Mucocutaneous ulcer positive for Epstein–Barr virus,misdiagnosed as a small bowel adenocarcinoma:A case report

BACKGROUND Epstein–Barr virus(EBV)-positive mucocutaneous ulcers(MCUs)are an uncommon disorder characterized by ulcerative lesions in the skin,oral cavity or gastrointestinal tract in patients with iatrogenic or aging-induced immunosuppression.The nonspecific lesions are difficult to differentiate from small bowel adenocarcinomas.We present the case of a 69-year-old woman who was initially misdiagnosed with a small bowel adenocarcinoma but was later surgically diagnosed with and treated for EBV-MCU.Through this case,we aim to emphasize the importance of accurately distinguishing between the two conditions.CASE SUMMARY The patient presented with an incidental finding of a small bowel tumor during computed tomography(CT)examination performed for hematuria.The CT scan showed irregular thickening of the distal ileum,which was suggestive of a malignant small bowel tumor.An exploratory laparotomy revealed an 8-cm mass in the distal ileum;thus,a segment of the small intestine,including the mass,was resected.Histopathological analysis revealed an ulceroinfiltrative mass-like lesion with luminal narrowing,marked inflammatory cell infiltration,and large atypical lymphoid cells(positive for EBV-encoded small RNA).A final diagnosis of an EBV-MCU was established.The postoperative course was uneventful,and the patient was discharged on postoperative day 7.The patient remained recurrencefree until 12 mo after surgery.CONCLUSION This case highlights the diagnostic challenges for EBV-MCUs and emphasizes the importance of comprehensive evaluation and accurate histopathological analysis.

Epstein Barr virus-positive mucocutaneous ulcer of thecolon associated Hodgkin lymphoma in Crohn＇s disease

Epstein Barr virus （EBV） positive mucocutaneous ulcers（EBVMCU） form part of a spectrum of EBV-associatedlymphoproliferative disease. They have been reportedin the setting of immunosenescence and iatrogenicimmunosuppression, affecting the oropharyngeal mucosa,skin and gastrointestinal tract （GIT）. Case reports andseries to date suggest a benign natural history respondingto conservative management, particularly in the GIT. Wereport an unusual case of EBVMCU in the colon, arisingin the setting of immunosuppression in the treatment ofCrohn＇s disease, with progression to Hodgkin lymphoma18 mo after cessation of infliximab. The patient presentedwith multiple areas of segmental colonic ulceration,histologically showing a polymorphous infiltrate withEBV positive Reed-Sternberg-like cells. A diagnosisof EBVMCU was made. The ulcers failed to regressupon cessation of infliximab and methotrexate for 18mo. Following commencement of prednisolone for herCrohn＇s disease, the patient developed widespreadHodgkin lymphoma which ultimately presented as alife-threatening lower GIT bleed requiring emergencycolectomy. This is the first report of progression ofEBVMCU to Hodgkin lymphoma, in the setting of ongoingiatrogenic immunosuppression and inflammatory boweldisease.

Epstein-Barr Virus Upper Gastrointestinal Tract Mucocutaneous Ulceration in a Renal Transplant Recipient with Leucopenia and Hypogammaglobulinaemia

Epstein-Barr virus (EBV) mucocutaneous ulceration is a rare complication of immunosuppression that results in painful ulceration of the tongue or gingiva, and less commonly, refractory ulceration of the gastrointestinal tract. High clinical suspicion is required, as failure to diagnose EBV mucocutaneous ulceration may result in significant morbidity and mortality. We report the case of a 64-year-old female renal transplant recipient requiring admission to hospital for management of severe oral and epigastric pain. Examination revealed a large, superficial, well-circumscribed ulcer at the base of the tongue. Blood tests suggested a secondary immunodeficiency characterised by mild leucopenia, hypogammaglobulinaemia, and low memory B-cells with normal immunophenotype. Endoscopy revealed four, cratered ulcers in the pre-pyloric region of the stomach. A core biopsy of the tongue ulcer confirmed EBV mucocutaneous ulceration. The patient’s immunosuppression was optimised, which resulted in complete resolution of the tongue and gastric ulcers at eight and twelve weeks of follow-up respectively.

Clinical Profile and Significance of Mucocutaneous Lesions of Primary Sjogren＇s Syndrome： A Large Cross-sectional Study with 874 Patients

Background： Mucocutaneous lesions are common features of primary Sjogren＇s syndrome （pSS）, but only a few studies have focused on them. To demonstrate the profile ofmucocutaneous lesions ofpSS and further explore their potential clinical significance, we perfermed a cross-sectional study on 874 patients. Methods： Demographic data, clinical manifestations, and laboratory results of 874 pSS patients were collected. Patients were divided into two groups according to the presence of mucocutaneous lesions. Differences in primary symptoms and systemic impairments between the two groups were analyzed. Results of laboratory tests were also compared alter excluding those who had taken corticosteroid from both groups. One-year follow-up was done, and occurrences of various new complications were compared. Results： Among the 874 pSS patients, 181 patients had mucocutaneous lesions, accounting for 20.7%. Multiple mucocutaneous manifestations were displayed, and the top four most common types of lesions were purpuric eruptions （39.8%）, urticaria （23.8%）, Raynaud＇s phenomenon （14.9%）, and angular stomatitis （9.9%）. Incidences of pulmonary interstitial fibrosis, pulmonary bullae, leukopenia, and anemia were significantly higher among patients with mucocutaneous lesions （P 〈 0.05）. Increase in lgG and decrease in C4 among patients with mucocutaneous lesions displayed statistical significance after excluding patients from both groups who had taken corticosteroid （P 〈 0.05）. Alter one-year follow-up, patients with mucocutaneous lesions presented a slightly higher incidence of new complications compared to those without. Conclusions： Mucocutaneous manifestations ofpSS patients were common and diverse. Patients with mucocutaneous manifestations had more systemic damages, higher level of IgG, and lower level of serum C4, suggesting a higher activity of the primary disease.

<i>STAT</i>1 gene mutation is not implicated in upper aerodigestive cancers

Autosomal Dominant Chronic Mucocutaneous Candidiasis (AD-CMC) is characterized by defective T cell immunity, leading to fungal infections limited to mucosal surfaces. Recently it was discovered that mutations in the coiled-coil (CC) domain of STAT1 are the cause of AD-CMC. STAT1 deficiency has been implicated in experimental models of oesophageal cancer (EC) and head and neck carcinoma (HNC). Both carcinoma types are prevalent among CMC patients. Consequently, we postulated that the same mutation in the STAT1 gene triggering AD-CMC, could also be involved in oesophageal- or head and neck carcinogenesis. However we failed to identify the c.820C>T mutation in the STAT1 CC domain in 3 cohorts of Dutch Caucasian origin: being 351 EC patients, 325 HNC patients and 309 controls. Although it seems valuable to investigate the relationship between AD-CMC and upper aerodigestive neo- plasms, the c.820C>T mutation in the STAT1 gene does not seem implicated in EC and HNC aetiology.

Oral lichenplanus: Etiology, pathogenesis, diagnosis, and management

Oral Lichen planus(OLP) is a common chronic mucocutaneous disorder with an immune mediated pathogenesis. Its appearance may vary from presence of keratotic to erythematous areas. Etiology of OLP is unknown, but it is thought to be the result of an autoimmune process with an unknown predisposing factor. Oral lichen planus is a complex and poorly understood clinicalcondition with periods of remissions and exacerbations. Management of the OLP is diversified with few lesions requiring treatment for years and few others are mild, requiring no treatment.

Perforation of the Nasal Septum and Nasal Ulcers

A case about a HIV woman with nasal ulcers is described in this paper. In every inmunodepressed patient who has mu-cosal or cutaneous ulcers, infection by leishmanial parasites needs to be ruled out, especially when ulcers have not re-gression with usual treatments. The Leishmanial nasal disease usually shows swelling and mucosal ulcers, that may progress to necrosis. Delaying in appropriate therapy might cause irreversible damage.

Epidemiologic features of Kawasaki disease in Shanghai from 2003 through 2007

Background The epidemiologic pictures of Kawasaki disease （KD） in Shanghai from 1998 through 2002 were reported while the current status of KD in the following five years remains unknown. Methods A questionnaire form and diagnostic guidelines for KD were sent to 50 hospitals providing pediatric medical care in Shanghai, China. All patients with KD diagnosed during January 2003 through December 2007 were recruited. Results In total, 1187 cases of KD were enrolled. The incidence of KD was 36.78 to 53.28 （mean 46.32±6.51） per 100 000 children under the age of 5 years between 2003 and 2007, which was higher than the year from 1998 to 2002 of （27.32±7.11） per 100 000, （t=4.406, P=0.002）. Ages at onset ranged from 12 days to 13.6 years （median 1.8 years）. It occurred more frequently in summer and spring. Coronary arterial lesions （CAL）, defined as ectasia or aneurysm, accounted for 19.8% （232 cases）. Flattened or inverted T wave was the most frequent finding （194 cases, 20.5%） by electrocardiogram. Intravenous gamma-globulin was administrated to 1028 cases （86.6%）. The occurrence of CAL seemed less frequent in the patients received gamma-globulin from day 5 to day 9 after the onset with the regimen of 1000 mg/kg once or 1000 mg/kg twice. Conclusions The incidence of KD was increasing in Shanghai. Treatment with intravenous gamma-globulin from day 5 to day 9 after the onset with the regimen of 1000 mg/kg once or 1000 mg/kg twice resulted in less coronary seauelae.

Genetic susceptibility to Candida infection： a new look at an old entity

The identification of the first molecular defect leading to Mendelian Susceptibility to Mycobacterial Disease ;MD）,1 a rare syndrome conferring predisposition to（MSMD）,1 a rare syndrome conferring predisposition to disease caused by weakly virulent mycobacteria （such as Mycobacterium bovis, Bacille Calmette Gu6rin vaccines and environmental mycobacteria）, has led to a paradigm shift in the field of primary immunodeficiencies in the last two decades. The ＂classic＂ patient with multiple immunologic abnormalities, conferring a broad susceptibility to multiple and recurrent infectious diseases caused by both weakly pathogenic and more virulent microorganisms, was the main Primary Immunodeficiency （PID） phenotype identified. Since 1996, mutations causing MSMD have been found in at least 6 genes related to interferon （IFN）-7-mediated immunity.2