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An Atrophic, Telangiectatic Patch at the Distal Border of the Tongue: A Mucous Membrane Manifestation of Xeroderma Pigmentosum
Author(s) -
Bologna Sheyla Batista,
Harumi Nakajima Teshima Tathyane,
Lourenço Silvia Vanessa,
Nico Marcello Menta Simonsen
Publication year - 2014
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/pde.12272
Subject(s) - tongue , xeroderma pigmentosum , medicine , mucocutaneous zone , lesion , pyogenic granuloma , pathology , dermatology , telangiectasia , tongue neoplasm , basal cell , dna repair , biology , disease , biochemistry , gene
Xeroderma pigmentosum ( XP ) is a rare autosomal recessive disorder characterized by clinical and cellular sensitivity, pigmentary changes, and early development of malignancies in sun‐exposed mucocutaneous and ocular structures due to a defective ability to repair intracellular DNA damage. Individuals with XP also have a greater frequency of oral cancer, particularly squamous cell carcinoma of the anterior third of the tongue. The current study reports four cases of XP that exhibited a characteristic crescent‐shaped, atrophic, telangiectatic area on the distal border of the tongue and correlates this lesion with the development of tumors at this site during follow‐up. The tongue lesion was photographed and biopsied in the four patients. During routine follow‐up visits, new biopsies were performed if additional tongue lesions were observed. The studied lesions were similar in the four patients. During follow‐up, squamous cell carcinoma developed in one patient and pyogenic granuloma developed in three patients and was relapsing in one. The lesion remained stable in one patient during the study. The atrophic and telangiectatic patches probably occur because of chronic sun damage to the exposed portion of the tongue, and this area has a high predisposition for the development of benign and malignant tumors.