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Darier disease: Histopathology revisited
Archana Manohar1, Meryl Antony2, Rajalakshmi Tirumalae1
1Department of Pathology, St. John's Medical College, Bengaluru, Karnataka, India.
Indian Journal of Pathology & Microbiology
|April 2, 2024
Summary
Darier disease (DD) histopathology shows classic features and unusual variations like cornoid lamella-like patterns. Pathologists need to recognize these diverse presentations for accurate diagnosis.
Area of Science:
- Dermatopathology
- Genodermatoses
Background:
- Darier disease (DD) is a rare genodermatosis.
- Existing literature primarily consists of case reports with limited histopathological detail.
Purpose of the Study:
- To comprehensively document the histopathological features of Darier disease.
- To identify and characterize variations in DD histopathology beyond classic findings.
Main Methods:
- Retrospective review of skin biopsies diagnosed as Darier disease over 12 years.
- Analysis of epidermal and dermal features in 16 Darier disease patients.
Main Results:
- Classic suprabasal acantholytic clefts were observed.
- Unusual features included absence of parakeratosis (19%), cornoid lamella-like pattern (62%), follicular acantholysis (13%), and multiple foci (63%).
- Dyskeratotic cells and minimal dermal infiltrates were consistent with prior reports.
Conclusions:
- Pathologists should be aware of Darier disease histopathological variations, especially with atypical clinical presentations.
- The Darier-like pattern can occur in other acantholytic diseases, emphasizing the need for clinicopathologic correlation.
