Skin nontumor
Infectious disorders
Epidermodysplasia verruciformis
Author: Nat Pernick, M.D.
Last author update: 1 July 2011
Last staff update: 19 September 2023
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: Epidermodysplasia verruciformis
Table of Contents
Definition / general | Etiology | Clinical features | Treatment | Clinical images | Microscopic (histologic) description | Microscopic (histologic) imagesCite this page: Pernick N. Epidermodysplasia verruciformis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skinnontumorepidermodysplasia.html. Accessed April 18th, 2024.
Definition / general
- Disorder of multiple flat warts on face or distal extremities, often disseminated throughout the body
Etiology
- Congenital or posttransplant
- Abnormal susceptibility to clinical HPV infection, which causes only asymptomatic infections in 80% of normal population (Wikipedia: Epidermodysplasia Verruciformis [Accessed 29 August 2018])
- 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: Epidermodysplasia Verruciformis [Accessed 29 August 2018])
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:
Papillomatous and macular lesions
Hypopigmented papules and plaques
Microscopic (histologic) 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
Microscopic (histologic) images
Contributed by @JMGardnerMD on Twitter
Epidermodysplasia verruciformis
Images hosted on other servers:
Various images
Characteristic enlarged keratinocytes with blue-gray pallor; no significant keratinocytic atypia, pleomorphism or mitotic figures
