[Atypical purpuric oedema of the nose during granulomatosis with polyangiitis]. / Un œdème purpurique du nez atypique au cours d'une granulomatose avec polyangéite.

INTRODUCTION: Granulomatosis with polyangeitis or Wegener's disease is a necrotizing vasculitis of small and medium vessels associated with antineutrophil cytoplasmic autoantibodies (ANCA). The most frequent sites are lung, ear, nose and throat and kidney. PATIENTS AND METHODS: We report the case of a 47-year-old woman presenting purpuric oedematous plaque with bullous detachment of the nose and hospitalised for the assessment of two suspicious neoplastic lung lesions discovered as a result of a recent stroke and repeated seromucosal otitis. Granulomatosis with polyangeitis was suspected because of multiple systemic lesions. The histopathology of skin lesions and laboratory investigation results were consistent with this diagnosis. A favourable outcome was achieved with corticosteroids and rituximab. DISCUSSION: The diagnosis of GPA is based on criteria established by the American College of Rheumatology. The cutaneous clinical aspect described in our case confirms the polymorphism of the cutaneous lesions possibly associated with this disease. They are rarely isolated but, in some cases, allow early diagnosis with improved prognosis, which remains severe in the absence of treatment.

Oral involvement in sarcoidosis: report of 12 cases.

AIM: To assess the clinical features, treatment and outcome of oral sarcoidosis and to determine whether oral involvement is associated with a particular clinical phenotype of sarcoidosis. DESIGN: Multicentric retrospective study. METHODS: Retrospective chart review. Each patient was matched with four controls. RESULTS: Twelve patients (9 women, 3 men) were identified. Their median age at sarcoidosis diagnosis was 38 years. Oral involvement was the first clinical evidence of sarcoidosis in seven cases and was a relapse symptom in five cases. Clinical presentations were nodules (n = 7) or ulcers (n = 5) and were mostly solitary. The tongue was the commonest site affected (n = 4), followed by lips (n = 3), oral mucosa (n = 2), palate (n = 2) and gingiva (n = 1). Patients with oral sarcoidosis were significantly younger and had more frequent lacrimal or salivary glands and upper airway tract clinical involvement than the controls; increased angiotensin-converting enzyme was less frequent in oral sarcoidosis. Multiple treatments of oral sarcoidosis were used: no treatment (n = 3), surgery (n = 2), corticosteroids (n = 7), hydroxychloroquine (n = 3), methotrexate (n = 2), doxycycline (n = 1). Methotrexate was efficient in one patient, hydroxychloroquine showed benefit in only 1 out of 3 patients. Three patients presented oral relapses. After a mean follow-up of 6 years, 10 patients experienced a complete (n = 7) or partial (n = 3) remission of oral sarcoidosis; stability was observed in the remaining two cases. CONCLUSION: Although oral manifestations of sarcoidosis are unusual, physicians should be aware that this specific localization is frequently the first manifestation of the disease. Treatment modalities range from observation in asymptomatic patients to immunosuppressants for severe involvement.

[Diagnostic value of lymphadenopathy associated with fever and inflammation of unknown origin: a study of 69 patients]. / Valeur diagnostique des adénopathies associées aux fièvres et syndromes inflammatoires d'origine inconnue : à propos d'une série de 69 patients.

PURPOSE: The diagnosis of fever or inflammation of unknown origin (FUO/IUO) is guided by the search of clinical clues. Lymphadenopathy is thought to be helpful but its actual contribution has never been tested, and little is known about the main causes of FUO/IUO with lymphadenopathy. The aim of this study was to clarify these issues from the experience of two departments of internal medicine. METHODS: We retrospectively studied a cohort of 69 consecutive inpatients with FUO or IUO and lymphadenopathy, hospitalized from January 2002 to February 2008. The patients were coded according to the final diagnosis and age. Recorded data included lymph node location, fever, CRP level, lymphocyte and platelet counts, presence of hyperbasophilic cells, hypogammaglobulinemia, monoclonal gammopathy, LDH level, and the results of histological and or cytological lymph node examination. RESULTS: Malignancy accounted for 54%, granulomatosis for 23%, mainly of infectious (60%) or malignancy-related origin (18%), the group classified as non-specific adenitis represented 17% of the cases and systemic diseases 4%. Diagnosis was obtained by histological examination of a lymph node biopsy in 80% and by cytology alone in 13% of the patients; no correlation was found between anatomic location, clinical and biological data and any pathological group. Univariate age-independent analysis showed significant correlation between intra-abdominal lymphadenopathy (P=0.05), increased serum CRP (P=0.01) and LDH levels (P=0.05) and malignancy, whereas superficial unique lymph node location (P<0.05), absence of deep site location (P<0.01), and presence of hyperbasophilic cells (P<0.01) were all related to benign non specific adenitis. CONCLUSION: FUO/IUO with lymphadenopathy must be considered as a separate entity, mostly represented by malignancies and granulomatosis, mainly of infectious or malignancy related origin. Fever, intra-abdominal lymphadenopathy, serum CRP and LDH levels and hyperbasophilic cells are relevant indicators.

[Fatal giant cell arteritis with severe bilateral involvement of the vertebral arteries]. / Maladie de Horton d'évolution fatale avec atteinte sévère bilatérale des artères vertébrales.

With steroid therapy, it is commonly considered that prognosis is good in giant cell arteritis. However serious or even fatal complications may occur. Here we report the case of a patient who developed fatal giant cell arteritis with severe stenosis of both vertebral arteries and right carotid siphon. Several similar cases have been reported in the literature. Initially diagnosis may be difficult because neurological manifestations are intermittent and classical signs of giant cell arteritis may be lacking. In such condition the reason of poor outcome is unknown and therapy remains empiric.

[Ortner's syndrome and giant-cell vasculitis]. / Syndrome d'Ortner et maladie de Horton.

INTRODUCTION: Ortner's syndrome was first described as a left laryngeal nerve palsy caused by a dilated left atrium in mitral stenosis. Aortic aneurysm is another well-documented etiology. CASE RECORD: We report the case of a 90 year-old woman with temporal arteritis with recent onset hoarseness, and simultaneous discovery of aortic arch aneurysm and left vocal cord palsy. DISCUSSION: The occurrence of hoarseness and aortic aneurysm in Giant-cell vasculitis is discussed. We suggest to consider Horton's disease (GCA) as a possible etiology of Ortner's cardio-vocal syndrome.

[Hypocholesterolemia during the acute phase of an inflammatory reaction of infectious origin. 120 cases]. / Hypocholestérolémie au cours de la phase aiguë de la réaction inflammatoire d'origine infectieuse. A propos de 120 cas.

OBJECTIVES: Transitory changes in the plasma levels of lipids, cholesterol and triglycerides have been observed since a long time by many authors, in the course of bacterial infections, with hypocholesterolemia, hypertriglyceridemia in the acute phase, increasing the third day of clinical evolution. Their decrease accompanies the return to normal. Lymphopenia is also observed during bacterial infections and as the very low level of cholesterol, is considered to be a factor of critical prognosis, predicting an unfavorable evolution, essentially in elderly people. C-reactive protein (CRP) proves to be a good marker protein in inflammation due to sepsis; its synthesis is directly influenced by the cytokines released during the acute phase response of inflammation in bacterial infection. The authors are researching a correlation between the intensity of the acute phase response represented by CRP levels, and a reduced cholesterol level, or a hypertriglyceridemia, or lymphocytopenia. METHODS: In this prospective study, blood samples at fasting state were obtained in 160 patients divided into four groups of 40 according to CRP levels, including a witness group (CRP levels lower than 10 mg/L) and three groups of patients presenting infectious diseases with acute phase response of different intensities. All patients were checked for cholesterol, triglyceride and CRP levels, blood cell count. The three pathologic groups were compared to the witness group and to each other. RESULTS: A significant correlation was established between the intensity of the acute phase response during sepsis and reduced levels of cholesterol. Cholesterolemia was reduced (P < 0.05) in all three pathologic groups when compared to the witness group; the difference existed when a low intensity inflammatory response was observed (mean CRP level 27.6 (10.5) mg/L in group 1). Moreover a significant response exists with reduced cholesterol levels between group 4 (mean CRP level 250 (73) mg/L) and group 1. Lymphocytopenia was observed in all three pathologic groups, without evident link with the CRP levels. No modification was observed concerning triglyceride levels. CONCLUSIONS: The authors report a negative correlation of total cholesterol to CRP levels at the early stage of infections diseases. Prior studies established a negative correlation between inflammatory parameters during bacterial infections and total cholesterol and HDL fraction. Considering the plasma lipid pathways, HDL fraction plays a major role in lipid transport and exchanges between lipoproteins, thanks to its apolipoproteins A1, A2 and C. IL-1 and TNF, two cytokines involved in the acute phase response, have metabolic functions which could possibly contribute to reduce plasma total cholesterol and HDL2 fraction. IL-1 also induces lymphocytopenia. The authors propose the hypothesis of a correlation between plasma cholesterol levels and the acute phase response during sepsis, which could be induced by the mediators or effectors of inflammation.

[Myocardial infarction following a bee sting. Apropos of a case and review of the literature]. / Infarctus du myocarde consécutif à une piqûre d'abeille. A propos d'un cas et revue de la littérature.

The authors report a case of limited inferior myocardial infarction in a young man following a bee sting. Coronary angiography, performed following the acute phase, showed angiographically healthy coronary arteries and minimal sequelae of myocardial necrosis in the inferior territory. The pathogenic role of the bee sting in myocardial necrosis was suggested by the timing of the events. The probable mechanism responsible for this myocardial infarction was severe coronary arterial spasm (partly mediated by psychological stress related to the intensity of the anaphylactic reaction) with secondary in situ thrombosis probably facilitated by cardiovascular collapse. The role of anaphylaxis, generating acute coronary insufficiency, is discussed in the light of this clinical case.