AI Article Synopsis
- - Epidermodysplasia verruciformis (EDV) is a rare genetic disorder that makes individuals more vulnerable to certain skin infections caused by the human papilloma virus (HPV) and is inherited in an autosomal recessive manner.
- - First identified in 1922, EDV can cause chronic skin lesions that resemble either pityriasis versicolor or flat warts, with the latter having a significant risk of transforming into skin cancers later in life.
- - About 35%-50% of patients may develop malignancies, particularly Bowen disease and invasive squamous cell carcinoma, usually emerging after the age of 40, although the spread of these cancers to other body parts is uncommon.
Article Abstract
Epidermodysplasia verruciformis (EDV) is a rare, autosomal recessive, genetic disorder of immune dysregulation characterized by increased susceptibility to cutaneous human papilloma virus infections. It was first described by Lewandowsky and Lutz in 1922 as a form of epidermal nevus. Its association with skin cancers was proposed by Sullivan and Ellis in 1939. Initial lesions often occur in childhood and are of two types; lifelong eruptions of pityriasis versicolor like lesions and flat wart like papules. The latter is characterized by malignant transformation in 35%-50% of patients especially after 40-50 years of age. Bowen disease is the most common tumor followed by invasive squamous cell carcinoma, however, metastasis is rarely encountered.
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| http://dx.doi.org/10.4103/ijmy.ijmy_19_21 | DOI Listing |
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