Uncommon Flexural Presentation of Darier Disease

Abstract

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. Methods and 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. Conclusion: 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.