First report of oral angiokeratoma in a xeroderma pigmentosum

INTRODUCTION AND IMPORTANCE: Xeroderma pigmentosum is an autosomal recessive genetic disorder with impaired ability to repair DNA damage. Detection of the intraoral benign and malignant lesion in Xeroderma pigmentosum is uncommon, and the reported few cases were in the lip and tip of the tongue which are related to ultraviolet light exposure. CASE PRESENTATION: We present a case of 20 years old male with erythematous to violet, painless and shiny papule at the right side of the tongue. The lesion was surgically excised while the histopathological examination of the lesion showed focally eroded and hyperplastic stratified squamous epithelial covering with underlying capillary sized blood vessel filled with RBCs and lined by endothelium with no atypia or malignancy. CLINICAL DISCUSSION: Oral angiokeratoma is a rare disease in patient with Xeroderma pigmentosum that should be early detected and diagnosed to decrease the liability for malignant transformation. CONCLUSION: Health case workers should be aware of the nature of the disease, protection of the patient from ultraviolet light as well as Frequent follow up of the patients for a better quality of life.