[Recklinghausen's disease with digestive localizations associated with gastric acid hypersecretion suggesting...

We report the case of a 49 year old male patient who had Recklinghausen's disease associated with hyperchlorhydria. The principal features of Recklinghausen's disease were cutaneous localizations and countless digestive tumors, found mainly on the small bowel. The ultrastructural aspect of these tumors was neurinoma or schwannoma. Basal acid gastric hypersecretion and a positive secretin test were highly suggestive of a Zollinger-Ellison syndrome. Gastrinoma was not found at laparotomy even though a gastrin gradient had been demonstrated by pancreatic venous sampling. In a patient with Recklinghausen's disease, neuroendocrine tumors (APUDomas) should be looked for systematically.