RT Book, Section A1 Torres-Zegarra, Carla A1 Appachi, Elumalai A2 Usatine, Richard P. A2 Sabella, Camille A2 Smith, Mindy Ann A2 Mayeaux, E.J. A2 Chumley, Heidi S. A2 Appachi, Elumalai SR Print(0) ID 1114880963 T1 Incontinentia Pigmenti T2 The Color Atlas of Pediatrics YR 2015 FD 2015 PB McGraw-Hill Education PP New York, NY SN 978-0-07-176701-9 LK accesspediatrics.mhmedical.com/content.aspx?aid=1114880963 RD 2024/03/28 AB A 6-day-old girl is brought by her parents to the emergency department for a rash on her arms and chest. The parents noted that over the past 3 days, the baby has had intermittent episodes of right eye deviation and upper extremity stiffening. These episodes lasted approximately 30 seconds, occurred approximately 3 times per day and were associated with cyanosis. The family history was notable for seizures in a maternal aunt. On physical examination the infant was quiet, seemingly withdrawn with stable vital signs. The infant had erythematous papules and vesicles, some of which had eroded with crusting on the arms and anterior chest (Figure 228-1). A direct fluorescent antibody test and culture from the lesions were negative for herpes simplex virus. Skin biopsy of the lesions showed spongiotic dermatitis with many eosinophils and large dyskeratotic cells. Based on the skin lesions and the neurological manifestations, the infant was diagnosed with incontinentia pigmenti. Genetic, neurologic, and ophthalmology consults were obtained.