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Localized Darier disease: three cases of Type 1 segmental mosaicism
D Phillips1, K Gumparthy2, C W Farrar1
1Department of Dermatology, Clatterbridge Hospital, Wirral University Teaching Hospital NHS Foundation Trust, Wirral, Merseyside, UK.
Clinical and Experimental Dermatology
|August 4, 2021
Summary
Darier disease (DD) is a rare genetic skin condition. This study presents three cases of localized Darier disease, a less common variant of this dermatosis.
Area of Science:
- Dermatology
- Genetics
- Rare Diseases
Background:
- Darier disease (DD) is an autosomal dominant acantholytic dermatosis.
- Prevalence ranges from 1 in 30,000 to 100,000.
- Localized Darier disease accounts for approximately 10% of all cases.
Observation:
- Clinical variants include unilateral, linear, segmental, or zosteriform presentations.
- Presents a case series of three patients diagnosed with localized Darier disease.
- Highlights the clinical diversity within localized Darier disease presentations.
Findings:
- The case series provides clinical insights into localized Darier disease.
- Demonstrates the varied manifestations of this rare dermatosis.
- Contributes to the understanding of localized Darier disease phenotypes.
Implications:
- Enhances the recognition of localized Darier disease variants.
- Aids in the differential diagnosis of rare skin conditions.
- Informs clinical management strategies for patients with Darier disease.
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