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To the Editor: This case report discusses a rare complication of Darier’s disease and emphasizes the importance of obtaining a thorough medical history, including a family medical history, and a physical examination to rule out medical causes of patient complaints.
Mr. A was a 22-year-old man in basic training with the military. On the 12th day of training, he was found waking other soldiers in their bunks while on guard duty. He was unresponsive to commands, talking only in riddles and puns. The results of a mental status examination were significant for a blunted affect and poverty of thought. There was no evidence of a sleep disturbance or a flight of ideas. Mr. A was admitted to the hospital to rule out a psychotic disorder.
Mr. A"s medical history revealed that he had sought medical care 3 days before for a rash. There was no personal or family psychiatric history. He was outgoing and socially involved, displayed no occupational difficulties, and maintained close friendships until his admission.
The results of Mr. A’s physical examination were notable for a maculopapular, erythematous, crusting rash over his upper body and feet. There were no vesicles, pustules, or target lesions. His nails were thickened and had V-shaped scalloping. His drill sergeant revealed that the marines had recently exercised without shirts and rested in the sun with their boots off. Mr. A’s father told us of his own history of Darier’s disease, which is exacerbated by exposure to the sun. A skin biopsy of Mr. A’s lesions revealed abnormal keratinization and loss of epidermal adhesion with acantholysis, which was consistent with keratosis follicularis, or Darier’s disease.
Mr. A was removed from exposure to the sun and treated with topical isotretinoin. On his fourth day in the hospital, 4 mg/day of risperidone was added to help his disorganized thought process. His symptoms faded by the 12th day of admission, and he displayed no further psychotic symptoms. He was discharged with a diagnosis of psychotic disorder secondary to Darier’s disease.
Darier’s disease is an autosomal dominant disorder characterized by altered keratinization of the epidermis, nails, and mucous membranes. It was first discovered in an American patient who developed the lesions under a knapsack while training for the U.S. Army in 1862. It usually begins in the first or second decade of life and is found in equivalent numbers among men and women. It is a rare condition, affecting one person in 100,000. Darier’s disease is frequently worse in the summer, when it is exacerbated by heat, humidity, ultraviolet B light, and mechanical trauma. Affective disorders, mainly bipolar disorders, that appeared as a result of Darier’s disease have been reported recently, but they are considered rare complications. The association between the two diseases has been postulated to result from a defect in cell adhesion expressed in both the skin and the brain, which share a common ectodermal origin (1). Treatment is supportive, with removal from and education about sun exposure. Topical retinoids are used to treat the rash; oral retinoids are reserved to treat more severe forms of the disorder.
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