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Surgical Pathology Criteria

Microvillus Inclusion Disease


  • Hereditary neonatal diarrhea characterized by microvillus abnormalities

Alternate/Historical Names

  • Davidson syndrome
  • Familial enteropathy
  • Familial microvillous atrophy
  • Microvillous inclusion disease

Diagnostic Criteria

  • Severe intractable diarrhea presents in first week of life
    • Rarely as late as 60 days
  • Severe villous atrophy
    • No crypt hyperplasia
    • No inflammation
  • PAS/d, polyclonal anti-CEA, CD10 stains highlight microvillus abnormalities in small intestine
    • Loss of brush border
    • Apical cytoplasmic positivity
      • May be recognizable as microlumina
  • Electron microscopy reveals loss of brush border and presence of apical cytoplasmic microlumina lined by microvilli
  • Autosomal recessive
  • Rare

Differential Diagnosis

  • Presentation at birth, lack of inflammation and presence of intracytoplasmic microlumina exclude other causes of villous atrophy and diarrhea
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


  • Groisman GM, Amar M, Livne E. CD10: a valuable tool for the light microscopic diagnosis of microvillous inclusion disease (familial microvillous atrophy). Am J Surg Pathol. 2002 Jul;26(7):902-7.
  • Phillips AD, Schmitz J. Familial microvillous atrophy: a clinicopathological survey of 23 cases. J Pediatr Gastroenterol Nutr. 1992 May;14(4):380-96.
  • Shillingford NM, Calicchio ML, Teot LA, Boyd T, Kurek KC, Goldsmith JD, Bousvaros A, Perez-Atayde AR, Kozakewich HP. Villin immunohistochemistry is a reliable method for diagnosing microvillus inclusion disease. Am J Surg Pathol. 2015 Feb;39(2):245-50. doi: 10.1097. PubMed PMID: 25517957.
  • Martin BA, Kerner JA, Hazard FK, Longacre TA. Evaluation of intestinal biopsies for pediatric enteropathy: a proposed immunohistochemical panel approach. Am J Surg Pathol. 2014 Oct;38(10):1387-95. doi: 10.1097. PubMed PMID: 25188866.

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

Original posting :11/11/09 Last update 1/17/15

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