Conjunctival neuromas and prominent corneal nerves as first symptoms of MEN 2B-syndrome

Background: Multiple endocrine neoplasias (MEN-syndromes) represent diverse , mostly autosomal-dominant inherited, seldom sporadic diseases. MEN 2B-syn drome comprises medullary thyroid carcinoma, pheochromocytoma and mucosal n euromas. Patient: A 30 year old male patient presented with bilateral chronic irrita tion of the ocular surface. The biomicroscopy revealed intraconjunctival wo rm-like alterations, prominent corneal nerves and nodules on the upper lid margins. Histology: The performed conjunctival biopsy showed nearly no goblet cells, but prominent intrastromal, immunohistochemical S100-positive neuromas. Results: The initiated investigations revealed bilateral multifocal medulla ry thyroid carcinoma and a left sited pheochromocytoma. Conclusion: Conjunctival neuromas and prominent corneal nerves can be diagn ostic for the MEN 2B-syndrom. Early diagnosis and prophylactic thyreoidecto my are expected to improve the life expectancy even in asymptomatic gene-ca rriers.