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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.
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]
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
[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]
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
[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]
The Koebner phenomenon or Köbner phenomenon (UK: / ˈ k ɜː b n ər /, US: / ˈ k ɛ b-/), [1] also called the Koebner response or the isomorphic response, attributed to Heinrich Köbner, is the appearance of skin lesions on lines of trauma. [2] The Koebner phenomenon may result from either a linear exposure or irritation.