Nasal oral fistula revealing Langerhans´ cell histiocytosis in adult patient: case report.

Langerhans cell histiocytosis (LCH) is a rare systemic disease caused by proliferation of mature histiocytes; its association to histiocyto fibroma is rarely reported. It rarely affects adults. We report a case of systemic LCH, in an adult patient with osteolytic lesion causing a fistula between the left nasal cavity and hard palate, involving the bone, lung, lymph node and associated to multiple histiocyto fibroma. The patient was operating for a fistula, and he was treated by chemotherapy and corticosteroids. Langerhans´ cell histiocytosis is a rare case, especially in adult patient. The diagnosis was based on histological and immunohistochemical analyses. This patient was treated by steroids and chemotherapy.

Elderly-onset adult Still's disease.

Adult-onset Still's disease is a rare inflammatory disorder usually affecting young adults. Elderly-onset Still's disease (EOSD) is reported in some cases, commonly in Japan, the USA and Europe. One of the most commonly used criteria for diagnosing EOSD is Yamaguci criteria. In elderly patients more severe course of the disease and more complications may be expected than in the younger group of patients with Still's disease. The lungs involvement is rather rare manifestation of this disease. In our article we discuss the problem of both the development of Still's disease in the elderly and interstitial lung changes in the course of the disease, based on available literature and own cases from one centre.

Influence of weather and air pollution on the occurrence of idiopathic pulmonary embolism in the region of Sousse (Tunisia).

Pulmonary embolism (PE) is the most serious manifestation of thromboembolic conditions. Its incidence varies considerably between countries, suggesting its interaction with the external environment. To analyze the influence of climate and air pollution on the occurrence of idiopathic PE in the region of Sousse (Tunisia). A total of 142 patients with idiopathic PE at two academic hospitals in Sousse (Tunisia) were enrolled in the study over a 7-year period. An analysis of two time series (environmental data and PE cases) was performed. Climatic data were collected from the National Institute of Meteorology. Air pollution data were obtained from the modeling platform of the National Agency for Protection of the Environment. The year 2015 was marked by the occurrence of the highest number of cases (24.6%). A statistically significant decrease in PE risk of 41.9% was observed during the summer with an OR of 0.59 (95% CI [0.36-0.94] and p = 0.026), compared with other seasons. Poisson GLM regression showed a significant increased risk of PE of 3.3% for each 1 °C temperature drop. After multiple binary logistic regression, the elevation of PM10 concentration was independently associated with an increased risk of PE (p < 10-3, OR 79.55, 95% CI [42.28-149.6]). Some environmental parameters may predispose to the onset of idiopathic PE. Understanding their accurate influence may have preventive and curative implications.

[Multifocal tuberculosis in immunocompetent patients]. / Tuberculose multifocale chez les immunocompétents.

Multifocal tuberculosis is defined as the presence of lesions affecting at least two extrapulmonary sites, with or without pulmonary involvement. This retrospective study of 10 cases aims to investigate the clinical and evolutionary characteristics of multifocal tuberculosis. It included 41 cases with tuberculosis collected between 1999 and 2013. Ten patients had multifocal tuberculosis (24%): 9 women and 1 man, the average age was 50 years (30-68 years). Our patients were correctly BCG vaccinated. The evaluation of immunodepression was negative in all patients. 7 cases had lymph node tuberculosis, 3 cases digestive tuberculosis, 2 cases pericardial tuberculosis, 2 cases osteoarticular tuberculosis, 1 case brain tuberculosis, 2 cases urinary tuberculosis, 4 cases urogenital tuberculosis, 1 case adrenal tuberculosis, 1 case cutaneous and 1 case muscle tuberculosis. All patients received anti-tuberculosis treatment for a mean duration of 10 months, with good evolution. Multifocal tuberculosis is difficult to diagnose. It can affect immunocompetent patients but often has good prognosis. Anti-tuberculosis therapy must be initiated as soon as possible to avoid sequelae.

Amyloïdosis, sarcoidosis and systemic lupus erythematosus.

The occurrence of renal and multiple organ Amyloïdosis is currently considered exceptional in the course of systemic lupus erythematosus. We report a case of a concomitant SLE and Amyloïdosis in a 57 year old female patient with hypothyroidism history, who presented with erythema nodosum, fever, arthralgia and sicca syndrome. Biological findings showed an inflammatory syndrome, renal failure, proteinuria (1g / 24h), positive auto antibodies and anti DNA. Lung radiology revealed medistinal lymphadenopathy, pleural nodules, ground glass infiltrates and pleuritis. Bronchial biopsy showed non specific inflammation. The salivary gland biopsy showed amyloïd deposits. This case report reminds us that lupus and Amyloïdosis association, although exceptional remains possible. The occurrence of Lofgren syndrome in this situation make the originality of this report.

[Catastrophic antiphospholipid syndrome. Report of two cases]. / Syndrome catastrophique des antiphospholipides. A propos de deux observations.

The antiphospholopid syndrome has been associated with thromboembolic events, thrombocytopenia and fetal loss. Some patients with the antiphospholipid syndrome may develop an acutely catastrophic syndrome characterized by multiple vascular occlusions which often results in death. Most patients dye as a result of a combination of cardiac and pulmonary failure. Although trigger factors are present in a minority, in the majority the condition develops quite suddenly. Precipitating factors include infections and trauma (surgical). Treatment of the condition, once recognized, needs to be heroic. Plasmapheresis seems to be useful in several cases who had not responded to conventional therapy (heparin, steroids, immunosuppressive) directed against immunologically mediated intravascular thrombosis. We report here two cases of fatal catastrophic antiphospholipid syndrome and highlight the need for vigilance in the management of patients with antiphospholipid syndrome.