Fabry Disease – Hypohidrosis / Anhidrosis

Impaired sweating, either hypohidrosis or anhidrosis, usually presents in childhood or adolescence and has been attributed to selective peripheral nerve damage or to intracytoplasmic lipid deposits in the small blood vessels surrounding sweat glands. ^{[1, 2]} This may result in dry skin and heat and exercise intolerance. In approximately 40% of Fabry disease patients, production of tears and saliva is also reduced.^[3]

References

1. Ohnishi A, Dyck PJ. Loss of small peripheral sensory neurons in Fabry disease. Arch Neurol. 1974;31:120-126.

2. Lao LM, Kumakiri M, Mima H, et al. The ultrastructural characteristics of eccrine sweat glands in a Fabry disease patient with hypohidrosis. J Dermatol Sci. 1998;18:109-117.

3. Cable WJL, Kolodny EH, Adams RD. Fabry disease: impaired autonomic function. Neurology. 1982;32:498-502.