Uncommon flexural presentation of Darier disease.
BACKGROUND
Darier disease is an autosomal dominant skin disorder characterized by keratotic papules, focal loss of adhesion, and abnormal keratinization. Several clinical variants of Darier disease have been described. Predominant flexural distribution of lesions in Darier disease is uncommon.


OBJECTIVE
We report a sporadic case of Darier disease in which the lesions were strictly localized to the flexures. We also briefly review the differential diagnoses of such atypical localization.


RESULTS
A 26-year-old female presented with erythematous and hyperpigmented papular lesions localized to flexures for 4 months. The skin of the other areas, hairs, nails, and mucosa was normal. Histopathologic examination revealed suprabasal acantholysis along with dyskeratotic keratinocytes, consistent with Darier disease.


CONCLUSIONS
Physicians should be aware of such atypical localizations and should consider the diagnosis of flexural Darier disease along with more common clinical conditions with flexural localization, such as Hailey-Hailey disease and occasionally Grover disease.
Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Dipankar
D
De
Amrinder J
AJ
Kanwar
Uma Nahar
UN
Saikia
D000328:Adult; D007644:Darier Disease / Q000175:diagnosis / Q000473:pathology*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans