131. Hypomelanosis of Ito/Pigmentary Mosaicism

  1. Alan D. Irvine MD, FRCPI, FRCP2,3,
  2. Peter H. Hoeger MD4,5 and
  3. Albert C. Yan MD, FAAP, FAAD6,7
  1. Saleem M. Taibjee and
  2. Celia Moss

Published Online: 24 MAY 2011

DOI: 10.1002/9781444345384.ch131

Harper's Textbook of Pediatric Dermatology, Volume 1, 2, Third Edition

Harper's Textbook of Pediatric Dermatology, Volume 1, 2, Third Edition

How to Cite

Taibjee, S. M. and Moss, C. (2011) Hypomelanosis of Ito/Pigmentary Mosaicism, in Harper's Textbook of Pediatric Dermatology, Volume 1, 2, Third Edition (eds A. D. Irvine, P. H. Hoeger and A. C. Yan), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781444345384.ch131

Editor Information

  1. 2

    Trinity College, Dublin, Ireland

  2. 3

    Our Lady's Children's Hospital, Dublin, Ireland

  3. 4

    University of Hamburg, Hamburg, Germany

  4. 5

    Catholic Children's Hospital Wilhelmstift, Hamburg, Germany

  5. 6

    University of Pennsylvania School of Medicine, Philadelphia, PA, USA

  6. 7

    The Children's Hospital of Philadelphia, Philadelphia, PA, USA

Author Information

  1. Department of Dermatology, Birmingham Children's Hospital, Birmingham, UK

Publication History

  1. Published Online: 24 MAY 2011
  2. Published Print: 3 JUN 2011

ISBN Information

Print ISBN: 9781405176958

Online ISBN: 9781444345384

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Keywords:

  • Blaschko's lines;
  • cytogenetic;
  • hypomelanosis of Ito;
  • linear and whorled naevoid hypermelanosis;
  • pigmentary mosaicism

Summary

Pigmentary mosaicism encompasses the disorders hypomelanosis of Ito (formerly incontinentia pigmenti achromians) and linear and whorled naevoid hypermelanosis. It is characterized by Blaschkoid or other mosaic patterns of cutaneous dyspigmentation in association with systemic abnormalities, most usually neurological and/or musculoskeletal. Cytogenetic mosaicism is demonstrable in a proportion of cases. The clinical heterogeneity, including multitude of associated systemic anomalies, may be explained by the wide range of reported cytogenetic abnormalities. Dyspigmentation may arise by disruption of a number of potential pigmentary genes, reflected by the variable histopathological and ultrastructural findings. Management is usually multidisciplinary, based on the additional systems involved. A number of treatment modalities have been employed to correct the dyspigmentation, including autologous epidermal transplantation, lasers and chemical peels, but with variable success.