Apparent predictors of good response to ECP include erythroderma, a relatively low (closer to normal) peripheral CD4 : CD8, and a normal LDH. This report confirms reports from other institutions indicating that CTCL patients who are erythrodermic with a relatively intact immune system are optimal candidates for ECP.
The goals of anesthesia are to provide for patient safety and comfort, to increase patient acceptance of the procedure, and to enhance the surgeon's efficiency and satisfaction.
Epidermolytic palmoplantar keratoderma (EPPK) is a localized keratinization disorder caused by mutations in the highly conserved coil 1A domain of the keratin 9 gene, KRT9. We present a Hispanic pedigree spanning three generations, with affected individuals in all generations. Using polymerase chain reaction amplification and direct sequencing we demonstrated a previously reported missense mutation in KRT9, which is expressed almost exclusively in the skin of palms and soles. The C-->T missense mutation R162W changes a basic amino acid (arginine) to a neutral amino acid (tryptophan). We describe this mutation in a Hispanic pedigree with EPPK for the first time, extending the finding of this mutation in other genetic backgrounds, and demonstrating the prevalence of this mutation in diverse populations.
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