A patient on long-term immunosuppression presents with verrucous (wart-like) lesions over the trunk and limbs. Histology shows koilocytes and intranuclear inclusions. HPV types 5 and 8 are implicated. This condition is associated with which genodermatosis?
- A Dyskeratosis congenita
- B Epidermodysplasia verruciformis ✓
- C Netherton syndrome
- D Kindler syndrome
Explanation
Epidermodysplasia verruciformis (EV) is a rare autosomal recessive genodermatosis caused by mutations in EVER1 (TMC6) or EVER2 (TMC8) genes, which are zinc transporter regulators. Patients are unusually susceptible to HPV types 5, 8, 9, 12, 14, and 15 (EV-specific types). The flat warts and pityriasis-versicolor-like lesions begin in childhood; SCC develops in 30–60% of patients by age 40 on sun-exposed areas. HPV-5 and HPV-8 have the highest oncogenic potential in EV.
Reference: Neena Khanna Illustrated Synopsis of Dermatology & STD, 6th ed.
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Written and medically reviewed by the StethoPrep medical team.