Darier-White Disease Research Articles

Darier-White disease: A review of the clinical features in 163 patients

Darier's disease is a rare, dominantly inherited genodermatosis; there have not been any large clinical studies of patients with this disease. Our purpose was to document the clinical features in a large group of patients with Darier's disease. Data were collected from 163 affected persons. The onset usually occurred between the ages of 6 and 20 years. The disease has a predilection for the skin in seborrheic areas; 96% had acral signs; 6% had hypertrophic flexural involvement; and 13% had oral mucosal lesions. There was no remission. Topical therapy sometimes provided relief of symptoms but had no effect on the progress of the disease. Oral retinoids were effective, but long-term therapy was tolerated poorly. Most patients did not have other medical problems. Although Darier's disease is a chronic and unremitting burden, most patients manage to lead a relatively normal life.

Therapeutic implications of an intraoral malignancy complicated by Darier-White's disease

A 69-year-old white male with a long history of severe Darier-White's disease presented with an extensive intraoral malignancy. High dose external supervoltage radiotherapy (Cobalt-60) to the tumour caused remarkable clearing of the hyperkeratotic lesions involving the overlying skin. Subsequently, surgical resection of the tumour with staged reconstruction of the jaw was carried out. The presence of the skin disorder did not adversely affect the surgical procedure nor the subsequent wound healing. Darier-White's disease, therefore, should not be a deterrent to radical radiotherapy and/or surgery for concomitant malignancy.

Commentary: Darier-White disease

In 1871, Harvard became the first American university to establish an independent chair of dermatology and James C. White, MD, was the first to occupy it. At that time, dermatology was becoming recognized as a specialty in this country and American dermatology had declared its independence from the European schools. In 1889, White¹described a case of keratosis (ichthyosis) follicularis, while, independently, Professor Ferdinand Jean Darier, MD, published a similar case in Paris, which he called "psorospermose folliculaire végétante."²From then on, keratosis follicularis, psorospermose folliculaire végétante, Darier-White disease, or Darier's disease became an accepted entity. The point-by-point, meticulous clinical description of the disorder by White was accurate; however, in this first account, he failed to describe dyskeratotic cells, the histopathologic hallmark of this disorder. He erroneously postulated that the hair follicle was the primary site of involvement. Darier first described the dyskeratotic regions in the epidermis, but

Commentary: Darier-White Disease

In 1871, Harvard became the first American university to establish an independent chair of dermatology and James C. White, MD, was the first to occupy it. At that time, dermatology was becoming recognized as a specialty in this country and American dermatology had declared its independence from the European schools. In 1889, White 1 described a case of keratosis (ichthyosis) follicularis, while, independently, Professor Ferdinand Jean Darier, MD, published a similar case in Paris, which he called folliculaire vegetante. 2 From then on, keratosis follicularis, psorospermose folliculaire vegetante, Darier-White disease, or Darier's disease became an accepted entity. The point-by-point, meticulous clinical description of the disorder by White was accurate; however, in this first account, he failed to describe dyskeratotic cells, the histopathologic hallmark of this disorder. He erroneously postulated that the hair follicle was the primary site of involvement. Darier first described the dyskeratotic regions in the epidermis, but

The Nail in Darier-White Disease

Longitudinal, subungual, red or white streaks, or both, associated with distal wedge-shaped subungual keratoses are the nail signs diagnostic for Darier-White disease. Histopathologic changes typical of Darier-White disease, with suprabasilar clefts containing acantholytic cells, are present in the dorsal portion of the proximal nail fold, the nail matrix, and the epidermis of the volar skin. In addition, multinucleate epithelial giant cells can be seen in the cornified cells of the nail bed and the nail plate. The cornified layer is thickened from the proximal nail fold to the volar skin. Keratotic papules on the dorsal portion of the nail fold clinically may resemble acrokeratosis verruciformis but histologically have features of Darier-White disease. Rarely, Darier-White disease can be limited to the nails.

The nail in Darier-White disease

The nail in Darier-White disease

Darier White disease

Darier’s disease was described inependently by Darier and White in 1989 AD. Reported prevalence varies from 1/100,000 in Denmark to 1 in 30- 35,000 in northern England and Scotland.1 Darier’s disease has been reported from other parts of the world, however,there has been no reported cases in Nepal. It is an inherited autosomal dominat disease characterized by dark crusty lesions over the seborrheic areas of the skin. We are reporting a case of Darier’s disease in a Nepalese patient who developed skin leison’s over his face and body at the age of 12 years. We took skin punch biopsy from the leisons, which showed findings suggestive of Darier’s disease. Our case was treated with isotrerenoin and topical keratolytics. He responded with the given treatment within two weeks of therapy. DOI: http://dx.doi.org/10.3126/njdvl.v9i1.5770 NJDVL 2010; 9(1): 34-36

Darier-White's disease: A cause of white lesions of the mucosa: Report of four cases

The general aspects of Darier-White's disease have been discussed, with a detailed description of the associated mucosal lesions. We have presented reports of four patients with generalized skin involvements, three of whom also had oral lesions. We have also described certain unusual findings in association with the dental tissues, involving hyperplastic and metaplastic changes seen in the rests of Malassez, which probably resulted from Darier-White's disease. No equivalent epithelial changes, however, were observed in an apical periodontal cyst associated with a grossly carious tooth which was examined.

Four hereditary mucosal syndromes: comparative histology and exfoliative cytology of Darier-White's disease, hereditary benign intraepithelial dyskeratosis, white sponge nevus, and pachyonychia congenita.

<h3>Introduction</h3> White lesions of the oral mucosa, clinically resembling leukoplakia, have been encountered in a variety of conditions. Among these diseases are 4 rare hereditary dyskeratoses: Darier-White's disease,^1,2hereditary benign intraepithelial dyskeratosis,³white sponge nevus of Cannon,⁴and pachyonychia congenita of Jadassohn and Lewandowsky.⁵In contrast to leukoplakia, which is characterized by premalignant dyskeratosis, these latter conditions run a chronic but benign course. Leukoplakia is usually localized to one site, while all 4 of the hereditary diseases may show involvement of other structures. A comparative study of tissue sections and exfoliative cytology of the oral lesions of these conditions was undertaken to investigate the cellular abnormalities revealed by this technique. To our knowledge only the exfoliative cytology pattern of hereditary benign intraepithelial dyskeratosis has been reported previously.³ <h3>Methods and Materials</h3> Patient material was obtained from families studied by the Genetics Section of the National Institute