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Skin-nontumor / Clinical Dermatology

Infectious disorders

Epidermodysplasia verruciformis


Reviewer: Nat Pernick, M.D., PathologyOutlines.com, Inc. (see Reviewers page)
Revised: 3 July 2011, last major update July 2011
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.

Definition
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● Disorder of multiple flat warts on face or distal extremities, often disseminated throughout the body

Etiology
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● 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
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● 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

Treatment
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● Excision or locally destructive methods

Clinical description
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● Flat, skin-colored, red or hyperpigmented papules resembling flat warts or scaly brown pityriasis versicolor-like macules and patches
● Also seborrheic keratosis-like

Clinical images
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Various images


Arm

Micro description
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● 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
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Various images


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

End of Skin-nontumor / Clinical Dermatology > Infectious disorders > Epidermodysplasia verruciformis


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