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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 ...
The disease is most often diagnosed as an infant, when parents take their baby in for what appears to be bug bites. The bug bites are actually the clumps of mast cells. Doctors can confirm the presence of mast cells by rubbing the baby's skin. If hives appear, it most likely signifies the presence of urticaria pigmentosa. [citation needed]
Acantholytic dyskeratotic epidermal nevus is a cutaneous condition identical to the generalized form of Darier's disease. [1]: 849 "Acantholytic dyskeratotic epidermal nevus" is probably the same disorder. [1]: 849
It was first described by Darier in 1916. [ 3 ] [ 4 ] [ 5 ] Many different terms have been used to classify these types of lesions and it is still controversial on what exactly defines EAC. Some of the types include annular erythema (deep and superficial), erythema perstans, erythema gyratum perstans, erythema gyratum repens, darier erythema ...
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]
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]
Ong was subsequently diagnosed with Darier's disease, a rare skin disorder that could cause disfiguring and red rashes on the skin, and it was also a hereditary disease that could be passed on from parent to child; Ong was suffering from this particular skin condition since age 14, and the disease itself was previously unspecified during the ...