Pediatric emergency medicine trisk 1332 1332
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mg/m2/day divided in 3 doses). Also, for both CNS and disseminated diseases,
repeat CSF analysis and CSF HSV PCR before stopping therapy and, if
detectable in CSF, continue therapy until negative CSF PCR results. Dose of
acyclovir should be weight-adjusted for the duration of therapy. Absolute
neutrophil count should be monitored every 2 weeks for the first month and then
monthly.
Incontinentia Pigmenti
Incontinentia pigmenti (IP) is a rare X-linked genodermatosis that primarily
affects female neonates (see Chapter 67 Rash: Vesiculobullous ). The first
manifestation occurs in the early neonatal period and progresses through four
stages: vesicular, verruciform, hyperpigmented, and hypopigmented. Clinical
features also manifest themselves through changes in the teeth, eyes, hair, CNS,
bone structures, skeletal musculature, and immune system. IP is often mistaken
for an infectious process ( Fig. 69.8 ). PCR and cultures of the vesicles yield
negative results. The clue to this diagnosis is that the vesicles usually occur on the
arms and/or legs in a linear pattern that follows the lines of Blaschko. Genetic
testing and/or a biopsy can help confirm the diagnosis. Rarely, boys that are XXY
or have somatic mosaicism present with IP.
FIGURE 69.8 Linear vesicles in a newborn with the first stage of incontinentia pigmenti.
