Inherited mutations in EVER1/TMC6 or EVER2/TMC8 lead to susceptibility to HPV infection
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
Clinically shows overlapping features with EDV
Epidermal acanthosis with hypergranulosis, hyperkeratosis, but lacks prominent bluish-gray staining cells of EDV
• Actinic keratosis
Basilar keratinocytes show budding, nuclear enlargement, and atypia
• Bowen disease/squamous cell carcinoma in situ
• EDV acanthoma
Epidermodysplasia Verruciformis Epidermodysplasia verruciformis (EDV) is characterized by irregular epidermal acanthosis associated with an intraepidermal proliferation of enlarged, bluish-gray staining keratinocytes . Note the overlying hypergranulosis .
Hypergranulosis, Hyperkeratosis, and Parakeratosis in EDV Higher magnification of EDV shows a proliferation of enlarged bluish-gray keratinocytes involving the mid to upper layers of the epidermis. Note the dense overlying hyperkeratosis and parakeratosis .
Nuclear Clearing in EDV Some of the superficial HPV-infected keratinocytes show prominent nuclear clearing .
Prominently Bluish/Gray Keratinocytes in EDV High magnification of another example of EDV shows a proliferation of enlarged, bluish-gray staining keratinocytes , some with prominent keratohyaline granules .
TERMINOLOGY
Abbreviations
• Epidermodysplasia verruciformis (EDV)
Definitions
• HPV-related intraepidermal proliferation of enlarged, often bluish-gray staining keratinocytes
ETIOLOGY/PATHOGENESIS
Environmental Exposure
• HPV infection is established as etiologic agent
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. Note the overlying hypergranulosis 
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involving the mid to upper layers of the epidermis. Note the dense overlying hyperkeratosis and parakeratosis 
.
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, some with prominent keratohyaline granules 
.
