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mg/m2/day divided in 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 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

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