that is, if one parent has DD, there is a 50% chance than a child will inherit DD. It was first reported by French dermatologist Ferdinand-Jean Darier in 1889.
Darier's sign is a change observed after stroking lesions on the skin of a person with systemic mastocytosis or urticaria pigmentosa. [1] In general, the skin becomes swollen, itchy and red. This is a result of compression of mast cells, which are hyperactive in these diseases. These mast cells release inflammatory granules which contain ...
Darier's disease (Darier–White disease, dyskeratosis follicularis, keratosis follicularis) Linear Darier disease; DeSanctis–Cacchione syndrome; Disseminated superficial actinic porokeratosis; Disseminated superficial porokeratosis; Dolichol kinase deficiency; Dominant dystrophic epidermolysis bullosa; Dyskeratosis congenita (Zinsser–Cole ...
Polydactylous longitudinal erythronychia has been most commonly associated with Darier's disease [10] and lichen planus [11] but has also occasionally been associated with acantholytic epidermolysis bullosa, [12] no association, [13] graft-versus-host disease, [14] [15] hemiplegia, [16] and systemic amyloidosis. [11]
Onychauxis presents with thickened nails without deformity, and this simple thickening may be the result of trauma, acromegaly, Darier's disease, psoriasis, or pityriasis rubra pilaris, or, in some cases, hereditary. [1]: 783 [2] It may appear as loss of nail palate translucency, discoloration, and subungual hyperkeratosis.
Dandy–Walker malformation with mental retardation, basal ganglia disease, and seizures; Dandy–Walker malformation with mental retardation, macrocephaly, myopia, and brachytelephalangy; Dandy–Walker syndrome; Daneman–Davy–Mancer syndrome; Darier's disease; Davenport–Donlan syndrome; DAVID syndrome; Davis–Lafer syndrome
Toxicodendron dermatitis lesions are often linear from brushing up against the plant. Causes of the Koebner phenomenon that are secondary to scratching rather than an infective or chemical cause include vitiligo, psoriasis, lichen planus, lichen nitidus, pityriasis rubra pilaris, and keratosis follicularis (Darier disease). [citation needed]
Whether acrokeratosis verruciformis and Darier disease are related or distinct entities has been controversial, like Darier's disease, it is associated with defects in the ATP2A2 gene. [4] however the specific mutations found in the ATP2A2 gene in acrokeratosis verruciformis have never been found in Darier's disease. [5]