[Calciphylaxis: a rare differential diagnosis of systemic vasculitis]. / La calciphylaxie: un diagnostic différentiel rare de vascularite systémique à ne pas méconnaître.

INTRODUCTION: Calciphylaxis is a rare phenomenon of medium- and small-vessel calcifications leading to cutaneous necrosis mimicking vasculitis. CASE REPORT: A 75 year-old-woman with chronic renal insufficiency was admitted for extensive cutaneous necrosis of the limb. Diagnosis of vasculitis was made, but inspite of corticosteroid therapy, the condition of the patient was worsening. After cutaneous biopsy, the diagnosis of calciphylaxis was established. CONCLUSION: Calciphylaxis must be suspected in cases of cutaneous necrosis occurring in a patient with chronic renal failure. Treatment requires rapid normalization of phosphocalcic balance. It is a condition with high mortality.

Seven years of recurrent severe strongyloidiasis in an HTLV-I-infected man who developed adult T-cell leukaemia.

OBJECTIVE: Human T-cell leukaemia/lymphoma virus type I (HTLV-I) is endemic in Japan, the Caribbean basin and Africa, where it has been aetiologically linked to certain chronic myelopathies and adult T-cell leukamia (ATL). We sought to investigate whether strongyloidiasis, a parasitic disease common in these areas, might be a cofactor in the pathogenesis of ATL, as some reports have suggested. PATIENTS, PARTICIPANTS: One 35-year-old HTLV-I-seropositive French West Indian man with a 7-year history of recurrent strongyloidiasis associated with episodic hyperinfestation presenting at the Centre Hospitalier Intercommunal, Villeneuve St Georges, France. INTERVENTIONS: Treatment with various chemotherapeutic agents and symptomatic therapy for hypercalcaemia and antiviral therapy (zidovudine and interferon). RESULTS: The patient developed ATL and died shortly after, despite chemotherapy. Immunological and virological studies performed during the last 15 months of his life showed an increase of the percentage of peripheral ATL cells, and progression from a polyclonal to a monoclonal integration of HTLV-I proviral DNA in the peripheral blood mononuclear and lymph-node cells. CONCLUSIONS: Recurrent strongyloidiasis appears to have been a possible cofactor associated with progression from healthy carrier state to ATL in our patient.

Histomorphological characteristics of gastric mucosa in patients with Zollinger-Ellison syndrome or autoimmune gastric atrophy: role of gastrin and atrophying gastritis.

The role of gastrin in the pathophysiology of two diseases affecting the human stomach, the Zollinger Ellison syndrome (ZES) and the pernicious anemia (PA), is reviewed. Both diseases present chronic hypergastrinemia but from different origins. The ZES is characterized by the occurrence of ectopic endocrine gastrin-secreting tumors and PA by a fundic atrophic gastritis leading to complete atrophy of fundus and resulting in achlorhydria. In PA, the lack of acid induces continuous gastrin cell activation and is responsible for the subsequent gastrin hypersynthesis and secretion. In ZES, hypergastrinemia causes hypertrophy of the oxyntic mucosa, which, in addition, displays hyperplasia of parietal and mucus cells. In both diseases, hypergastrinemia also induces the hyperproliferation of enterochromaffin-like endocrine cells in the fundic mucosa, which can offer all aspects from hyperplasia, then dysplasia, until true carcinoid tumor. The influence of antisecretory treatments and MEN 1 in the ZES as well as that of several other factors and antrectomy in PA on the behavior of the different gastric cells is evoked. Finally, the role that gastrin and its receptor play in the maintenance of the normal development of gastric mucosa and gastric acid secretion is emphasized by results observed in gene knockout models.

[10-year retrospective analysis of the sequelae of loco-regional treatment of breast cancer]. / Analyse rétrospective à 10 ans des séquelles du traitement loco-régional des cancers du sein.

With a minimum follow-up period of 10 years, the incidence of sequelae after loco-regional treatment of 349 breast cancers remains low, being dominated by lymphoedema. Rare after treatment by irradiation alone, lymphoedema appears more frequently in patients treated by radio-surgical association, in spite of a moderate dose of radiation. If a rigorous technique is carried out, the possibility of achieving satisfactory local control in the axilla with radiation alone without neurological sequelae, therefore brings into question the need for axillary dissection without clinically significant adenopathy.

Peptic ulcer disease: one in five is related to neither Helicobacter pylori nor aspirin/NSAID intake.

BACKGROUND: The proportion (and even the reality) of peptic ulcer disease (PUD) not related to H. pylori or NSAID/aspirin is debated. AIM: To analyse the current epidemiological and clinical characteristics of peptic ulcer disease in French general hospitals. METHODS: Prospective multicentre study of patients with peptic ulcer disease in 32 French general hospitals over 1 year. H. pylori status was assessed by histology, and/or serology and/or C13-urea breath test. NSAID/aspirin intake (obtained by direct interview) and data about concomitant diseases were collected on the day of endoscopy. RESULTS: Nine hundred and thirty-three patients were selected during the year 2009. After exclusion of 118 patients with only erosive duodenitis, 24 with major missing data, 13 with other causes of ulcer and 65 negative for H. pylori by only one test, 713 patients were classified into four groups: 285 (40.0%) had only H. pylori infection; 133 (18.7%) only gastrotoxic drugs; 141 (19.8%) had both and 154 (21.6%) neither H. pylori infection nor gastrotoxic drug intake ('idiopathic ulcers'). Patients with idiopathic ulcers differed in many ways both from H. pylori and NSAID/aspirin groups. However, multivariate analysis identified only three independent predictors: age, French metropolitan origin and the presence of comorbidities. CONCLUSION: In a general hospital-based population in France, peptic ulcer disease appears idiopathic in a fifth of cases.

Evolution of fundic argyrophil cell hyperplasia in nonantral atrophic gastritis.

Fundic argyrophil cells were studied for a mean period of 68.7 months (range, 11-170) in 18 patients with fundic atrophic gastritis and achlorhydria. Initially, 12 patients had hyperplasia of the argyrophil cells, the severity of which was assessed using a semiquantitative classification based on the number of argyrophil clusters per square millimeter. At the end of the study, the degree of hyperplasia was unchanged in 9 patients, had decreased in 2, and had increased in 1; no significant increase in the number of argyrophil clusters, precarcinoid changes, or carcinoid tumors were observed and the high level of gastrinemia [mean, 4.8 (range, 1.9-8.1) times the upper limit for normal) did not change significantly. Of the 6 patients with no hyperplasia at the outset of the study, 4 continued without hyperplasia and 2 presented a low-grade hyperplasia at the 20th and 130th month. Gastrinemia increased significantly in the last patient and stayed normal in the other 5. This study argues in favor of the stable appearance of fundic argyrophil cells in patients with atrophic gastritis and stable gastrinemia.

[Kikuchi-Fujimoto disease: histiocytic necrotising lymphadenitis]. / Maladie de Kikuchi - Fujimoto: lymphadénite histiocytaire nécrosante.

BACKGROUND: Kikuchi-Fujimoto disease or necrotizing histiocyte lymphadenitis is a disease of unknown cause whose main clinical feature is adenopathy, generally in a cervical location. The disease was described for the first time in Japan in 1972 and the first case in France was reported in 1986. Clinicians should be aware of this uncommon disease in order to differentiate it from infectious adenitis, lymphoma and collagenosis. CASE REPORT: A 21-year-old woman consulted the emergency physician in January 2002 for a right cervical mass which had developed rapidly with no associated symptom. Other smaller masses developed within the following days. Laboratory results demonstrated discrete inflammation. All serologies tested were normal. Diagnostic cervicotomy was performed. The histological diagnosis was necrotizing adenitis. DISCUSSION: Necrotizing histiocyte lymphadenitis is generally observed in young women in their twenties. There has been no predominant ethnic background. Laboratory tests are normal excepting for discrete signs of inflammation. Diagnosis is provided by pathology and immunohistochemistry. The clinical course is generally spontaneously favorable in six months. Relapse is uncommon.

Sarcoidosis of the nose: report of a case with nasal perforation.

We report a case of multisystem sarcoidosis with involvement of the nose and septal perforation in a young West Indian woman. We discuss the etiology of nasal perforation and prognosis of such a lesion.

[Papillary cystadenocarcinoma of the submaxillary gland. A rare diagnosis]. / Le cystadénocarcinome papillaire de la glande sous-maxillaire. Un diagnostic rare.

The authors describe a case of a "separate" type of salivary adenocarcinoma-like tumor. The growth is described as a papillary cystadenocarcinoma of the submaxillary gland, which should be distinguished from degenerate adenolymphoma. This malignant tumor, which remains "enclosed" until late, grows slowly and should be checked regularly over a protracted period of time following surgical excision performed in accordance with cancer therapy specifications.

[Venous hemangioma of the temporal muscle. Review of the literature: apropos of a case]. / Hémangiome veineux du muscle temporal. Revue de la littérature: à propos d'un cas.

A 41-year-old woman progressively developed a painful tumefaction in the left temporal fossa. The scan showed a isodense intramuscular structure weakly enhanced by contrast medium. Surgical exeresis was performed via the coronal access. Histology of the surgical specimen reported the diagnosis of venous muscular haemangioma. The diagnosis and management of these rare tumours is discussed.

Expanding cerebellar lacunes due to dilatation of the perivascular space associated with Binswanger's subcortical arteriosclerotic encephalopathy.

An 80-year-old hypertensive woman developed right hemiplegia and died 24 hours after admission. Neuropathologic examination revealed multiple cerebral infarcts of various ages and diffuse subcortical arteriosclerotic encephalopathy. Clusters of asymptomatic "expanding" lacunes, due to dilatation of the perivascular spaces, were found in both dentate nuclei. These cavities, which presented as space-occupying lesions, were surrounded by a single layer of flattened cells and contained 1 or more sections of normal-looking arterioles. Such a topographic grouping of lacunes in the dentate nucleus has not been described previously. The mechanism of widening of the perivascular compartment remains unclear; its occurrence in a hypertensive patient and its association with typical Binswanger's subcortical arteriosclerotic encephalopathy and severe atherosclerosis with multiple infarcts suggest a common pathophysiologic mechanism possibly including an alteration of the blood-brain barrier.