Histopathology and Differential Diagnosis of Celiac Disease

Overview

Marsh’ classification of celiac disease or malabsorption syndrome sensu stricto is based on increasednumber of intraepithelial lymphocytes, as the first and constant sign/feature of the disease.Thereafter follow structural alterations of the mucous membrane, particularly of villous architecture,crypt height and enterocytes. According to the Marsh’ classification, type 0 corresponds tonormal mucous membrane, and type 1, the infiltrative type, is characterised by an increase inintraepithelial lymphocytes (IEL), which amount to more than 40 lymphocytes/100 enterocytes.Type 2 has normal villous architecture, an increase in IEL numbers and crypt hyperplasia. Type3, the destructive type, is characterised by villous atrophy and may be divided into three subgroupsdepending on the degree of the atrophy - mild, marked, and total (flat mucosa). Combinationof these histopathological findings, patient’s history and clinical course may result in thedefinition of six „states“ of celiac disease. Marsh’ classification is helpful for an early diagnosis ofthe disease and should be applied in any case of the bioptical examination of the malabsorptionsyndrome. The disadvantage of this system, applied on formol-paraffin tissue sections, is impossibilityof revealing disacharidases (particularly lactase) deficiency. From this point of view, histochemicaldetection of disacharidases should accompany the bioptical examination of jejunalmucosa.

Key words:
celiac disease - malabsorption syndrome - biopsy - histochemistry.