Epidermodysplasia Verruciformis

 Inherited mutations in EVER1/TMC6 or EVER2/TMC8 lead to susceptibility to HPV infection


image Increased risk for development of in situ and invasive SCC





Clinical Issues




• As most cases are benign, aggressive treatment not usually indicated

• Topical antivirals (similar to those used for verrucae) may be used

• Liquid nitrogen (cryotherapy) is usually effective

• Radiotherapy should be avoided (increased risk for malignancy)


Microscopic




• Epidermal acanthosis with proliferation of enlarged cells with abundant bluish-gray staining cytoplasm

• Perinuclear halos usually prominent

• High-grade atypia usually not present

• Mitotic figures are rare


Top Differential Diagnoses




• Verruca plana
image Clinically shows overlapping features with EDV

image Epidermal acanthosis with hypergranulosis, hyperkeratosis, but lacks prominent bluish-gray staining cells of EDV

• Actinic keratosis
image Basilar keratinocytes show budding, nuclear enlargement, and atypia

• Bowen disease/squamous cell carcinoma in situ

• EDV acanthoma

image
Epidermodysplasia Verruciformis
Epidermodysplasia verruciformis (EDV) is characterized by irregular epidermal acanthosis associated with an intraepidermal proliferation of enlarged, bluish-gray staining keratinocytes image. Note the overlying hypergranulosis image.


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Hypergranulosis, Hyperkeratosis, and Parakeratosis in EDV
Higher magnification of EDV shows a proliferation of enlarged bluish-gray keratinocytes image involving the mid to upper layers of the epidermis. Note the dense overlying hyperkeratosis and parakeratosis image.

image
Nuclear Clearing in EDV
Some of the superficial HPV-infected keratinocytes show prominent nuclear clearing image.

image
Prominently Bluish/Gray Keratinocytes in EDV
High magnification of another example of EDV shows a proliferation of enlarged, bluish-gray staining keratinocytes image, some with prominent keratohyaline granules image.


TERMINOLOGY


Abbreviations




• Epidermodysplasia verruciformis (EDV)


Definitions




• HPV-related intraepidermal proliferation of enlarged, often bluish-gray staining keratinocytes

Apr 24, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Epidermodysplasia Verruciformis
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