The ultrastructural characteristics of eccrine sweat glands in a Fabry disease patient with hypohidrosis.
J Dermatol Sci
; 18(2): 109-17, 1998 Nov.
Article
en En
| MEDLINE
| ID: mdl-9833977
ABSTRACT
As an investigation of the pathogenetic mechanism of diminished sweating in Fabry disease, an electron microscopy ultrastructural study was conducted on specimens of eccrine sweat glands from a typical patient with Fabry disease who had hypohidrosis, a low skin moisture content, and diminished thermoregulation ability. Numerous characteristic cytoplasmic inclusions were observed in the eccrine sweat glands, the lamellar pattern of which was considerably variable in various types of gland cells. Large vacuolar inclusions predominated in clear cells of secretory coil; lesser vacuoles were also seen in the coiled duct, and the basal cells of the straight duct toward the coiled duct displayed mulberry-like figures. There were some clear cells showing cell damage and necrosis in the secretory coil. Lamellated inclusions were noted in the unmyelinated axons innervating the eccrine sweat glands. The small blood vessels around the eccrine glands were narrowed by swollen endothelial cells with heavy inclusions. These intracytoplasmic deposits may be responsible for the decreased sweating ability in Fabry disease. The factors related to hypohidrosis are also discussed.
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Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Enfermedad de Fabry
/
Glándulas Ecrinas
/
Hipohidrosis
Límite:
Humans
Idioma:
En
Revista:
J Dermatol Sci
Asunto de la revista:
DERMATOLOGIA
Año:
1998
Tipo del documento:
Article
País de afiliación:
Japón