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Chapter 100: Epidermodysplasia Verruciformis

Sivan Sheffer-Levi; Yuval Ramot

EPIDERMODYSPLASIA VERRUCIFORMIS

Therapeutic Approach

Epidermodysplasia verruciformis (EV) is a rare genetic disease that leads to an abnormal susceptibility to cutaneous infections with a large number of genus β human papilloma viruses. Patients with EV present with generalized verrucae planae, pityriasis versicolor-like macules, and an early development of squamous cell carcinomas (SCC), especially in sun-exposed areas. There is currently no cure for EV. Patients require lifelong monitoring for skin cancer and education regarding strict sun protection. Treatment is aimed at alleviating the skin lesions. See Table 100-1.

Table 100-1Epidermodysplasia Verruciformis Treatment Table
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Table 100-1 Epidermodysplasia Verruciformis Treatment Table
MEDICATION NAME INDICATIONb MECHANISM OF ACTION DOSING ADVERSE EFFECTS SUGGESTED MONITORING LEVEL OF EVIDENCE (REFERENCE)
Topical Therapy
Imiquimoda SCC, BD, AK, warts Immunomodulator, anti-viral, anti-tumoral Cream 5%; 5 d weekly for 2-3 mo Local skin reactions, nausea, headache, and flu-like symptoms None IV1-4
5-Fluorouracila BD, AK Antimetabolite 5% cream, apply BID Local skin reactions, potentially teratogenic None IV5
Squaric acid dibutylester Flat warts Unclear, contact sensitizer/immunomodulatory

Sensitization: 2% solution, then 0.2% solution

Application frequency ranging from daily to monthly

 Local skin reactions None IV6
Tacalcitol Keratotic plaques, BD Inhibition of proliferation and promotion of differentiation of keratinocytes NA Mild local skin reactions None IV8
Systemic Therapy
Isotretinoin Flat warts, prevention of SCC Antiproliferative and differentiation-inducing effect 0.33-1 mg/kg/QD for 6 mo, then 0.3 mg/kg QD Teratogenic, dry skin, cheilitis, dry eyes, myalgia, headache, dyslipidemia, and elevated liver enzymes CBC, CMP, and lipid panel at baseline, monthly for the first 2 mo and then every 3 mo; pregnancy test monthly IV9,10
Acitretin Flat warts, prevention of SCC Antiproliferative and antiviral 1 mg/kg QD Teratogenic, dry skin, itching, dry eyes, dry lips, hair loss, hypercholesterolemia CBC, CMP, and lipid panel at baseline, monthly for the first 2 mo and then every 3 mo; pregnancy test monthly IV5,11,12
Etretinate Warts Antiproliferative 0.7-1 mg/kg/d, gradual tapering after 1 mo Teratogenic, dry skin, dry lips, dry eyes, muscle cramps, bone toxicity, dyslipidemia CBC, CMP, and lipid panel at baseline, monthly for the first 2 mo and then every 3 mo; pregnancy test monthly IV13-15
Systemic interferon alpha Warts, NMSC Antiviral, antiproliferative, immunomodulatory 8 × 104 U/kg IM thrice weekly for 4 wk/6 million units twice weekly for 17 wk Flu-like symptoms, rash, cardiotoxicity, GI upset, bone marrow suppression CBC, CMP, and lipid panel at baseline and then weekly III3,16
Intralesional interferon alphaa Warts, BD Antiviral, anti-proliferative, immunomodulatory l05 U thrice weekly for 4 wk Flu-like symptoms, rash, cardiotoxicity, GI upset, bone marrow suppression CBC, CMP, and lipid panel at baseline and then weekly III13,16
Intralesional interferon beta Warts Antiviral, antiproliferative, immunomodulatory 3 mL (105 U) once every other day for 6 treatments Flu-like symptoms, rash, ...

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