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Please use this identifier to cite or link to this item: http://repository.li.mahidol.ac.th/dspace/handle/123456789/23832
Title: Incontinentia pigmenti achromians of Ito: An ultrastructural study
Authors: Piti Palungwachira
Pranee Palungwachira
Srinakharinwirot University Skin Center
Mahidol University
Srinakharinwirot University
Keywords: Medicine
Issue Date: 1-Feb-2006
Citation: Journal of the Medical Association of Thailand. Vol.89, No.2 (2006), 253-257
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.
URI: https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=33645963975&origin=inward
http://repository.li.mahidol.ac.th/dspace/handle/123456789/23832
ISSN: 01252208
01252208
Appears in Collections:Scopus 2006-2010

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