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Treatment of paraneoplastic autoimmune multiorgan syndrome (PAMS) can be challenging; a p53 tumor suppressor mutation adds a further layer of complexity.
A 30-year-old African American woman with Li-Fraumeni syndrome was evaluated for a 4-month history of oral ulcerations. Her history was significant for adrenal carcinoma at age 3 years, undifferentiated leukemia at age 12 years, and breast cancer at age 18 years. Her oral ulcerations were painful causing difficulty eating and weight loss. Previous treatment with prednisone, 20 mg/d, provided limited relief.
On examination she had ulcerations affecting her labial, gingival, lingual, and buccal mucosae with no involvement of the skin or conjunctiva (Figure, A).
O'Gorman SM, Torgerson R. Paraneoplastic Autoimmune Multiorgan Syndrome in a Patient With Li Fraumeni Syndrome. JAMA Dermatol. 2013;149(9):1116–1117. doi:10.1001/jamadermatol.2013.4982
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