American Journal of Medical Case Reports, 2014, Vol. 2, No. 9, 187-193 Available online at http://pubs.sciepub.com/ajmcr/2/9/7 © Science and Education Publishing DOI:10.12691/ajmcr-2-9-7 Epidermodysplasia Verruciformis Associated with Astrocytoma, Mantle Lymphoma and Hepatitis B Virus Infection Mahmoud Ibrahim Yousef Elbadry * , Ahmed Othman Ahmed Emam Department of Internal Medicine, Sohag Faculty of Medicine, Sohag University, Sohag, Egypt *Corresponding author: mahmoudibrahem83@yahoo.com Received September 05, 2014; Revised September 16, 2014; Accepted September 22, 2014 Abstract Epidermodysplasia verruciformis (EV) is a lifelong, rare autosomal recessive genetic hereditary skin disorder characterized by a unique susceptibility to human papilloma virus, associated with a high risk of malignant transformation. The disseminated verrucous lesions and pityriasis versicolor-like lesions persist from early childhood and can transform into a cutaneous malignancy in a fourth of patients. No definitive treatment against Epidermodysplasia verruciformis has been found yet. Extracutaneous cancers reported in Epidermodysplasia verruciformis are intestinal adenocarcinoma, plasmablastic lymphoma and leiomyosarcoma. Mantle cell lymphoma is uncommon under the age of 40 years and rare in patients under the age of 30 years. Here we are reporting a 19 year male patient with Epidermodysplasia verruciformis, astrocytoma and pulmonary tuberculosis who presented with symptoms of weight loss, indigestion and burning epigastric pain four months duration, Upper GIT endoscopy showed a gastric mass with active peptic ulcers. Biopsy revealed Mantle cell lymphoma. We investigated further and found to have immunodeficiency and hepatitis B virus infection. EV patients should receive regular follow-up for possible cutaneous or extracutaneous malignancy. Keywords: Epidermodysplasia verruciformis, Astrocytoma, Lymphoma, Mantle-Cell, Human papillomavirus DNA Tests, Hepatitis B virus Cite This Article: Mahmoud Ibrahim Yousef Elbadry, and Ahmed Othman Ahmed Emam, “Epidermodysplasia Verruciformis Associated with Astrocytoma, Mantle Lymphoma and Hepatitis B Virus Infection.” American Journal of Medical Case Reports, vol. 2, no. 9 (2014): 187-193. doi: 10.12691/ajmcr-2-9-7. 1. Background Lewandowsky and Lutz dysplasia which also called as Epidermodysplasia verruciformis (EV) is an extremely rare autosomal recessive genetic hereditary skin disorder associated with a high risk of malignant transformation [1]. It has no racial or geographic predilection, but increased incidence in consanguineous marriages [2]. It is characterized by abnormal susceptibility to human papilloma viruses (HPVs) of the skin [3]. The resulting uncontrolled HPV infections result in the growth of scaly macules and papules, particularly on the hands and feet. It is typically associated with HPV types 5 and 8 which are found in about 80 percent of the normal population as asymptomatic infections [4,5]. The condition usually has an onset between the ages of 1–20, but can occasionally present in middle-age without gender predisposition [6]. It is named after the physicians who first documented it, Felix Lewandowsky and Wilhelm Lutz [7]. Carcinogenic cofactors, such as ultraviolet ray irradiation, decreased the cell mediated immunity and impaired DNA repair are probably involved in the progression from benign warts (verrucae) to cancer [8,9,10,11]. Malignant transformation of skin lesions, particularly squamous cell carcinoma has been observed in more than half the patients followed for 20-30 years. Malignant tumors are typically found after age 30 years, usually during the fourth and fifth decades of life [12,13]. No definitive treatment for EV has been found yet. Treatment of EV includes preventive measures, the most important of which are the strict sun protection and life- long observation for early diagnosis of malignant/ premalignant lesions for improved survival. Acitretin (0.5- 1 mg/ day) is the effective and the palliative drug of choice [14]. We report this young patient who had Astrocytoma, pulmonary tuberculosis, Mantle cell lymphoma, Hepatitis B Virus infection and immunodeficiency after the previous diagnoses of EV represent an interesting uncommon occurrence of these diseases together. 2. Case Report A 19-year-old orphan boy, born to second-degree consanguineous parents, he had been well until 6 years of age when his mother found multiple asymptomatic dark colored and light colored lesions over the neck and trunk. At the age of 8 years, a prominent, persistent dark raised