To the Editor: A case report in the Journal (N Engl J Med 284:963–964, 1971) described a patient with multiple neurofibromas, metastatic medullary carcinoma of the thyroid gland, bilateral pheochromocytomas and the characteristic physical appearance of this syndrome. Dr. Baum, in addition, noted absence of a flare response to intradermal histamine, ectopic lacrimal puncta, an abnormal Schirmer tests. A letter in the Journal (N Engl J Med 284:983, 1971) by Gorlin confirmed the absence of the flare response in patients with thyroid medullary carcinoma, probably because of elevated histaminase levels. We have been following a 16-year-old boy with the syndrome ….
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