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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.
[5] He married Martha Anna Ellis in 1862; they had three sons. [5] His son Charles J. White took over his medical practice in 1914, [1] and became Edivard Wigylesicorth Professor of Dermatology at Harvard and chair of the Harvard dermatology department. His grandson, James Clarke White, was also a professor at Harvard Medical School. [6]
The other diseases were a follicular keratosis (Darier-White syndrome), dermatofibrosarcoma (Darier-Ferrand disease), erythema annularis, subcutaneous sarcoidosis (Darier-Roussy sarcoid), and a sign, Darier's sign observed in mastocytosis. From 1909 to 1922, Darier was head of the clinical department at the Hôpital Saint-Louis. [5]
[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 (deep gyrate erythema) and erythema figuratum ...
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 ...
Clinical signs of acrokeratosis include verrucous plaques and flat-topped, polygonal papules. [6] The lesions range in hue from brown to skin tone, and their friction might cause vesicles. [ 4 ] [ 7 ] The backs of the hands and feet's proximal and distal interphalangeal joints are typically where the lesions are seen. [ 8 ]
[6] Although classically associated with psoriasis, subsequent research has found Auspitz's sign to be of very little diagnostic value for the disease. This is because several other diseases display the sign (including Darier's disease and actinic keratosis). Additionally, only a minority of psoriasis scales show it when removed (<18%). [7]
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