Incontinentia pigmenti achromians

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Incontinentia pigmenti achromians
Other namesHypomelanosis of Ito, Linear nevoid hypopigmentation
Diagnostic methodClinical; use of Wood's lamp; skeletal imaging; computed tomography and MRI if there are neurological symptoms

Incontinentia pigmenti achromians, also known as hypomelanosis of Ito (HI) or pigmentary mosaicism,[1] is a cutaneous (skin) condition characterized by various patterns of hypopigmentation following the lines of Blaschko on one or both sides of the body.[2]: 548–9  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, including defects of the ocular, musculoskeletal, and central nervous systems. As opposed to incontinentia pigmenti, hypomelanosis of Ito affects both genders equally. This disorder was first described by Japanese dermatologist Minoru Ito in 1952.

Diagnosis

HI is diagnosed through clinical examination, sometimes with the aid of Wood's lamp to highlight the differences in pigmentation.[1] The skeleton should also be imaged.[1]

A CT scan and magnetic resonance imaging should be completed if the patient has neurologic symptoms, and patients with seizure disorders should undergo electromyography.[1]

History

This disorder was first described by Japanese dermatologist Minoru Ito in 1952.[1][3]

See also

References

  1. 1.0 1.1 1.2 1.3 1.4 Chamli, Amal; Litaiem, Noureddine (2025), "Hypomelanosis of Ito", StatPearls, Treasure Island (FL): StatPearls Publishing, PMID 30855856, retrieved 2025-04-18
  2. James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.
  3. Ito, Minoru (1952). "Studies on Melanin XI. Incontinentia pigmenti achromians. A single case of nevus depigmentosus systematicus bilaterlis". Tohoku J Exp Med. 55 (Supplement): 57–59. doi:10.1620/tjem.55.Supplement_34.

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