Debilitating Darier's disease

Title

Debilitating Darier's disease

Creator

Brodell D W; Frisch L E; Brodell R T

Publisher

Signa Vitae

Date

2007
2007-04

Description

Darier's disease is an inherited dermatitis that is due to a mutation in the ATP2A2 gene. This mutation causes disrupted signaling within cells and leads to a skin condition that is resistant to many dermatologic treatments. Though serious complications are rare, Darier's disease can result in bacterial sepsis and Kaposi's varicelliform eruption. In some families seizure disorders are linked to Darier's disease. There is also a paraneoplastic variant that could be considered in patients without a family history of Darier's disease. This article reports a case with typical verrucous masses and waxy papules which, over twenty years, became disabling. Symptoms included pruritus and foul odor and were relieved, along with the keratotic rash, utilizing systemic treatment with acitretin though the condition recurred rapidly when treatment was interrupted. Darier's disease must be considered when patients present with hyperkeratotic lesions that are resistant to usual forms of therapy for inflammatory dermatoses.

Subject

Emergency Medicine; acetretin; Darier's Disease; inherited dermatoses

Identifier

n/a

Format

Journal Article or Conference Abstract Publication

URL Address

n/a

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Rights

Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).

Pages

33-34

Issue

1

Volume

2

Citation

Brodell D W; Frisch L E; Brodell R T, “Debilitating Darier's disease,” NEOMED Bibliography Database, accessed June 22, 2021, https://neomed.omeka.net/items/show/8730.

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