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Description
  • Herein, we report the case of a 12-year-old female who noted the recent onset of an oval, circumscribed, 10-cm papillomatous plaque affecting the thigh and vulva that showed histologic signs of lymphedema without evidence of secondary lymphedema. The sequencing of genes associated with a delayed onset of lymphedema or epidermal nevi (EN) - GATA2 and GJC2 , and HRAS and KRAS , respectively - showed wild-type alleles. Polymerase chain reaction for human papillomavirus (HPV) DNA demonstrated infections with 15 HPV genotypes. Evidence of productive HPV infection, HPV capsid expression, and cytopathic changes was detected. At the 6-month follow-up, no evidence of recurrence was found after complete excision. The analysis of a consecutive series of 91 EN excision specimens revealed that 76% exhibited histologic evidence of lymphostasis. Notably, multiple acrochordon-like EN, which most closely resembled this case, showed similar signs of localized lymphedema. The late onset and evidence of lymphedema favors the diagnosis of congenital unisegmental lymphedema. However, the clinical findings and epidermal changes point to the diagnosis of EN. Moreover, localized verrucosis also accurately describes this patient's cutaneous findings. Based on the above evidence, we postulate that an abnormal development of lymphatics may play a primary role in the pathogenesis of some types of EN and facilitate productive HPV infection.
  • Herein, we report the case of a 12-year-old female who noted the recent onset of an oval, circumscribed, 10-cm papillomatous plaque affecting the thigh and vulva that showed histologic signs of lymphedema without evidence of secondary lymphedema. The sequencing of genes associated with a delayed onset of lymphedema or epidermal nevi (EN) - GATA2 and GJC2 , and HRAS and KRAS , respectively - showed wild-type alleles. Polymerase chain reaction for human papillomavirus (HPV) DNA demonstrated infections with 15 HPV genotypes. Evidence of productive HPV infection, HPV capsid expression, and cytopathic changes was detected. At the 6-month follow-up, no evidence of recurrence was found after complete excision. The analysis of a consecutive series of 91 EN excision specimens revealed that 76% exhibited histologic evidence of lymphostasis. Notably, multiple acrochordon-like EN, which most closely resembled this case, showed similar signs of localized lymphedema. The late onset and evidence of lymphedema favors the diagnosis of congenital unisegmental lymphedema. However, the clinical findings and epidermal changes point to the diagnosis of EN. Moreover, localized verrucosis also accurately describes this patient's cutaneous findings. Based on the above evidence, we postulate that an abnormal development of lymphatics may play a primary role in the pathogenesis of some types of EN and facilitate productive HPV infection. (en)
Title
  • Adolescent onset of localized papillomatosis, lymphedema, and multiple Beta-papillomavirus infection: epidermal nevus, segmental lymphedema praecox, or verrucosis? A case report and case series of epidermal nevi
  • Adolescent onset of localized papillomatosis, lymphedema, and multiple Beta-papillomavirus infection: epidermal nevus, segmental lymphedema praecox, or verrucosis? A case report and case series of epidermal nevi (en)
skos:prefLabel
  • Adolescent onset of localized papillomatosis, lymphedema, and multiple Beta-papillomavirus infection: epidermal nevus, segmental lymphedema praecox, or verrucosis? A case report and case series of epidermal nevi
  • Adolescent onset of localized papillomatosis, lymphedema, and multiple Beta-papillomavirus infection: epidermal nevus, segmental lymphedema praecox, or verrucosis? A case report and case series of epidermal nevi (en)
skos:notation
  • RIV/00216208:11140/14:10283638!RIV15-MSM-11140___
http://linked.open...avai/riv/aktivita
http://linked.open...avai/riv/aktivity
  • I
http://linked.open...iv/cisloPeriodika
  • 2
http://linked.open...vai/riv/dodaniDat
http://linked.open...aciTvurceVysledku
http://linked.open.../riv/druhVysledku
http://linked.open...iv/duvernostUdaju
http://linked.open...titaPredkladatele
http://linked.open...dnocenehoVysledku
  • 1671
http://linked.open...ai/riv/idVysledku
  • RIV/00216208:11140/14:10283638
http://linked.open...riv/jazykVysledku
http://linked.open.../riv/klicovaSlova
  • congenital lymphedema; Kras; HRAS; GJC2; GATA2; Blaschko's lines; elephantiasis; lymfphedema; human papillomavirus; epidermal nevus (en)
http://linked.open.../riv/klicoveSlovo
http://linked.open...odStatuVydavatele
  • DE - Spolková republika Německo
http://linked.open...ontrolniKodProRIV
  • [F5966BE49550]
http://linked.open...i/riv/nazevZdroje
  • Dermatopathology
http://linked.open...in/vavai/riv/obor
http://linked.open...ichTvurcuVysledku
http://linked.open...cetTvurcuVysledku
http://linked.open...UplatneniVysledku
http://linked.open...v/svazekPeriodika
  • 1
http://linked.open...iv/tvurceVysledku
  • Kazakov, Dmitry
  • Carlson, J. Andrew
  • Šedivcová, Monika
  • Stehlík, Jan
  • Agag, Richard
  • Hill, Jarome
  • Kadam, Pooja
  • Rady, Peter
  • Rand, Janne
  • Ray, Kathy
  • Tyring, Stephen
issn
  • 2296-3529
number of pages
http://bibframe.org/vocab/doi
  • 10.1159/000367967
http://localhost/t...ganizacniJednotka
  • 11140
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