Skin-nontumor / Clinical dermatology
Infectious disorders
Epidermodysplasia verruciformis

Author: Nat Pernick, M.D. (see Authors page)

Revised: 23 May 2016, last major update July 2011

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: Epidermodysplasia verruciformis

Cite this page: Epidermodysplasia verruciformis. PathologyOutlines.com website. http://pathologyoutlines.com/topic/skinnontumorepidermodysplasia.html. Accessed December 4th, 2016.
Definition / General
  • Disorder of multiple flat warts on face or distal extremities, often disseminated throughout the body
Etiology
  • Congenital or post-transplant
  • Abnormal susceptibility to clinical HPV infection, which causes only asymptomatic infections in 80% of normal population (Wikipedia)
  • Congenital cases are usually autosomal recessive; rarely X linked recessive or autosomal dominant transmission
  • Often appears before age 10 years or after renal transplantation
  • Usually HPV 5 or 8 (Am J Surg Pathol 2000;24:1400), less commonly 3, 4, 51
  • Congenital cases often due to mutation that inactivates EVER1 and EVER2 (eMedicine)
Clinical Features
  • 30 - 50% progress to invasive squamous cell carcinoma, mean age 31 years
  • Equivocal cases are best distinguished by histology and viral testing, in context of clinical setting
  • Flat, skin-colored, red or hyperpigmented papules resembling flat warts or scaly brown pityriasis versicolor-like macules and patches
  • Also seborrheic keratosis-like
Treatment
  • Excision or locally destructive methods
Clinical Images

Images hosted on other servers:

Various images



Arm

Micro Description
  • Classic features are mild to moderate acanthosis and hyperkeratosis
  • Large cells with blue-gray cytoplasm, perinuclear halos, often dysplastic changes, particularly post kidney transplantation (J Cutan Pathol 2002;29:480)
  • More commonly has irregular granular layer with rare perinuclear halos
Micro Images

Images hosted on other servers:

Various images



Characteristic enlarged keratinocytes with blue-gray pallor; no significant keratinocytic atypia, pleomorphism or mitotic figures