Abstract
Hypomelanosis of Ito (HI), a neurocutaneous syndrome, is characterized by skin depigmentation and skeletal, muscular, central nervous system, cardiac, and renal manifestations. A wide variety of cutaneous manifestations besides depigmentation have been reported. Herein we describe a 23-year-old woman with HI whose extracutaneous symptoms included severe mental and motor impairment, convulsions, and deformity of the orofacial region. She also had severe obesity, asthma, multiple allergies, and skin hypersensitivity. Although no extracutaneous manifestations were problematic during perioperative management of dental procedures under general anesthesia, erythema developed at 3 time points: during induction, during emergence, and in recovery. We speculated that mechanical stimuli to the skin and administration of multiple drugs likely caused histamine release, leading to the 3 episodes of erythema. Because patients with HI often have hypersensitivity reactions in the skin, both cutaneous and extracutaneous manifestations should be considered in the anesthetic management of patients with HI.