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Medical condition
Epidermodysplasia verruciformis
Other names
Lewandowsky–Lutz dysplasia, treeman syndrome
Abul Bajandar from Bangladesh
Specialty
Dermatology
Causes
Genetic
Treatment
Surgery
Epidermodysplasia verruciformis (EV) is a skin condition characterised by warty skin lesions.[1] It results from an abnormal susceptibility to HPV infection (HPV) and is associated with a high lifetime risk of squamous cell carcinomas in skin.[1] It generally presents with scaly spots and small bumps particularly on the hands, feet, face and neck; typically beginning in childhood or in a young adult.[1] The bumps tend to be flat, grow in number and then merge to form plaques.[1] On the trunk, it typically appears like pityriasis versicolor; lesions there being slightly scaly and tan, brown, red or looking pale.[1] On the elbows, it may appear like psoriasis.[1] On the forehead, neck and trunk, the lesions may appear like seborrheic keratosis.[1]
It is most frequently inherited as an autosomal recessive trait, with some reports of autosomal dominant and X-linked inheritance.[1][2] Other types include atypical EV which develops due to gene mutations that cause an impaired immune system, and acquired EV which occurs due to acquired immunodeficiency.[2][3] It is characterized by an inability to protect against HPV infection of skin.[4][5] HPV types 5 and 8 are detected in around 90% of skin cancers in people with EV.[1] Other types are also associated with EV.[1] In rare cases, the warts may develop into giant horns resulting in treeman syndrome.[6]
Prevention of skin cancer requires sun protection.[1] Treatment typically involves surgery; sometimes with the addition of skin grafting.[1] Medications used to treat the lesions include ALA-PDT (photodynamic therapy with aminolevulinic acid), applying 5-FU, imiquimod, and retinoids by mouth.[1] The lesions tend to recur on stopping treatment.[1]
The condition is rare.[1] The lesions have been noted to occur at a younger age in warmer climates.[1] EV associated skin cancer develops less frequently in Africans.[1] The condition was first described by Felix Lewandowsky and Wilhelm Lutz in 1922.[7]
^ abcdefghijklmnopqJames, William D.; Elston, Dirk; Treat, James R.; Rosenbach, Misha A.; Neuhaus, Isaac (2020). "19. Viral diseases: epidermodysplasia verruciformis". Andrews' Diseases of the Skin: Clinical Dermatology (13th ed.). Edinburgh: Elsevier. pp. 411–412. ISBN 978-0-323-54753-6.
^ abShimizu, Akira; Yamaguchi, Reimon; Kuriyama, Yuko (March 2023). "Recent advances in cutaneous HPV infection". The Journal of Dermatology. 50 (3): 290–298. doi:10.1111/1346-8138.16697. ISSN 1346-8138. PMID 36601717. S2CID 255476033.
^Moore, Stephen; Rady, Peter; Tyring, Stephen (November 2022). "Acquired epidermodysplasia verruciformis: clinical presentation and treatment update". International Journal of Dermatology. 61 (11): 1325–1335. doi:10.1111/ijd.15857. ISSN 1365-4632. PMID 34403500. S2CID 237198699.
^Myers, David J.; Kwan, Eddie; Fillman, Eric P. (2022). "Epidermodysplasia Verruciformis". StatPearls. StatPearls Publishing. PMID 30480937.
^Mogensen, Trine H. (November 2022). "Genetic susceptibility to viral disease in humans". Clinical Microbiology and Infection. 28 (11): 1411–1416. doi:10.1016/j.cmi.2022.02.023. ISSN 1469-0691. PMID 35218976. S2CID 247114913.
^Uitto, Jouni; Saeidian, Amir Hossein; Youssefian, Leila; Saffarian, Zahra; Casanova, Jean-Laurent; Béziat, Vivien; Jouanguy, Emmanuelle; Vahidnezhad, Hassan (May 2022). "Recalcitrant Warts, Epidermodysplasia Verruciformis, and the Tree-Man Syndrome: Phenotypic Spectrum of Cutaneous Human Papillomavirus Infections at the Intersection of Genetic Variability of Viral and Human Genomes". The Journal of Investigative Dermatology. 142 (5): 1265–1269. doi:10.1016/j.jid.2021.10.029. ISSN 1523-1747. PMC 9038624. PMID 34843682.
^Emsen, IM; Kabalar, ME (2010). "Epidermodysplasia verruciformis: An early and unusual presentation". The Canadian Journal of Plastic Surgery. 18 (1): 21–4. doi:10.1177/229255031001800105. PMC 2851455. PMID 21358870. S2CID 207964064.
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85, 97 Human papillomavirus types 5 and 8 (in patients with epidermodysplasiaverruciformis) Hydrochlorothiazide 1-Hydroxyanthraquinone Indeno[1,2,3-cd]pyrene...
flat, plantar and anogenital warts, laryngeal papillomas, epidermodysplasiaverruciformis) Malignancies for some species (cervical carcinoma, squamous...