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A light and electron microscopic study of mannosidosis
Human Pathology
|May 1, 1980
Summary
Mannosidosis causes progressive gingival hyperplasia and inflammation, characterized by widespread cellular vacuolation. These vacuoles, resembling secondary lysosomes, indicate excessive substrate storage in gingival cells.
Area of Science:
- Cell Biology
- Oral Pathology
- Lysosomal Storage Diseases
Background:
- Mannosidosis is a rare genetic lysosomal storage disease.
- Gingival hypertrophy is a known manifestation in some lysosomal storage diseases.
Purpose of the Study:
- To investigate the ultrastructural changes in hypertrophied gingiva of a patient with mannosidosis.
- To evaluate the effect of zinc sulfate therapy on gingival pathology.
Main Methods:
- Light and electron microscopy of gingival biopsy specimens over 20 months.
- Specimens were collected before and after a therapeutic trial with zinc sulfate.
Main Results:
- Progressive disease despite zinc therapy, with epithelial hyperplasia and stromal inflammation.
- Widespread cytoplasmic vacuolation observed in gingival cells, including histiocytes, fibroblasts, endothelial cells, plasma cells, and epithelial cells.
- Vacuoles in histiocytes were large, intercommunicating, membrane-bound, and contained granular material, consistent with secondary lysosomes.
Conclusions:
- The gingival pathology in mannosidosis is characterized by cellular vacuolation indicative of lysosomal storage.
- Zinc sulfate therapy did not halt the disease progression or alter the observed ultrastructural pathology.