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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.
White is one of the namesakes of Darier–White disease, having discovered it independently of Ferdinand-Jean Darier. [1] [2] He also wrote a book, Dermatitis Venenata, published in 1887. [3] White's Back Bay residence, designed by Peabody & Stearns
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]
[2] [3] However sporadic forms, whose less than 10 cases have been reported, presents at a later age, usually after the first decade and generally lack palmoplantar keratoses. Whether acrokeratosis verruciformis and Darier disease are related or distinct entities has been controversial, like Darier's disease , it is associated with defects in ...
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 ...
[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]