Cutaneous neuropathology in multiple endocrine neoplasia, type 2b

Increase in masses of mixed (myelinated and nonmyelinated) nerve are observed in the dermis of clinically normal skin of patients with multiple endocrine neoplasia, type 2b (MEN 2b). Preliminary histochemical studies revealed nucleoside triphosphatase and nonspecific acid esterase. Electron microscopy showed axonal organelles, mild degeneration of axons, and numerous axons unassociated with Schwann cells. The normal-appearing skin in MEN 2b seems to contain abnormal nerve tissue development equally with the mucosal and the gastrointestinal tract.