[Total villous atrophy, mesenteric lymph-node cavitation, splenic atrophy. An unusual form of celiac disease in adults, apropos of a new case].
The authors report a new case concerning the syndrome "total villous atrophy, mesenteric lymph-node cavity formation and splenic atrophy" in a 41 year old woman. This pathologic association is characterized by: a) a clinical and biological malabsorption syndrome; b) proximal small bowel alterations as observed in coeliac disease, with, especially subtotal or total villous atrophy, abnormal enterocytic epithelium, hyperplastic crypts and lymph-plasmacytic infiltrate in the lamina propria; c) lymph node mesenteric cavity formation with an heterogeneous necrotic, sometimes liquefied, substance, without germ or parasite. Rare cortical lymphoid follicles are still persistent; 3) a splenic atrophy. A temporary improvement with gluten-diet (G.F.D.) was followed by a one-year period of total resistance. A treatment including corticotherapy was then successful and since the 14 past months her health remained satisfactory. Clinical, pathological data and the evolution about this patient are compared with those of the six published cases. The aetiology of this syndrome only observed in adults at the present time is unknown; the fact that patient's child presents with a coeliac disease, allows to authenticate, for the first time, this syndrome as a special form of adult coeliac disease.
D000328:Adult; D000367:Age Factors; D001284:Atrophy; D002446:Celiac Disease / Q000473:pathology*; D005260:Female; D006801:Humans; D007421:Intestine, Small / Q000473:pathology*; D008198:Lymph Nodes / Q000473:pathology*; D008643:Mesentery / Q000473:pathology*; D010532:Peritoneal Diseases / Q000473:pathology*; D013154:Spleen / Q000473:pathology*; D013577:Syndrome
Atrophie villositaire totale, cavitation ganglionnaire mésentérique, atrophie splénique. Forme particulière de la maladie coeliaque de l'adulte, à propos d'un nouveau cas.