[Unusual localisation of rare primary skin carcinoma: a case report]. / Une localisation inhabituelle d'un carcinome primitif cutané rare: à propos d'un cas.

Adnexal carcinomas are rare, accounting for less than 1% of skin carcinomas. Sclerosus carcinoma of the sweat glands was first described by Goldstein et al. in 1982. We here report the case of a 33-year-old female patient with a retracted perianal skin lesion. Histological examination of perilesional skin biopsy, immunohistochemistry, and negative results of laboratory tests, radiological and endoscopic investigations allowed for the diagnosis of eccrine sclerosus carcinoma. This is a rare tumor, usually characterized by facial localization and slow but aggressive progression. It poses problems in differential diagnosis with benign and malignant tumors; hence the challenge encountered by pathologist of suspecting this carcinoma in patients with any sclerotic and infiltrating skin lesion characterized by slow progression, in a context of preservation of the general state and in the absence of neoplastic history as well as of feeling free to ask for new deep biopsies when in doubt.

[Cheilitis granulomatosa revealing Crohn's disease]. / Macro-chéilite granulomatose révélant une maladie de Crohn.

Many disorders of the digestive tract cause cutaneous manifestations that may be an indication of an underlying condition; hence dermatologist plays a key role in recommending that the patient see a gastroenterologist. Conversely, gastroenterologist often sees patients with mucocutaneous lesions suggesting possible association with well-known digestive disorders. Chronic inflammatory bowel diseases are the typical example of this essential collaboration between the two specialities. We report a case of Crohn's disease revealed by cheilitis granulomatosa. This study involved a 30-year old female patient followed up for cheilitis granulomatosa in the Department of Dermatology. Somatic examination was unremarkable apart from dermatologic lesions (indurated white faciolabial edema and diffuse gingivitis). Biopsy of the labial lesion revealed perivascular epithelioid and giant-cell granulomas without caseous necrosis. Ileocolonoscopy was performed due to suspected inflammatory colitis. It showed gaping and ulcerated Bauhin valve with slightly erythematous ileal mucosa. Anatomo-pathologic study objectified chronic inflammatory bowel disease (CIBD) such as ileocolic Crohn's disease. Patient's evolution was marked by some episodes of rectal bleeding with anoperineal lesions 6 months later: a wide anterior anal fissure with aphthous ulceration 5 cm from the anal margin, complicated, a few weeks later, by the onset of perianal fistulized abscess requiring surgical drainage and seton installation. Hence the indication for thorough treatment of Crohn's disease after complete drying up of the abscess. Dermatologic lesions vary in patients with CIBD. In some cases, they occur in patients with IBD whereas, in other cases, they precede or accompany digestive manifestations, allowing diagnosis of sometimes clinically latent bowel disease.

[Tumors of the small bowel: about 27 cases]. / Les tumeurs du grêle: à propos de 27 cas.

Tumors of the small bowel account for 5% of all tumors of the gastrointestinal tract. Although important progress has been made from a radiological and endoscopic point of view, they have a poor prognosis due to delayed diagnosis. This occurs because of the absence of clinical manifestations and of the difficulties of exploration. This study aimed to highlight the endoscopic and histologic features of tumors of the small bowel. We conducted a retrospective descriptive study in the Department of Hepato-Gastroenterology at the University hospital Hassan II, Fez, over a period of 11 years (2002-2012). We analyzed the epidemiological, clinical, endoscopic and histologic features of tumors of the small bowel. Data were collected from the medical records of 27 patients. The average age was 48 years [21-80 years] with a male predominance (H/F:2.4). Melenas led to the discovery of tumor in 55% of cases, abdominal mass in 15% of cases and Koenig syndrome or occlusive syndrome in 11% of cases. One patient had a history of celiac disease. CT enteroclysis showed the seat of the tumor in 18 cases. Push enteroscopy for detection of proximal tumors and double-balloon enteroscopy for detection of distal tumor were performed in 8 patients (30%). Endoscopy showed ulcerated lesion in 37.5% of cases, ulcerated stenotic lesion in 50% of cases and ulcerobudding stenotic lesion in 25% of cases. Surgery with anatomopathological examination of the surgical specimen allowed the diagnosis in 18 cases. The histologic type was dominated by stromal tumors in 14 cases (51%), followed by adenocarcinomas in 5 cases (18.5%), B-cell non-Hodgkin lymphoma in 4 cases (15%), neuroendocrine carcinoma of the jejunum in 1 case. Tumors of the small bowel are rare but they are characterized by a poor prognosis. Current imaging techniques (CT enteroclysis and MR enterography) coupled with endoscopic examinations, in particular double-balloon enteroscopy, allow earlier diagnosis and mortality reduction.

[Lynch syndrome: case report and review of the literature]. / Syndrome de Lynch: à propos d'un cas et revue de la litterature.

Lynch syndrome or hereditary nonpolyposis colorectal cancer (HNPCC) is the most common form of hereditary colorectal cancers. It increases cancer susceptibility, the risk of colorectal cancer in first-degree, endometrial cancer in women, and to a lesser extent, other cancers (ovarian, small bowel, stomach, urinary tract and hepatobiliary). Thus, the cumulative risk of developing colorectal cancer or endometrial cancer at the age of 80 years rises to 20 and 40% respectively. These cancers are characterized by a positive family history, their occurrence at an early age, and by the development of metachronous cancers in the same individual. This syndrome is transmitted in an autosomal dominant manner. The genes whose alteration is associated with the presence of an HNPCC belong to the family of DNA mismatch repair genes (DNA mismatch repair or MMR): MSH2, MLH1, and MSH6 are involved, in decreasing order of frequency, in 35%, 25% and 2% of cases respectively. Colonoscopic and gynecological monitoring is recommended for patients with a constitutional mutation in MSH2, MLH1 or Msh6 genes. We report one of the first moroccan case with Lynch syndrome whose constitutional mutation in the MLH1 gene was identified in a family member with colon cancer. In reply to the inquiry ofother healthy family members, a presymptomatic diagnosis was made allowing to formulate an appropriate monitoring strategy. Our study aims to highlight the role of oncogenetics in the management of patients with cancer and their families.

Acute esophageal necrosis: a case report and review.

Acute esophageal necrosis, commonly referred to as "black esophagus" or "acute necrotizing esophagitis", is a rare clinical disorder with an unclear etiology. The definition excludes patients with a history of recent caustic ingestion. Oesophageal necrosis can be diagnosed at endoscopy by the presence of black necroting appearing oesophagus. Contrary to the caustic oesophagitis whose treatment is often surgical, treatment of the acute necrositing oesophagitis is primarily medical. The prognosis for patients who develop acute necrotizing oesophagitis is generally poor. We report a new case of acute necrotizing oesophagitis and undertook a literature review of this rare diagnosis.

Sequential therapy versus standard triple-drug therapy for Helicobacter pylori eradication: result of the HPFEZ randomised study.

OBJECTIVE: The aim of this study was to assess and compare the efficacy and safety of sequential treatment with standard triple therapies in a located population in Morocco. METHODS: Consecutive H. pylori-positive patients with endoscopy-proven ulcer or non ulcer dyspepsia were prospectively randomized in the trial into one of three groups: AM and AC group were administered a tri-therapy for 7 days including PPI + amoxicillin + metronidazole (AM group)/clarithromycin (AC group) and SQ group was administered a sequential regimen consisting of PPI + amoxicillin for 5 days followed by PPI + clarithromycin + metronidazole for the remaining 5 days. Eradication was confirmed by 13C-urea breath test 3 months after the end of the treatment. RESULTS: Groups AM, AC and SQ included respectively 104, 115 and 104 patients. They were comparable in terms of age, sex, clinical and endoscopic presentation. The rate of H. pylori eradication with sequential therapy was found at 94.2% (n=98) in ITT and 96% (n=98) in PP. It was higher than those found in the AM group: 70% (n=73) in ITT and 70.8% (n=73) in PP and the AC group: 78.2% (n=90) in ITT and 79.6% (n=90) in PP (0.001). The prevalence of side effects following the sequential treatment was 9.6% (n=10) versus 22% (n=22) and 27.8% (n=32) in the AM and AC groups, respectively, (P=0.006). CONCLUSIONS: Sequential treatment was better tolerated and achieved a significantly higher eradication rate of H. pylori compared with standard triple therapies in this population.

Dysphagia caused by a lateral medullary infarction syndrome (Wallenberg's syndrome).

A 68-year-old man was referred to our hospital for a dysphagia evolving for 10 days. Clinical examination had found neurological signs as contralateral Horner's syndrome, ipsilateral palatal paresis, gait ataxia and hoarseness. Video-fluoroscopy showed a lack of passage of contrast medium to the distal esophagus. Esogastroduodenoscopy was normal. The cranial MRI had shown an acute ischemic stroke in the left lateral medullar region and the diagnosis of Wallenberg syndrome (WS) was established. WS remains an unknown cause of dysphagia in the clinical practice of the gastroenterologist.

Multisystem Sarcoidosis in a Patient on Interferon-α Therapy for Chronic Hepatitis C.

Sarcoidosis is a chronic multisystemic granulomatous disease that is triggered by an autoimmune process. Nowadays, this pathology represents a well-recognized but uncommon complication for antiviral treatment in hepatitis C virus (HCV) infection. Herein, we report a remarkable case of 47-year-old woman treated for chronic HCV infection; the patient has developed interferon alfa-induced sarcoidosis involving the central nervous system. The evolution was fatal despite disrupting the antiviral therapy and initiating a high-dose corticotherapy. This complication of interferon alfa treatment was reported in the literature in only one case. Through this case and a review of the literature, we aim to underline the importance of screening for sarcoidosis before and during the follow-up of HCV patients undergoing antiviral therapy.

Acute hepatitis secondary to parenteral amiodarone does not preclude subsequent oral therapy.

Amiodarone chlorhydrate is a diiodated benzofuran derivative used to treat cardiac rhythm abnormalities. Hepatotoxicity is a relatively uncommon side effect of amiodarone and symptomatic hepatic dysfunction occurs in less than 1% to 3% of patients taking amiodarone. We report here on an unusual case of amiodarone-induced hepatotoxicity. A 29 year old woman with normal liver function was given amiodarone intravenously to treat her atrial fibrillation. She developed acute toxic hepatitis after 24 h. The intravenous form of amiodarone was immediately avoided and replaced by the oral form, using conventional loading doses as soon as the deranged liver function tests had normalized, without recurrence of the hepatitis. These observations show that the occurrence of acute hepatic impairment with intravenous amiodarone does not necessarily preclude the use of this drug by mouth and the necessity of monitoring the hepatic function of patients treated with amiodarone.

Pathological complete response in advanced gastric stromal tumor after imatinib mesylate therapy: a case report.

INTRODUCTION: Gastrointestinal stromal tumors are a rare neoplasm exhibiting, in most cases, mutations of c-kit. Imatinib mesylate is the standard treatment for patients who have advanced gastrointestinal stromal tumors. Although the response rate in patients treated with imatinib mesylate in prospective clinical studies is above 50%, a complete response is very rare. We report the case of a patient with a gastric gastrointestinal stromal tumor who had a pathological complete response after neoadjuvant treatment with imatinib mesylate. CASE PRESENTATION: We report the case of a 54-year-old Arab woman with a gastrointestinal stromal tumor who had a pathological complete response after neoadjuvant treatment with imatinib mesylate. CONCLUSION: The pathological examination of our patient documented a complete pathological response after imatinib therapy. Recently, it has been confirmed that the kinase genotype of KIT and platelet-derived growth factor receptor α can accurately predict a good response to imatinib mesylate therapy. We propose that this patient had a mutation conferring high sensitivity to imatinib mesylate.

Torsion of a wandering spleen.

Wandering spleen is a rare condition defined as a mobile spleen only attached with its pedicle. It can be complicated by a volvulus, which is a surgical abdominal emergency. Preventing infarction is the aim of a prompt surgery that can preserve the spleen and then proceed to splenopexy. We report a rare case of torsion of a wandering spleen associated with a dolichosigmoοd.

Peritoneal tuberculosis in the Fes University Hospital (Morocco). Report of 123 cases.

AIMS: Peritoneal tuberculosis is an important public health issue in Morocco. Our aim was to describe the clinical, biological, and therapeutic features of peritoneal tuberculosis treated in a University Hospital in Morocco. PATIENTS AND METHODS: We retrospectively included 123 patients with peritoneal tuberculosis diagnosed at the gastroenterology unit of the Fes University Hospital between January 2001 and August 2003. RESULTS: The mean age was 28 years with a clear female predominance (sex ratio 2.61). Ascites associated with fever were the most frequent signs found in 80.5% of patients. The ascitic fluid was exsudative in 90% of cases and lymphocytic in 88%. The diagnosis was based on laparoscopy or laparotomy with peritoneal biopsy demonstrating caseating granulomatous lesions in 92.4% of patients. Patients were given antituberculous therapy for 6 months, and the outcome was favourable in 90%. CONCLUSION: Peritoneal tuberculosis is very frequent in Morocco, where the diagnosis is based exclusively on peritoneal biopsies obtained during laparoscopy. With an adapted treatment, the course of the disease is favourable in most cases.