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.
First Episode of Erythema During Induction
After intubation, the patient developed generalized erythema extending from her face to the thoracoabdominal region.
Second Episode of Erythema After Administration of Sugammadex
The second episode of erythema, which involved the patient’s neck and chest, developed after administration of sugammadex with the erythema being more pronounced on the right side of the chest. This photograph was taken approximately 15 minutes after the erythema appeared and after extubation.
Third Episode of Erythema First Noted in Recovery
Reticular erythema, attributed to the noninvasive blood pressure cuff compression, was noted on the patient’s right hand during recovery and before returning to the ward.
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