P260 : Lentigo maligna in a patient with xeroderma pigmentosum, variant type: a case report

Xeroderma Pigmentosum (XP) is a rare autosomal recessive disorder. XP patients usually develop multiple malignant skin tumors. Therefore, early diagnosis of skin tumors is crucial. However, extensive actinic damages and subsequent multiple pigmented lesions in XP patients make the differentiation of malignant lesion from benign pigmented spots difficult. A 58-year-old Korean woman presented with significant pigmentary changes on her skin. An irregularly shaped brown pigmented patch on her right cheek was observed. Dermoscopic findings suggested lentigo maligna. Histologically, atypical melanocytic proliferation with a pagetoid spreading of melanocytes was detected, confirming the diagnosis of lentigo maligna. We performed surgical removal of the lesion. Since the patient’s excessive pigmentary changes make us suspect a diagnosis of XP, targeted panel sequencing was done. The test was consistent with the findings of XP variant type. In this report, we report a rare case of XP variant type with lentigo maligna.