Paraneoplastic Autoimmune Multiorgan Syndrome in a Patient With Li Fraumeni Syndrome | Dermatology | JAMA Dermatology | JAMA Network
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Observation
September 2013

Paraneoplastic Autoimmune Multiorgan Syndrome in a Patient With Li Fraumeni Syndrome

Author Affiliations
  • 1Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota
  • 2Division of Clinical Dermatology, Mayo Clinic, Rochester, Minnesota
JAMA Dermatol. 2013;149(9):1116-1117. doi:10.1001/jamadermatol.2013.4982

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).

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