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March 2010

Multiple Facial Papules and Macrocephaly—Quiz Case

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Copyright 2010 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.2010

Arch Dermatol. 2010;146(3):337-342. doi:10.1001/archdermatol.2010.8-a

A 32-year-old white woman presented with countless small papules on her face and ears that had gradually increased in number over the years. Her medical history included Hodgkin disease at the age of 18 years, a benign thyroid follicular tumor at the age of 20 years, and invasive breast cancer at the age of 29 years. She denied any family history of thyroid, breast, or uterine disease. Physical examination revealed hundreds of pinpoint, skin-colored papules on the central part of her face and ears (Figure 1), palmar and plantar keratoses, and a cobblestone appearance to the tongue as a result of multiple papillomas (Figure 2). She was macrocephalic, with a head circumference in the 99th percentile. A shave biopsy specimen was obtained from her ear for microscopic examination (Figure 3).