Incontinentia pigmenti achromians

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Incontinentia pigmenti achromians
OMIM 300337
DiseasesDB 31154
MedlinePlus 001461
MeSH D010859

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Kiran Singh, M.D. [2]

Synonyms and Keywords: Hypomelanosis of Ito; Ito's hypomelanosis

Overview

Incontinentia pigmenti achromians is a cutaneous condition characterized by various patterns of bilateral or unilateral hypopigmentation following the lines of Blaschko.[1]. Though the consistency of the skin findings have led to the term "hypomelanosis of Ito", it actually refers to a group of disorders with various genetic causes including polyploidies and aneuploidies. Based upon the specifics of the genetic defect, the skin findings can be accompanied by a great range of systemic findings. These include central nervous system, ocular, and musculoskeletal defects. Nonetheless, the vast majority of cases are limited to the skin. As opposed to incontinentia pigmenti, hypomelanosis of Ito affects both genders equally.

Diagnosis

Physical Examination

Skin

Trunk
Extremity
Gluteal Region

See Also

References

  1. James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.
  2. 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 2.11 2.12 2.13 2.14 2.15 "Dermatology Atlas".