Early childhood–onset mucosal neuromas of the lips, tongue, and eyelids are pathognomonic for multiple endocrine neoplasia (MEN) 2b syndrome, which is also associated with medullary thyroid carcinoma and pheochromocytoma. It has recently been suggested that multiple mucocutaneous neuromas may also represent an early manifestation of PTEN hamartoma-tumor syndrome (PHTS), including Cowden syndrome and others.1,2 Mucocutaneous neuromas in PHTS appear in early childhood and characteristically involve acral sites.1,2
Misago N, Joh K, Soejima H, Narisawa Y. The Possibility That Multiple Mucocutaneous (Palisaded Encapsulated and Nonencapsulated) Neuromas May Be a Distinct Entity. JAMA Dermatol. 2013;149(4):498–500. doi:10.1001/jamadermatol.2013.2153
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