Surgical Pathology Criteria

Microvillus Inclusion Disease

Definition

Alternate/Historical Names

Diagnostic Criteria

Differential Diagnosis

Tufting Enteropathy Microvillus Inclusion Disease
Surface epithelial tufts Apical cytoplasmic inclusions
Both may have scant inflammation with villous atrophy

 

Enteroendocrine Cell Dysgenesis Microvillus Inclusion Disease
Normal villi Severe villus atrophy
Intestinal endocrine cells markedly decreased or absent Normal intestinal endocrine cells
Normal enterocyte cytoplasm PASd positive apical cytoplasmic inclusions (microlumena)
Normal brush border Loss of brush border

Markedly decreased or absent endocrine cells

Endocrine cells present

Both present with neonatal diarrhea and lack significant inflammation

 

Microvillus Inclusion Disease Abetalipoproteinemia
Apical cytoplasmic microlumena Foamy cytoplasm filled with fat vacuoles
Severe villus atrophy Normal villus length
Both present with neonatal diarrhea and lack significant inflammation

Bibliography

Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342

Original posting : November 11, 2009

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