Epidermodysplasia verruciformis: a rare case presentation
Keywords:
Epidermodysplasia verruciformis, human papilloma virusAbstract
Epidermodysplasia verruciformis (EV) is a rare genodermatosis characterized by a unique susceptibility to cutaneous infection by a group of phylogenetically related human papilloma viruses (HPVs). These patients show a defect in cell-mediated immunity specific toward the causative HPVs that leads to lifelong disease. The defect is usually inherited as autosomal recessive trait and presents clinically with plane warts, pityriasis versicolor-like lesions and reddish verrucous plaques. Dysplastic and malignant changes in the form of actinic keratoses, Bowen's disease and squamous cell carcinoma (SCC) are common but metastasis occurs rarely. A totally effective treatment against EV is as yet highly desirable. We report a case of EDV in a 13-year-old female patient with confluent wart like lesions, pityriasis versicolor like lesions and reddish plaques. The case is being reported in view of rarity of disease.References
Garcνa-Rνo I, Garcia-F-Villalta MJ, Daudιn E, Fraga J, Garcνa-Dνez A. Epidermodysplasia verruciformis-like lesions in a patient with systemic lupus erythematosus. Acta Derm Venereol. 2003;83:229-30.
Ostrow RS, Manias D, Mitchell AJ, Stawowy L, Faras AJ. Epidermodysplasia verruciformis. A case with primary lymphatic dysplasia, depressed cell-mediated immunity, and Bowen's disease containing human papilloma 16 DNA. Arch Dermatol. 1987;123:1511-6.
Pfister H. Human papilloma viruses and impaired immunity vs epidermodysplasia verruciformis. Arch Dermatol. 1987;123:1469-70.
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. 2001;45:296-9.
Rallis E, Paparizos V, Kyriakis K, Katsambas A. Treatment of epidermodysplasia verruciformis in human immunodeficiency virus-positive patients. J Eur Acad Dermatol Venereol. 2009;23:195-6.
Hayashi J, Matsui C, Mitsuishi T et al. Treatment of localized epidermodysplasia verruciformis with tacalcitol ointment. Int J Dermatol. 2002;41:817-20.
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. 2007;56:882-6.
Ishiji T, Ibe M, Kawase M, Niimura M. Patients with epidermodysplasia verruciformis show no response to contact immunotherapy. Dermatology. 2001;202:76-7.
Micali G, Nasca MR, Dall’Oglio F, Musumeci ML. Cimetidine therapy for epidermodysplasia verruciformis. J Am Acad Dermatol. 2003;48:S9-10.
de Oliveira WR, Neto CF, Rivitti EA. The lack of a clinical effect of cimetidine in the treatment of epidermodysplasia verruciformis. J Am Acad Dermatol. 2004;50:e14.
