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Rare disease
Incontinentia pigmenti in the neonatal period
  1. Vera Rodrigues1,
  2. Filipa Diamantino2,
  3. Olga Voutsen3,
  4. Manuel Sousa Cunha3,
  5. Rosalina Barroso3,
  6. Maria João Paiva Lopes2,
  7. Helena Carreiro4
  1. 1Department of Pediatrics, Hospital de Dona Estefânia, Centro Hospitalar de Lisboa Central, EPE, Lisboa, Portugal
  2. 2Department of Dermatology, Hospital de Santo António dos Capuchos, Centro Hospitalar de Lisboa Central, EPE, Lisboa, Portugal
  3. 3Department of Neonatology, Hospital Prof. Doutor Fernando Fonseca, EPE, Amadora, Portugal
  4. 4Department of Pediatrics, Hospital Prof. Doutor Fernando Fonseca, EPE, Amadora, Portugal
  1. Correspondence to Dr Vera Rodrigues, veralarodrigues{at}gmail.com

Summary

Incontinentia pigmenti (IP) is a rare multisystem disease, X linked dominant disorder. As all X linked dominant diseases, it is usually male-lethal. Female newborn admitted to the neonatal intensive care unit on the first day of life was diagnosed as having probable herpetic infection with vesicular skin lesions distributed on upper right limb and inferior limbs. Family history showed that her 22-year-old mother had hypopigmented lesions on the lower limbs and her 13-month-old sister had hyperpigmented lesions on the trunk and limbs. In newborns, herpes infection emerges as the principal diagnosis of vesicular rash, due to the importance of precocious diagnosis and treatment. Other hypothesis must be considered in a newborn with vesicobullous rash, such as IP.

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Footnotes

  • Competing interests None.

  • Patient consent Obtained.