eMedicine Specialties > Dermatology > Viral Infections

Epidermodysplasia Verruciformis: Follow-up

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

Follow-up

Complications

  • Malignant transformation of skin lesions has been observed in more than one half of the patients followed up for 20-30 years. Malignant tumors are typically found after age 30 years, usually during the fourth and fifth decades of life.

Prognosis

  • EV tumors evolve progressively, from childhood through adolescence, to adulthood.
  • Fatality due to metastasizing invasive squamous cell carcinoma arising in conjunctiva has been occasionally reported.11

Patient Education

Miscellaneous

Medicolegal Pitfalls

  • Failure to perform a skin biopsy for early detection of premalignant and malignant skin lesions and for the identification of EV-associated HPVs is a pitfall.
 


More on Epidermodysplasia Verruciformis

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

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  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].

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Further Reading

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Keywords

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

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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.

 
 
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