Hepatic and Appendiceal Whipple''s Disease with Negative Jejunal Biopsies

A 20-year-old man with Whipple's disease, right upper quadrant pain, abnormal liver tests and multiple negative peroral small bowel biopsies is presented. The patient underwent laparotomy: foamy macrophages were noted in the appendix and mesenteric lymph nodes; using electron microscopy, bacillary bodies were seen in the appendix, the mesenteric lymph node, and the liver. This patient had the rare occurrence of Whipple's disease of the appendix with bacillary bodies in the liver and a clinical picture compatible with hepatic involvement or cholangitis and a slow response to conventional antibiotic therapy.     

Not Just a Popliteal Aneurysm: A Case of Metastatic Epithelioid Angiosarcoma

IntroductionPopliteal aneurysms are the second most common aneurysm. This case report describes a case of angiosarcoma in a popliteal aneurysm, illustrating the importance of post-operative surveillance in expanding popliteal aneurysms post-treatment.ReportA 79-year-old male presented with an enlarging mass in the left popliteal fossa, 18 months post-ligation and bypass for a left popliteal aneurysm. The case proved to be metatstatic epithelioid angiosarcoma, arising in the left popliteal fossa.DiscussionEpithelioid angiosarcomas are rare, aggressive vascular neoplasms. An awareness of these lesions during surveillance of patients with expanding popliteal aneurysms post-treatment, would allow for earlier diagnosis and more timely interventions.     

Metastatic Crohn's Disease

The case of a 25-yr-old female with Crohn's disease who developed recurrent erythematous nodules on her lower extremities is described. These nodules enlarged and ulcerated. Repeated bacterial, fungal, and acid-fast cultures remained negative. A skin biopsy revealed noncaseating granulomata, confirming the diagnosis of cutaneous "metastatic" Crohn's disease. The lesions completely resolved with systemic corticosteroid therapy. Although cutaneous metastatic Crohn's disease is uncommon, patients with Crohn's disease who develop unusual skin lesions that persist should have them biopsied.     

Cutaneous Metastatic Crohn''s Disease: Treatment with Metronidazole

A 55-yr-old man with ileocolic Crohn's disease developed granulomatous dermatitis in the malar region. He was treated with metronidazole 1.5 g/day for 1 month, with remission of the skin lesion after 10 days.     

Anti-endomysial Antibody Negative Celiac Disease

Anti-endomysium antibodies (AEM) fail to identify all untreated celiac disease (CD) patients. This study aims to determine if additional serology, in particular, IgA anti-tissue transglutaminase (tTG) antibodies, increases detection. Fifty-three biopsy-proven untreated CD patients (39 women, 14 men; median age 51 years) and 65 control patients with normal duodenal histology (46 women, 19 men; age range 17–90 years, median 45 years) were prospectively studied. Serum total IgA, IgA anti-tTG, IgA AEM, IgA anti-gliadin (AGA) and IgG AGA antibodies were measured. Thirteen (25%) CD patients were AEM negative. None were IgA deficient. Three AEM-negative CD patients had a raised IgA anti-tTG and IgA AGA. IgG AGA was raised in 10 AEM-negative CD patients, but also in 14/65 (22%) of controls. In conclusion, AEM-negative CD is common and detection is only modestly enhanced by testing for IgA anti-tTG antibodies. Duodenal biopsy is still recommended for the accurate diagnosis of CD.     

Electrogenic Ion Transport in Duodenal Biopsies from Children with Coeliac Disease

Background: The aim of this work was to study whether different degrees of duodenal mucosal damage in coeliac disease (CD) influenced secretory responses to well-known secretagogues. Methods: Intestinal biopsies from 53 patients in different clinical phases of CD and 34 patients without CD and with normal histology were studied in a modified Ussing chamber. The electrogenic responses--with and without pretreatment with indomethacin--to prostaglandin E 2 , aminophylline, dibutyryl-cAMP and acetylcholine were followed by continuous measurements of potential difference (Pd). Tissue resistance and epithelial current (I m ) were calculated. Results: All secretagogues induced a similar pattern, with a greater increase in Pd and I m in biopsies with villous atrophy compared to controls. The electrophysiological response was correlated to the serum levels of IgA gliadin antibodies. The most prominent electrophysiological increase was found in the biopsies with partial atrophy. Indomethacin had a greater impact on the response to secretagogues in the more severely damaged mucosa. Conclusion: The electrogenic secretory response in the proximal small intestine was enhanced and related to serum levels of IgA gliadin antibodies and to the degree of mucosal damage in biopsies from children with active CD compared to controls.     

Crohn's Disease in Endoscopic Biopsies of the Gastric Antrum and Duodenum

Forty-five patients with Crohn's disease in whom the upper gastrointestinal tract was normal by x-ray were examined by gastroduodenoscopy. Biopsies were analyzed histologically from the lower esophagus, body of the stomach, gastric antrum and duodenal bulb. Cell counts were made of 500 connective tissue cells of the duodenal mucosa. Histological examinations and cell counts of the duodenal mucosa were also performed on 50 healthy volunteers used as controls.
Histological lesions were found in 19 Crohn's disease cases; 11 (24%) were considered pathologically diagnostic and all these were found in the antrum or duodenum. In 11 the mucosa was endoscopically normal. Granulomas were present in three cases (7%), all from normal appearing mucosa. Microscopic alterations of the antrum and duodenum, similar to findings in the normal appearing rectal mucosa, support the concept that Crohn's disease involves the entire alimentary canal and that lesions are seen grossly only where the disease is most advanced.     

Stopping Therapy in HBeAg Negative Disease

Chronic hepatitis B infection confers a major risk of cirrhosis and hepatocellular carcinoma worldwide. The ultimate long-term goal of chronic hepatitis B therapy is to reduce morbidity and mortality related to liver disease progression. The ideal treatment endpoint is HBsAg seroconversion. As HBsAg clearance is rarely achieved, the short-term goal of therapy is maintained suppression of hepatitis B replication. For HBeAg-positive patients, durable HBeAg seroconversion with undetectable HBVDNA is a satisfactory intermediate end-point. For HBeAg-negative patients, the treatment endpoint remains unclear, hence, sustained suppression of HBV DNA level to low or undetectable levels is the optimal treatment response. Several studies in HBeAg-negative population have proposed that discontinuation of antiviral treatment can be considered if undetectable serum HBV DNA is demonstrated on three separate occasions at least 6 months apart. Preliminary data suggests that on-treatment HBsAg quantification may play a role as a predictor of sustained response off-therapy.