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Uncommon flexural presentation of Darier disease
Dipankar De1, Amrinder J Kanwar, Uma Nahar Saikia
1Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Journal of Cutaneous Medicine and Surgery
|October 11, 2008
Summary
This study details a rare case of Darier disease presenting exclusively in skin flexures. Awareness of this atypical presentation is crucial for accurate diagnosis, distinguishing it from similar conditions.
Area of Science:
- Dermatology
- Genetics
- Pathology
Background:
- Darier disease is an autosomal dominant genodermatosis.
- It typically presents with keratotic papules and abnormal keratinization.
- Flexural Darier disease is an uncommon clinical variant.
Observation:
- A 26-year-old female presented with a 4-month history of lesions.
- Lesions were erythematous and hyperpigmented, strictly localized to flexural areas.
- Other cutaneous structures, hair, nails, and mucosa were unaffected.
Findings:
- Histopathology confirmed suprabasal acantholysis and dyskeratotic keratinocytes.
- These findings are characteristic of Darier disease.
- The case highlights an atypical, flexural-predominant presentation.
Implications:
- Clinicians should consider Darier disease in cases of unexplained flexural dermatoses.
- Differential diagnoses include Hailey-Hailey disease and Grover disease.
- Recognizing atypical Darier disease localization improves diagnostic accuracy.
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