Abstract

Epidermodysplasia verruciformis (EDV) is a rare, inherited disorder that is characterized by an increased susceptibility to infection with specific human papilloma viruses (HPV) and a greater propensity for developing malignant skin tumors.
 A 26 years old Nepalese male patient presented with complaints of multiple asymptomatic raised warty lesions on bilateral hands and multiple light colored flat to raised lesions present over the neck, trunk, bilateral extremities sparing the face and genitals.A skin biopsy was performed which revealed hyperkeratosis, focal parakeratosis, acanthosis, keratinocytes were swollen and irregularly shaped, had abundant basophilic cytoplasm and contained numerous round basophilic keratohyaline granules. Nuclei were large, round and empty with marginal distribution of chromatin, some were pyknotic.
 Epidermodysplasiaverruciformis is a rare inherited disorder spread by HPV. It is characterized by hypopigmented or hyperpigmented macular lesions, pityriasis versicolor-like lesions and an early tendency to develop skin malignancies. The management of EDV includes early diagnosis, sun protection, life-long observation for the malignant transformation and therapeutic modalities like acitretin, imiquimod, topical retinoids, cryotherapy, etc.

Highlights

  • Epidermodysplasiaverruciformis(EDV) is a rare disease characterized by susceptibility to infections caused by human papillomaviruses (HPV) HPV 5, 8, 9, 12, 14, 15, 17, 19 -25 and due to defect in cell mediated immune response.[1]

  • A 26 years old Nepalese male patient presented to dermatology outpatient department (OPD) with

  • There is a high risk of development of cutaneous squamous cell carcinomas in early adult life.[2]

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Summary

Introduction

Epidermodysplasiaverruciformis(EDV) is a rare disease characterized by susceptibility to infections caused by human papillomaviruses (HPV) HPV 5, 8, 9, 12, 14, 15, 17, 19 -25 and due to defect in cell mediated immune response.[1] The skin lesions start in childhood and are predominantly seen in the sun exposed sites.[2] Lesions can present as widespread skin eruptions of flat to papillomatous wart-like lesions and reddish brown pigmented plaque on the trunk, extremities and face, and can present as pityriasis versicolor-like lesions.[3] There is high risk of developing cutaneous squamous cell carcinomas (SCC) in early adult life.[2]

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