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July 1975

Multiple Mucosal Neuroma Syndrome-Reply

Author Affiliations

New Haven, Conn

Arch Dermatol. 1975;111(7):931. doi:10.1001/archderm.1975.01630190121032

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To the Editor.—  The association of thyroid carcinoma and pheochromocytoma was first described in the American literature by Eisenberg in 1932; Sipple redirected attention to this disorder in 1961. It is unfortunate that in most early studies, family histories and other related findings were neither undertaken nor recognized. It has not been until recent years that mucosal neuromas, hyperparathyroidism, eye disorders, Cushing syndrome, von Recklinghausen disease, diabetes mellitus, and pigmentary disorders have been noted in association with medullary thyroid carcinoma and pheochromocytoma.Terms associated with variations of the aforementioned constellation of findings include the Sipple syndrome (medullary thyroid carcinoma and pheochromocytoma), multiple endocrine neoplasia type II (medullary thyroid carcinoma, pheochromocytoma, Cushing disease, and multiple mucosal neuromas), and the medullary thyroid carcinoma syndrome. The multiple mucosal neuroma syndrome (MMNS) includes neuromas of the lips, tongue, eyelids, and intestinal mucosa (with or without medullary thyroid carcinoma, depending upon the author describing the

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