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Darier disease is a rare type of autosomal dominant genodermatosis, and it is caused by a mutation in the gene coding for the endoplasmic reticulum membrane calcium pump Ca<sup>2+</sup>-ATPase type 2, leading to compromised intercellular adhesion. Moreover, this condition is characterized by multiple keratotic greasy papules with a seborrheic distribution and is worsened by heat and sun exposure, sweating, and friction. Occasionally, it may be associated with nail abnormalities and may involve the mucosa. Unilateral segmental Darier disease is a rare variant characterized by unilateral eruption of erythematic keratotic papules not associated with other conditions. Herein, we report a case of type 1 segmental Darier disease. Furthermore, the main characteristics and treatment options are discussed.

Type 1 Segmental Darier Disease: Case Report and Discussion of the Treatment Options