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Epidermodysplasia Verruciformis : Introduction

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Introduction: Epidermodysplasia verruciformis (EV) is a rare genodermatosis (genetic disorder with cutaneous expression) that results in abnormal susceptibility to cutaneous HPV infections (caused mostly by HPV5 and HPV8 of the beta genus) and a higher risk of non-melanoma skin cancers.

It has also been referred to as Lewandowsky-Lutz syndrome. The transmission is autosomal recessive in most patients; however, rare sex-linked and autosomal dominant cases have been documented. The patients are otherwise healthy and are not immunocompromised. They have normal resistance to other microorganisms, viruses, and even other cutaneous and mucosal HPVs.

This skin biopsy is from an HIV-positive individual who presented with several papules in the vicinity of a tatoo. An acquired EV-like condition has been observed in patients with compromised immune system (SLE, Hodgkin lymphoma, HIV infection, organ transplantation, CD4 lymphopenia, CD8 lymphopenia, and chemotherapy etc).

The image shows acanthosis, hyperkeratosis, and enlarged keratinocytes with bluish-gray cytoplasm in the upper layers of epidermis. Further discussion of microscopic features is in slide 39.

Image courtesy of: Carles Saus, MD; Hospital Son Espases, Mallorca, Spain.

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