Collagenous sprue

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Collagenous sprue is a very rare disorder of the small bowel. There are approximately 60 reported cases in the world literature.[1]

Collagenous sprue
Diagnosis in short

LM subepithelial collagen layer > 12 micrometres, villous atrophy, crypt atrophy.
LM DDx celiac disease
Stains demonstrate collagen - Masson trichrome, Mallory trichrome
Site small bowel

Associated Dx possibly celiac disease
Clinical history unresponsive to gluten free diet
Prevalence very rare

General

Clinical:

  • Diarrhea.
    • Not responsive to Gluten-free diet.

Microscopic

Features:[1]

  • Subepithelial collagen layer > 12 micrometres - key feature.
  • Villous atrophy.
  • Crypt atrophy.

DDx:

Images

Stains

Stains to demonstrate collagen:

See also

References

  1. 1.0 1.1 1.2 Zhao, X.; Johnson, RL. (Jun 2011). "Collagenous sprue: a rare, severe small-bowel malabsorptive disorder.". Arch Pathol Lab Med 135 (6): 803-9. doi:10.1043/2010-0028-RS.1. PMID 21631278.
  2. Busto-Bea, V.; Crespo-Pérez, L.; García-Miralles, N.; Ruiz-Del-Árbol-Olmos, L.; Cano-Ruiz, A. (May 2013). "Collagenous sprue: Don´t forget connective tissue in chronic diarrhea evaluation.". Rev Esp Enferm Dig 105 (3): 171-174. PMID 23735026.
  3. Vakiani, E.; Arguelles-Grande, C.; Mansukhani, MM.; Lewis, SK.; Rotterdam, H.; Green, PH.; Bhagat, G. (Jan 2010). "Collagenous sprue is not always associated with dismal outcomes: a clinicopathological study of 19 patients.". Mod Pathol 23 (1): 12-26. doi:10.1038/modpathol.2009.151. PMID 19855376.
  4. Xiao, Z.; Dasari, VM.; Kirby, DF.; Bronner, M.; Plesec, TP.; Lashner, BA. (Jun 2009). "Collagenous sprue: a case report and literature review.". Gastroenterol Hepatol (N Y) 5 (6): 418-24. PMID 20574500.