eMedicine Specialties > Dermatology > Viral Infections

Epidermodysplasia Verruciformis: Multimedia

Author: Grace F Kao, MD, Clinical Professor of Dermatopathology, Department of Dermatology, George Washington University Medical School; Director of Dermatopathology, Department of Pathology and Laboratory Medicine, Veterans Affairs Maryland Healthcare System
Contributor Information and Disclosures

Updated: Nov 13, 2007

Multimedia

Eruptive, polymorphic, warty papules and plaques ...Media file 1: Eruptive, polymorphic, warty papules and plaques on the lower extremities of a patient with epidermodysplasia verruciformis. Courtesy of Professor S. Jablonska and Professor S. Majewski.
Eruptive, polymorphic, warty papules and plaques ...

Eruptive, polymorphic, warty papules and plaques on the lower extremities of a patient with epidermodysplasia verruciformis. Courtesy of Professor S. Jablonska and Professor S. Majewski.

Left: Photomicrograph of a precancerous, verrucou...Media file 2: Left: Photomicrograph of a precancerous, verrucous skin lesion from a patient with epidermodysplasia verruciformis depicts the characteristic microscopic features of specific cytopathic effect, that is, the presence of clear cells and an occasional enlarged, hyperchromatic, atypical nucleus (center of the field) in the epidermis. These changes are seen in human papillomavirus (HPV)-associated epithelial lesions (hematoxylin-eosin stain, original magnification X250). Right: Photomicrograph of the same skin lesion shows positive staining of keratinocytes infected with HPV type 8 (in situ hybridization, original magnification X250). Note darker, spherical-to-ovoid shaped positive nuclear staining. These are sites of HPV DNA.
Left: Photomicrograph of a precancerous, verrucou...

Left: Photomicrograph of a precancerous, verrucous skin lesion from a patient with epidermodysplasia verruciformis depicts the characteristic microscopic features of specific cytopathic effect, that is, the presence of clear cells and an occasional enlarged, hyperchromatic, atypical nucleus (center of the field) in the epidermis. These changes are seen in human papillomavirus (HPV)-associated epithelial lesions (hematoxylin-eosin stain, original magnification X250). Right: Photomicrograph of the same skin lesion shows positive staining of keratinocytes infected with HPV type 8 (in situ hybridization, original magnification X250). Note darker, spherical-to-ovoid shaped positive nuclear staining. These are sites of HPV DNA.

Large amounts of human papillomavirus (HPV) DNA a...Media file 3: Large amounts of human papillomavirus (HPV) DNA are demonstrated by immunostaining a skin biopsy of a warty lesion of epidermodysplasia verruciformis. Note prominent vacuolation of the cytoplasm of the infected cells (koilocytosis), typical of lesions associated with HPV infection. The darker positive staining areas are the sites of HPV DNA (in situ hybridization, original magnification X450).
Large amounts of human papillomavirus (HPV) DNA a...

Large amounts of human papillomavirus (HPV) DNA are demonstrated by immunostaining a skin biopsy of a warty lesion of epidermodysplasia verruciformis. Note prominent vacuolation of the cytoplasm of the infected cells (koilocytosis), typical of lesions associated with HPV infection. The darker positive staining areas are the sites of HPV DNA (in situ hybridization, original magnification X450).

A photomicrograph shows an invasive well-differen...Media file 4: A photomicrograph shows an invasive well-differentiated squamous cell carcinoma that arose in a warty lesion on sun-exposed skin of a middle-aged patient with epidermodysplasia verruciformis. Notice the atypical, neoplastic squamous cancer cells with irregular, hyperchromatic nuclei and an occasional bizarre mitotic figure (shown near the 12-o'clock position in this field) invading into the dermis. A moderate host lymphocytic inflammatory response is present within the tumor (hematoxylin-eosin stain, original magnification X300). Squamous cell carcinoma is the most common type of skin cancer found in patients with epidermodysplasia verruciformis.
A photomicrograph shows an invasive well-differen...

A photomicrograph shows an invasive well-differentiated squamous cell carcinoma that arose in a warty lesion on sun-exposed skin of a middle-aged patient with epidermodysplasia verruciformis. Notice the atypical, neoplastic squamous cancer cells with irregular, hyperchromatic nuclei and an occasional bizarre mitotic figure (shown near the 12-o'clock position in this field) invading into the dermis. A moderate host lymphocytic inflammatory response is present within the tumor (hematoxylin-eosin stain, original magnification X300). Squamous cell carcinoma is the most common type of skin cancer found in patients with epidermodysplasia verruciformis.

More on Epidermodysplasia Verruciformis

Overview: Epidermodysplasia Verruciformis
Differential Diagnoses & Workup: Epidermodysplasia Verruciformis
Treatment & Medication: Epidermodysplasia Verruciformis
Follow-up: Epidermodysplasia Verruciformis
Multimedia: Epidermodysplasia Verruciformis
References

References

  1. Androphy EJ, Dvoretzky I, Lowy DR. X-linked inheritance of epidermodysplasia verruciformis. Genetic and virologic studies of a kindred. Arch Dermatol. Jul 1985;121(7):864-8. [Medline].

  2. Gober MD, Rady PL, He Q, Tucker SB, Tyring SK, Gaspari AA. Novel homozygous frameshift mutation of EVER1 gene in an epidermodysplasia verruciformis patient. J Invest Dermatol. Apr 2007;127(4):817-20. [Medline].

  3. Sun XK, Chen JF, Xu AE. A homozygous nonsense mutation in the EVER2 gene leads to epidermodysplasia verruciformis. Clin Exp Dermatol. Sep 2005;30(5):573-4. [Medline].

  4. de Oliveira WR, Rady PL, Grady J, Hughes TK, Festa Neto C, Rivitti EA, et al. Polymorphisms of the interleukin 10 gene promoter in patients from Brazil with epidermodysplasia verruciformis. J Am Acad Dermatol. Oct 2003;49(4):639-43. [Medline].

  5. Morrison C, Eliezri Y, Magro C, Nuovo GJ. The histologic spectrum of epidermodysplasia verruciformis in transplant and AIDS patients. J Cutan Pathol. Sep 2002;29(8):480-9. [Medline].

  6. Vu J, Wallace GR, Singh R, Diwan H, Prieto V, Rady P, et al. Common variable immunodeficiency syndrome associated with epidermodysplasia verruciformis. Am J Clin Dermatol. 2007;8(5):307-10. [Medline].

  7. Berthelot C, Dickerson MC, Rady P, He Q, Niroomand F, Tyring SK, et al. Treatment of a patient with epidermodysplasia verruciformis carrying a novel EVER2 mutation with imiquimod. J Am Acad Dermatol. May 2007;56(5):882-6. [Medline].

  8. Anadolu R, Oskay T, Erdem C, Boyvat A, Terzi E, Gürgey E. Treatment of epidermodysplasia verruciformis with a combination of acitretin and interferon alfa-2a. J Am Acad Dermatol. Aug 2001;45(2):296-9. [Medline].

  9. Gubinelli E, Posteraro P, Cocuroccia B, Girolomoni G. Epidermodysplasia verruciformis with multiple mucosal carcinomas treated with pegylated interferon alfa and acitretin. J Dermatolog Treat. Sep 2003;14(3):184-8. [Medline].

  10. Majewski S, Skopinska M, Bollag W, Jablonska S. Combination of isotretinoin and calcitriol for precancerous and cancerous skin lesions. Lancet. Nov 26 1994;344(8935):1510-1. [Medline].

  11. Partridge ME, Pariser RJ. Ocular and cutaneous squamous cell carcinoma in an African American man with epidermodysplasia verruciformis resulting in blindness and death. J Am Acad Dermatol. Nov 2003;49(5 Suppl):S262-4. [Medline].

  12. Azzimonti B, Mondini M, De Andrea M, Gioia D, Dianzani U, Mesturini R, et al. CD8+ T-cell lymphocytopenia and lack of EVER mutations in a patient with clinically and virologically typical epidermodysplasia verruciformis. Arch Dermatol. Oct 2005;141(10):1323-5. [Medline].

  13. de Koning M, Struijk L, Feltkamp M, ter Schegget J. HPV DNA detection and typing in inapparent cutaneous infections and premalignant lesions. Methods Mol Med. 2005;119:115-27. [Medline].

  14. Deau MC, Favre M, Orth G. Genetic heterogeneity among human papillomaviruses (HPV) associated with epidermodysplasia verruciformis: evidence for multiple allelic forms of HPV5 and HPV8 E6 genes. Virology. Oct 1991;184(2):492-503. [Medline].

  15. Harris AJ, Purdie K, Leigh IM, Proby C, Burge S. A novel human papillomavirus identified in epidermodysplasia verruciformis. Br J Dermatol. Apr 1997;136(4):587-91. [Medline].

  16. James W, Berger T, Elston D. Andrews' Diseases of the Skin: Clinical Dermatology. 10th ed. Amsterdam: Elsevier; 2006.

  17. Joblonska S. Epidermodysplasia verruciformis. In: Friedman RJ, ed. Skin Cancers. Philadelphia, Pa: WB Saunders; 1991:101-13.

  18. Kao G, et al. Cutaneous carcinogenesis: Etiologic Factors-Viruses. In: Miller S, Mahoney M, eds. Cutaneous Oncology: Pathophysiology, Diagnosis, and Treatment. London, England: Blackwell Science; 1997:148-57.

  19. Kunishige JH, Hymes SR, Madkan V, Wyatt AJ, Uptmore D, Lazar AJ, et al. Epidermodysplasia verruciformis in the setting of graft-versus-host disease. J Am Acad Dermatol. Nov 2007;57(5 Suppl):S78-80. [Medline].

  20. Lane JE, Bowman PH, Cohen DJ. Epidermodysplasia verruciformis. South Med J. Jun 2003;96(6):613-5. [Medline].

  21. Lane JE, Bowman PH, Cohen DJ. Epidermodysplasia verruciformis. South Med J. Jun 2003;96(6):613-5. [Medline].

  22. Lutzner MA, Blanchet-Bardon C, Orth G. Clinical observations, virologic studies, and treatment trials in patients with epidermodysplasia verruciformis, a disease induced by specific human papillomaviruses. J Invest Dermatol. Jul 1984;83(1 Suppl):18s-25s. [Medline].

  23. Majewski S, Jablonska S. Epidermodysplasia verruciformis as a model of human papillomavirus-induced genetic cancer of the skin. Arch Dermatol. Nov 1995;131(11):1312-8. [Medline].

  24. Mitsuishi T, Kawana S, Kato T, Kawashima M. Human papillomavirus infection in actinic keratosis and bowen's disease: comparative study with expression of cell-cycle regulatory proteins p21(Waf1/Cip1), p53, PCNA, Ki-67, and Bcl-2 in positive and negative lesions. Hum Pathol. Sep 2003;34(9):886-92. [Medline].

  25. Nuovo GJ, Ishag M. The histologic spectrum of epidermodysplasia verruciformis. Am J Surg Pathol. Oct 2000;24(10):1400-6. [Medline].

  26. Ortak T, Uysal AC, Alagoz MS, Orbay H, Sensoz O. Epidermodysplasia verruciformis: an unusual presentation. Dermatol Surg. Feb 2006;32(2):302-6. [Medline].

  27. Orth G, Favre M, Majewski S, Jablonska S. Epidermodysplasia verruciformis defines a subset of cutaneous human papillomaviruses. J Virol. May 2001;75(10):4952-3. [Medline].

  28. Ramoz N, Rueda LA, Bouadjar B, Montoya LS, Orth G, Favre M. Mutations in two adjacent novel genes are associated with epidermodysplasia verruciformis. Nat Genet. Dec 2002;32(4):579-81. [Medline].

Further Reading

Keywords

EV, human papillomavirus, human papillomavirus infection, HPV, HPV infection, squamous cell carcinoma

Contributor Information and Disclosures

Author

Grace F Kao, MD, Clinical Professor of Dermatopathology, Department of Dermatology, George Washington University Medical School; Director of Dermatopathology, Department of Pathology and Laboratory Medicine, Veterans Affairs Maryland Healthcare System
Grace F Kao, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and International Society of Dermatopathology
Disclosure: Nothing to disclose.

Medical Editor

Kathryn Schwarzenberger, MD, Associate Professor of Medicine, Division of Dermatology, University of Vermont College of Medicine; Consulting Staff, Division of Dermatology, Fletcher Allen Health Care
Kathryn Schwarzenberger, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Contact Dermatitis Society, American Dermatological Association, Dermatology Foundation, Medical Dermatology Society, and Women's Dermatologic Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Lester F Libow, MD, Dermatopathologist, South Texas Dermatopathology Laboratory
Lester F Libow, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, European Academy of Dermatology and Venereology, International Society of Dermatology, Massachusetts Medical Society, New York Academy of Sciences, Phi Beta Kappa, Society for Investigative Dermatology, and Texas Medical Association
Disclosure: Nothing to disclose.

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.