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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]
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]
[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 ...
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]
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]
[5] [6] [7] The epidermis is the most superficial layer of skin, a squamous epithelium with several strata: the stratum corneum, stratum lucidum, stratum granulosum, stratum spinosum, and stratum basale. [8] Nourishment is provided to these layers by diffusion from the dermis since the epidermis is without direct blood supply.
[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]