Vitamin a in Darier's Disease

Porter, A. D.; Godding, E. W.; Brünauer, St. R.

doi:10.1001/archderm.1947.01520090026003

Cited by 31 publications

(2 citation statements)

References 18 publications

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“…Vitamin A therapy has previously been the only therapy of help in Darier's disease (Porter et al, 1947, Leitner & Moore, 1948, and other disorders of keratinization. The use of very high doses of vitamin A although sometimes helpful, often produced significant side-effects.…”

Section: Discussionmentioning

confidence: 99%

13-cis-retinoic acid for dyskeratinizing diseases-clinicopathological responses

Risch¹,

Ashton²,

Lowe³

et al. 1984

Clin Exp Dermatol

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Summary Fifteen patients with Darier's disease (five patients), epidermolytic hyperkeratosis (five patients), and pityriasis rubra pilaris (five patients) were treated with doses ranging from 1.0 to 3.0 mg per kg body weight of 13 ‐cis retinoic acid. There was marked clinical improvement in the patients with Darier's disease which was sustained in two out of five on stopping treatment after 6 months. Three patients with pityriasis rubra pilaris went into remission, and required no further treatment on stopping 13‐cis retinoic acid after 6 months; however two patients did not respond at all. All five patients with epidermolytic hyperkeratosis responded, but relapsed rapidly on discontinuation of therapy. The major histological change noted was the reduction in stratum corneum organisation and cohesion, with reduction in stratum corneum layers. Serum cholesterol and triglycerides became raised after 1 month's therapy, but tended to fall during the following 5 months of therapy. High density lipoproteins remained depressed during treatment. In selected cases, 13‐cis retinoic acid is a useful therapy for Darier's disease, pityriasis rubra pilaris and epidermolytic hyperkeratosis.

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Section: Discussionmentioning

confidence: 99%

13-cis-retinoic acid for dyskeratinizing diseases-clinicopathological responses

Risch¹,

Ashton²,

Lowe³

et al. 1984

Clin Exp Dermatol

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“…Vitamin A therapy has been described in several skin diseases e.g. Darier's Disease (Porter et al, 1947), pityriasis rubra pilaris (Leitner and Ford, 1947), ichthyosis (Rapaport et al, 1942;Peck d al., 1043) and even acne vulgaris (Straumford, 1943: Lynch andCook, 1947). Reports of success have been contradictory and other signs of vitamin A deficiency have been present in some cases and not ill others.…”

Section: Discussionmentioning

confidence: 99%

The Use of Retinoic Acid in Congenital Ichthyosiform Erythroderma *

Thomson

Milne

1969

Br J Dermatol

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Darier's disease and vitamin A. Concentrations of retinoids in serum and epidermis of untreated patients

Vahlquist¹

1982

Archives of Dermatology

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Blood samples and skin biopsy specimens from 13 male patients with Darier s disease (DD) and 11 healthy male controls were analyzed for carotene, retinol (vitamin A), dehydroretinol (vitamin A2), and retinol-binding protein (RBP). The serum carotene level was lower in the patients than in the controls, but the vitamin A and RBP levels did not differ from those of the controls regardless of the severity of the disease. The RBP could be visualized by immunofluorescence microscopy in the epidermis of both unaffected and lesional skin of patients with DD. In the epidermis of patients with DD (uninvolved and lesional), the carotene content was similar to that of the controls. The retinol content was probably higher than in the controls. By contrast, the dehydroretinol concentration was normal in the uninvolved skin but markedly higher than normal in the lesions, especially in the severe cases. The abnormal distribution of retinoids in DD may reflect localized disturbances of epidermal vitamin A metabolism rather than a defect in serum vitamin A transport.

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Vitamin a in Darier's Disease

Cited by 31 publications

References 18 publications

13-cis-retinoic acid for dyskeratinizing diseases-clinicopathological responses

13-cis-retinoic acid for dyskeratinizing diseases-clinicopathological responses

The Use of Retinoic Acid in Congenital Ichthyosiform Erythroderma *

Darier's disease and vitamin A. Concentrations of retinoids in serum and epidermis of untreated patients

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