Publication:
Incontinentia pigmenti achromians of Ito: An ultrastructural study

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Issued Date

2006

Resource Type

Article

File Type

application/pdf

ISSN

1252208

Other identifier(s)

2-s2.0-33645963975

Rights Holder(s)

Scopus

Bibliographic Citation

Journal of the Medical Association of Thailand. Vol 89, No.2 (2006), p.253-257

Suggested Citation

Palungwachira P., Palungwachira P. Incontinentia pigmenti achromians of Ito: An ultrastructural study. Journal of the Medical Association of Thailand. Vol 89, No.2 (2006), p.253-257. Retrieved from: https://hdl.handle.net/20.500.14740/5933

Title

Incontinentia pigmenti achromians of Ito: An ultrastructural study

Author(s)

Palungwachira P.
 Palungwachira P.

Abstract

A clinico-pathological and EM study of a Thai boy with hypomelanosis of Ito, one of the neurocutaneous syndromes, is reported. At birth, typical skin hypopigmentation on the trunk and a hypopigmented streak on the left lower extremity were noted. Associated cutaneous pathology shows a decrease of melanin granules within basal and malpighian keratinocytes. Ultrastructural studies highlight a normal appearance for basal and malpighian keratinocytes, but a lack of melanosomes in the malpighian cells. Melanosomes are also dramatically reduced in the basal keratinocytes, which appear small, single or clustered and surrounded by a membrane. Melanocytic degeneration has been observed and dendritic melanocytes contained various stages of nonmelanised (stage II), partially melanised premelanosome (stage III) and rarely stage 4 melanosomes. The authors observed an increase in the number of Langerhans cell which have not previously been described. There were unmyelinated axon of nerve containing melanosomes at the dermoepidermal junction. The significance of these findings will be worthwhile to note that abnormal nerve termination in close relationship with basal keratinocyte, degenerated melanocyte, premelanosomes and langerhans cell are important in explaining the pathogenesis of Hypomelanosis of Ito.

Subject(s)

Melanin
 Anamnesis
 Article
 Case report
 Cell ultrastructure
 Clinical feature
 Dermoepidermal junction
 Disease course
 Human
 Hypomelanosis
 Hypopigmentation
 Incontinentia pigmenti
 Ito cell
 Keratinocyte
 Langerhans cell
 Male
 Melanosome
 Nonmyelinated nerve
 Preschool child
 Electron microscopy
 Follow up
 Hospitalization
 Immunohistochemistry
 Needle biopsy
 Pathology
 Pigment disorder
 Sensitivity and specificity
 Skin
 Ultrastructure
 Biopsy, Needle
 Child, Preschool
 Follow-Up Studies
 Humans
 Immunohistochemistry
 Male
 Microscopy, Electron
 Pigmentation Disorders
 Sensitivity and Specificity
 Severity of Illness Index
 Skin

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URI

https://hdl.handle.net/20.500.14740/5933

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Scopus: Year 1983-2021

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