Cutaneous innervation in hereditary sensory and autonomic neuropathy type IV

The authors investigated immunocytochemically the innervation of a skin bio psy in a rare case of hereditary sensory and autonomic neuropathy type IV. A few protein gene product 9.5-, growth-associated protein 43-, calcitonin gene-related peptide-, and substance P-immunoreactive nerve fibers were obs erved in the deeper regions of the dermis. Neuropeptide Y-, nitric oxide-, and vasoactive intestinal polypeptide-immunoreactive fibers were completely absent. Their observations support the hypothesis that the sensory and aut onomic defects reported in hereditary sensory and autonomic neuropathy are based on profound developmental alterations of the peripheral nervous syste m.