Compensatory alterations in dermal innervations in patients with congenital insensitivity to pain

Jan Minde, Olle Svensson, Göran Toolanen and Sol-Britt Lonne-Rahm*

Context: The purpose of this study was to determine whether the expression of sensory neuropeptides, NK1, 5-HT1A receptors, as well as mast cells in the skin of patients with hereditary neuropathy and sensory and autonomic deficits (HSAN type 5) was elevated. Such increase might reflect an attempt to compensate for nerve loss.

Materials and methods: Six patients with HSAN type 5, three of which were heterozygous and three were homozygous and examined whether there were any compensatory mechanisms in the skin cells to prevent tissue damage.

We compared the innervation of nerve fibres and mast cells in skin biopsies from the arms and legs of these patients and compared these to biopsies from healthy control individuals.

Results: Both types of patient groups showed reduced cutaneous nerve fibres and the existence of the sensory neuropeptide substance P in the skin. In the homozygous patients, the Neurokinin 1 (NK1) Receptor(R) was present at a higher level in the dermis of the legs than in both the heterozygous patients and in the healthy controls. 

In addition, cells staining positively for serotonin (5-HT; 5-hydroxytryptamin) as well as 1AR and tryptase positive cells (mast cells) were more frequent in the patients compared to controls.

Conclusion: Thus, increase in the number of cells that express the receptors for NK1 and 5-HT1A, along with tryptase positive cells, may be associated with the severely reduced number of nerve fibres observed in our patients.

Keywords:

Published on: Dec 18, 2019 Pages: 22-26

Full Text PDF Full Text HTML DOI: 10.17352/2640-7876.000018
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