[en] A 67-year-old man is presented with longstanding and severe Darier disease treated by topical antiseptics and potent corticosteroids, in combination with oral glucocorticoids and etretinate. After cardiac bypass surgery in 1997, the patient experienced herpes simplex virus (HSV type-1) infection of the skin that was treated by intravenous aciclovir. In 2003, he presented a widespread atypical exacerbation of his Darier disease, involving the face, trunk, buttocks, intertriginous areas and arms. Initial clinical signs and bacteriological findings suggested a bacterial involvement by multiresistant Staphylococcus aureus, Escherichia coli, Pseudomonas aeruginosa, and Proteus mirabilis. Despite antibiotherapy, the clinical presentation progressively worsened. A skin biopsy was performed and immunohistochemical examination identified a type-2 HSV infection. Although intravenous aciclovir was administered, the widespread cutaneous HSV infection was followed by systemic dissemination. A severe acute respiratory distress syndrome (ARDS) developed, leading to a fatal issue. At autopsy, a severe interstitial type-2 HSV pneumonitis with extensive necrotic areas was found, in association with gastro-intestinal involvement. This case represents, to the best of our knowledge, the first case of Darier disease presenting a fatal type-2 HSV infection. It underlines the importance of rapidly recognizing HSV infection in Darier disease and stresses the risk of lethal outcome. The different risk factors for HSV infection in this patient are reviewed.