Penicillamine for the Treatment of Darier's Disease and Other Disorders of Keratin Formation

Beer, W.E.; Lyle, W. H.

doi:10.1016/s0140-6736(66)92084-8

Cited by 10 publications

(6 citation statements)

References 7 publications

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“… 52 In a case series of six patients receiving this medication, three patients experienced moderate to significant improvement of their DD, while three patients experienced only minimal or negligible improvement of their DD. 52 Urticaria and eczema was also reported as a side effect of this treatment regimen in three of the six patients. 52 Relapse of DD was also reported in every patient after discontinuation of penicillamine, suggesting that a long-term regimen or multiple treatment courses may be required.…”

Section: Resultsmentioning

confidence: 96%

“…52 Urticaria and eczema was also reported as a side effect of this treatment regimen in three of the six patients. 52 Relapse of DD was also reported in every patient after discontinuation of penicillamine, suggesting that a long-term regimen or multiple treatment courses may be required. 52 Systemic Antibiotics.…”

Section: Vitamin a Analogs A Variety Of Other Systemic Vitaminmentioning

confidence: 84%

“…The proposed mechanism of action of penicillamine involves its role in chelating copper used in the disulphide bonds of keratin, thereby decreasing keratinocyte proliferation. 52 There was some evidence to support a trial of penicillamine as a treatment for DD, although it was poor and inconsistent (Grade C). 52 In a case series of six patients receiving this medication, three patients experienced moderate to significant improvement of their DD, while three patients experienced only minimal or negligible improvement of their DD.…”

Section: Resultsmentioning

confidence: 99%

“… 52 There was some evidence to support a trial of penicillamine as a treatment for DD, although it was poor and inconsistent (Grade C). 52 In a case series of six patients receiving this medication, three patients experienced moderate to significant improvement of their DD, while three patients experienced only minimal or negligible improvement of their DD. 52 Urticaria and eczema was also reported as a side effect of this treatment regimen in three of the six patients.…”

Section: Resultsmentioning

confidence: 99%

“…52 Relapse of DD was also reported in every patient after discontinuation of penicillamine, suggesting that a long-term regimen or multiple treatment courses may be required. 52 Systemic Antibiotics. Although antibiotics are commonly used by patients with DD to treat superimposed cutaneous infections, we identified multiple case reports which investigated the use of doxycycline for the clearance of DD (Grade C).…”

Section: Vitamin a Analogs A Variety Of Other Systemic Vitaminmentioning

confidence: 99%

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Therapeutic Options for the Treatment of Darier’s Disease: A Comprehensive Review of the Literature

et al. 2021

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Darier’s disease (also known as keratosis follicularis or dyskeratosis follicularis) is an autosomal dominant inherited disorder which manifests as hyperkeratotic greasy papules in the first or second decade of life. Aside from symptom management and behavioral modifications to avoid triggers, there are currently no validated treatments for Darier’s disease (DD). However, a variety of treatments have been proposed in the literature including retinoids, steroids, vitamin D analogs, photodynamic therapy, and surgical excision. The purpose of this review article is to identify therapeutic options for treating DD and to outline the evidence underlying these interventions. A search was conducted in Medline for English language articles from inception to July 4, 2020. Our search identified a total of 474 nonduplicate studies, which were screened by title and abstract. Of these, 155 full text articles were screened against inclusion/exclusion criteria, and 113 studies were included in our review. We identified Grade B evidence for the following treatments of DD: oral acitretin, oral isotretinoin, systemic Vitamin A, topical tretinoin, topical isotretinoin, topical adapalene gel, topical 5-flououracil, topical calciptriol and tacalcitol (with sunscreen), grenz ray radiation, and x-ray radiation. All other evidence for treatments of DD consisted of case reports or case series, which is considered grade C evidence. Considering the quality and quantity of evidence, clinicians may consider initiating a trial of select topical or oral retinoids first in patients with localized or generalized DD, respectively.

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Section: Resultsmentioning

confidence: 96%