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	Incontinentia pigmenti achromians;
OMIM	300337
DiseasesDB	31154
MedlinePlus	001461
MeSH	D010859

VisualWikitext

Latest revision as of 19:19, 7 September 2014

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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

Incontinentia pigmenti achromians of Ito. Adapted from Dermatology Atlas.^[2]

Incontinentia pigmenti achromians of Ito. Adapted from Dermatology Atlas.^[2]

Extremity

Incontinentia pigmenti achromians of Ito. Adapted from Dermatology Atlas.^[2]