Lymphangiographic Findings in Behcet's Disease With Lymphangiectasia of the Small Intestine

Abstract

Bipedal lymphography was performed in 5 cases of Behçet's disease. In 4 of 5 cases, histologic examination of intestinal biopsy specimens showed lymphatic dilatation of the small intestine which were considered to be a characteristic finding of intestinal lymphangiectasia, a protein-losing enteropathy, but no patient with Behçet's disease showed reduced concentration of serum total protein as well as abnormal value of 131I-PVP test. Lymphograms showed an increase in number of iliopelvic and lumbar lymphatic vessels as a major finding, but they failed to demonstrate a hypoplasia or aplasia of lymphatic system or obstruction of thoracic duct. It is conceivable that the lymphatic dilatation of the small intestine in Behçet's disease may be related to increased flow of lymph due to excessive vascular hyperpermeability and may not be related to a block of lymphatic system which has been considered to be a cause of enteric protein loss in intestinal lymphangiectasia. The authors wish to emphasize that the dilatation of lymphatic vessel of the small intestine is not a sufficient finding to indicate the presence of protein-losing enteropathy.

Enfermedad de Behçet y enteropatía perdedora de proteínas secundaria a linfangiectasia intestinal

“…En el presente caso se cree que la linfangiectasia intestinal asociada a la EB es secundaria a la hipertensión portal causada por los múltiples trombos en el territorio portal. Morita et al 10 evidenciaron un aumento en el número de vasos linfáti-cos iliopélvicos y lumbares en linfogramas de pacientes afectados de EB y linfangiectasia intestinal; concluyeron que la dilatación de las vías linfáticas podría estar relacionada a un aumento en el flujo causado por la hiperpermeabilidad vascular y no un bloqueo en la circulación de la linfa.…”

Intestinal Malabsorption and Skin Diseases

“…An association between IL and skin diseases has been described only in case reports and in particular in children. Behçet's disease [57] , viral warts [55] , aplasia cutis congenita [58] , alopecia [59] , cutis marmorata telangectasia congenita syndrome [60] , and yellow nail syndrome [61] are skin diseases which are reported in the literature and associated with IL. It is possible that the onset of cutaneous lesions in patients affected by IL was likely triggered by a generalized depression of the immune system.…”

Enteraler Eiweißverlust: Pathophysiologie, Ursachen, Diagnostik und Therapie mit Fallbeispielen

“…Unverträglichkeitsreaktionen (Eosinophile Enteropathie [50], Kuhmilchallergie [39,52], Zöliakie [30] chronisch-entzündliche Darmerkrankungen Morbus Crohn [3] Kolitis ulzerosa [74,78] Morbus Behcet [58] Autoimmunenteropathie [56,57,86] Graft-versus-Host-Erkrankung [88] Infektionen Viren (Adenoviren; Masern [23]; HIV [4,47]; M. Menetrier: CMV, Hepatitis C [75]) bakterielle Infektionen der Darmwand (Klostridien [17], Shigellen [6], Salmonellen [29], Yersinien [49], Tuberkulose [53]) bakterielle Überwucherung des Dünndarms [38] Parasiten (Lamblien [25,79], Schistosomiasis [26]…”