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Acantholytic dyskeratotic epidermal naevus.

I Nirmala¹, M Jayaraman, V R Janaki

¹Department of Dermatology, Madars Medical College and Government General Hospital, Madras, India.

Indian Journal of Dermatology, Venereology and Leprology

|October 15, 2010

Summary

This summary is machine-generated.

This case report describes a rare mosaic form of Darier's disease presenting as unilateral warty papules and whitish macules. The patient showed no typical family history or clinical signs, highlighting diagnostic challenges.

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Area of Science:

Dermatology
Genetics
Histopathology

Background:

Darier's disease is a rare autosomal dominant genodermatosis.
It typically presents with characteristic skin lesions, nail changes, and palmoplantar abnormalities.
Atypical or segmental presentations can occur, posing diagnostic challenges.

Purpose of the Study:

To report a unique case of Darier's disease.
To highlight the presentation of a mosaic form of Darier's disease.
To discuss the histopathological findings and diagnostic considerations.

Main Methods:

Clinical presentation and patient history.
Dermatoscopic examination.
Histopathological analysis of skin biopsy specimens.

Main Results:

A 43-year-old male presented with a 20-year history of unilateral warty papular eruptions and whitish macules on the trunk and limbs.
Histopathology confirmed features consistent with Darier's disease.
No family history, palmar pits, or nail changes typical of Darier's disease were observed.

Conclusions:

The case represents an acantholytic dyskeratotic epidermal nevus, likely a mosaic form of Darier's disease.
This presentation underscores the importance of histopathology in diagnosing atypical Darier's disease.
Mosaicism should be considered in unilateral presentations of genodermatoses.