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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
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]
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.
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The diagnosis of FDCWD is made clinically. There is no specific laboratory test or biopsy finding that is diagnostic of the disorder. The differential diagnosis includes other disorders that can cause comedones, such as acne vulgaris, familial dyskeratotic comedones, and Darier-White disease. There is no cure for FDCWD.
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