Fabry's disease without angiokeratomas showing unusual eccrine gland vacuolation
Magazine: The Journal of Pathology
Date: May 1, 1992Pathological Anatomy [SP]
An unusual case of Anderson-Fabry's disease with renal damage and bilateral corneal deposits but without angiokeratomas is presented.
Diagnosis was made by renal biopsy. Typical foamy transformation of endothelium, mesangium, tubular epithelial cells, vascular smooth muscle, and fibroblasts of kidney biopsy and similar changes of myoepithelial, perineural, and endothelial cells on the skin biopsy were observed. At electron microscopy, 'fingerprint-like' myelin figures were seen. The epithelial cells of eccrine sweat glands showed striking vacuolation but myelin figures were not seen.
We suggest that electron microscopic study of skin biopsies is indicated in the evaluation of patients clinically suspected of having Fabry's disease.
CITATION J Pathol. 1992 May;167(1):65-8
you mayBE INTERESTED
The Clínica is the greater private hospital with technological equipment of Spain, all in a single center.
The professionals of the Clínica perform continuous research and training, always to the benefit of the patient.
Learn why we are different from other healthcare centers. Quality, speed, comfort and results.