We describe the case of a 71-year-old men suffering from Darier's disease since his youth and presenting with intensely itching, vesicular and pustular lesions, localized on his arms, legs and on the ulnar side of his right hand. The patient also had a 30 year-history of bipolar psychiatric disorders. The histopathological examination of lesional skin showed suprabasal acantholytic clefting, hyperparakeratosis, and dyskeratosis. Histopathological and immunopathological (direct and indirect immunofluorescence) results, were consistent with vesiculo-bullous Darier's disease. Corticosteroid therapy with methylprednisolone at low dosage (0.2 mg/kg/day) was started with a quick resolution of the vesicular lesions on his arms and legs.