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April 2013

The Possibility That Multiple Mucocutaneous (Palisaded Encapsulated and Nonencapsulated) Neuromas May Be a Distinct Entity

Author Affiliations

Author Affiliations: Department of Internal Medicine, Division of Dermatology (Drs Misago and Narisawa), and Department of Biomolecular Sciences, Division of Molecular Genetics & Epigenetics (Drs Joh and Soejima), Faculty of Medicine, Saga University, Saga, Japan.

JAMA Dermatol. 2013;149(4):498-500. doi:10.1001/jamadermatol.2013.2153

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