PMID: 7544893Jun 1, 1995Paper

CHILD syndrome: analysis of abnormal keratinization and ultrastructure

Pediatric Dermatology
K HashimotoM Edwards

Abstract

A new patient with CHILD syndrome (congenital hemidysplasia, ichthyosiform erythroderma, and limb defects), the thirtieth in the literature, was observed for over three years. Initially, the right-sided lesion spared the breast area. At 10 months of age the trunk lesion extended to cover the entire area of the right chest. At age 20 months the patient developed linear, bandlike, keratotic, brown-black lesions on her left thigh that subsided within six weeks, leaving a slight hyperpigmentation. This patient was studied by routine histologic methods as well as with markers of keratinization and electron microscopy. In hematoxylin and eosinstained sections, parakeratosis and orthokeratosis alternated. In some parakeratotic areas, large granular cells, and in others, ghost granular cells, were present. The latter showed basophilic cytoplasm, and palestaining or vacuolated nucleus and were seen either above the normal granular layer or without it. Although regional variations existed, basal cell-type keratins as recognized by AE1 continued to be expressed in suprabasal layers. Filaggrin- and involucrin-positive layers were expanded, particularly the latter, down to the lower prickle cell layer. Ultrastructurally, numerous lamellar o...Continue Reading

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Citations

Dec 9, 2000·Biochimica Et Biophysica Acta·G E Herman
Sep 1, 1993·Pediatric Dermatology·K HashimotoT Kanzaki
Nov 10, 2001·Annual Review of Genomics and Human Genetics·R I Kelley, G E Herman
Jun 2, 2011·International Journal of Alzheimer's Disease·Roberta LizioClaudio Babiloni
Mar 8, 2016·The Journal of Dermatology·Kozo Yoneda
Sep 11, 2003·American Journal of Medical Genetics. Part a·Marybeth HummelGail E Herman
Jun 1, 1996·Journal of Cutaneous Pathology·K HoweW Johnson
Jun 17, 2006·Journal of Cutaneous Pathology·Masafumi IshibashiAkira Ishiko
Nov 13, 2009·Journal of the European Academy of Dermatology and Venereology : JEADV·G P AvgerinouR Happle
Mar 10, 2000·American Journal of Medical Genetics·D K GrangeR I Kelley

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