[Three cases of bullous lupus erythematosus]. / Étude de trois cas de lupus érythémateux bulleux.

BACKGROUND: Bullous lupus is a subepidermal autoimmune bullous dermatosis, a rare entity that forms one of the cutaneous signs of systemic lupus erythematosus. We report on the clinical, immunopathological and progressive features of bullous lupus in three patients. PATIENTS AND METHODS: Our patients consisted of two women and one man aged 34, 22 and 30 years respectively. A diagnosis of bullous lupus erythematosus was evoked by blisters or vesicular blisters and confirmed, in addition to criteria for the diagnosis of systemic lupus erythematosus, by the presence of subepidermal blistering with infiltrate containing neutrophils and eosinophils as revealed by histological analysis, and of deposits of IgG and IgM (two cases) or of IgA (one case) at the dermo-epidermal junction observed under direct immunofluorescence. Indirect immunofluorescence showed anti-collagen VII antibodies. Lupus nephritis was present in two cases. Our patients were treated with corticosteroids and immunosuppressants. CONCLUSION: Bullous lupus erythematosus may be the first sign of systemic lupus erythematosus with severe visceral involvement, especially renal involvement, suggesting that it may be a marker of activity and prognosis.

[Haemophagocytic syndrome as a complication of acute pancreatitis during systemic lupus erythematosus]. / Syndrome hémophagocytaire compliquant une pancréatite aiguë dans le cadre d'un lupus érythémateux systémique.

BACKGROUND: Acute pancreatitis and haemophagocytic syndrome (HS) are rarely seen in systemic lupus erythematosus (SLE). PATIENTS AND METHODS: We report the case of a young female patient without any noteworthy prior history, who was hospitalised for abdominal pain associated with acute pancreatitis possibly related to SLE with associated haemophagocytic syndrome. DISCUSSION: Screening for cutaneous symptoms of lupus in patients with pancreatitis can help avoid diagnostic errors.

[Ulcerative colitis: exceptional consequence after rituximab therapy]. / La colite ulcérative : une conséquence exceptionnelle après traitement par rituximab.

INTRODUCTION: Possible adverse complications related to rituximab (RTX) are low, some of which are extremely rare. The authors describe one situation visibly waning exceptional treatment with RTX for SLE refractory to conventional therapies. COMMENT: The authors report a patient of 34 years followed for months for an illness in its bullous lupus, with cutaneous, articular, hematologic and immunologic. Given a corticosteroid resistance, several therapeutic background based hydroxychloroquine, cyclophosphamide and methotrexate, were initiated without any improvement. Immunomodulatory therapy type RTX was introduced to this form refractory at a rate of 375mg/m(2)/week. The waning of the second infusion, the patient presented a sudden intense abdominal pain syndrome, revealing an acute catarrhal appendicitis. At distance from the appendectomy, the consequences of which were favorable, treatment with RTX was resumed. In the aftermath of the third infusion, the patient presented in table tract marked by profuse watery diarrhea whose explorations reveal a morphological endoscopic appearance of erythematous, ulcerative colitis, reversible upon discontinuation of treatment. Histological data revealed important infiltrates composed mainly of CD8T lymphocytes. CONCLUSION: Gastrointestinal immunological consequences to the requirements of the targeted therapies deserved very careful and rigorous monitoring. However, at the slightest sign of digestive, a detailed morphological exploration is essential, to avoid in particular surgical emergency, evolution without treatment could engage in short-term vital prognosis.

[Durable efficacity and remission after treatment with imatinib mesylate for FIP1L1-PDGFRA transcript negative associated eosinophilic cardiomyopathy]. / Efficacité et rémission durables après traitement par imatinib mésylate pour une cardiomyopathie éosinophilique à transcrit FIP1L1-PDGFRA négatif.

INTRODUCTION: The cardiac involvement in hypereosinophilia remains a major cause of morbidity and mortality. Recent advances have identified new molecular mechanisms responsible for the expansion of the eosinophilic lineage, allowing a better classification of the different forms of Hypereosinophilic syndrome (HES) and especially targeted therapy. Since the discovery of the involvement of deregulated tyrosine kinases in the pathophysiology of these diseases, and particularly the identification of the fusion gene FIP1L1-PDGFRA, new molecules inhibiting specifically this signaling pathway (imatinib) were individualized, leading to dramatic therapeutic benefits in proliferative forms of HES considered before that of very poor prognosis. CASE REPORT: We report here the dramatic effectiveness of imatinib used as second line therapy for dilated cardiomyopathy revealing a hypereosinophilic syndrome in a patient in whom the search for FIP1-L1-PDGFRA fusion gene was negative. CONCLUSION: If hypereosinophilia has varied clinical and morphological outcome, its clinical consequences, particularly on heart function, are sometimes dreadful, and are not correlated either with blood eosinophil levels or with a specific etiology. We report here a case of HES lacking the FIP1-L1-PDGFRA fusion gene showing that despite the absence of this molecular defect, imatinib mesylate may have therapeutic interest in those cases of HES resistant to first line therapies.

[Efficacity of rituximab in hemolytic anemia with cold autoantibodies case]. / Efficacité exemplaire du rituximab dans un cas d'anémie hémolytique à autoanticorps froids.

INTRODUCTION: Autoimmune hemolytic anemia with cold autoantibodies or cold agglutinin disease is a rare chronic disorder in which no treatment has, until now, evidence of its effectiveness. CLINICAL CASE: We report a patient who successfully responded to rituximab for a cold agglutinin disease refractory to conventional therapy with very good tolerance and a complete remission. CONCLUSION: There are only few observations that have been reported in the literature regarding the efficacity of rituximab in the treatment of cold agglutinin disease. This promising therapy could, in the future, constitute a real alternative.

[Severe rhabdomyolysis revealing a myopathy linked to autoimmune hypothyroidism]. / Rhabdomyolyse sévère révélant une myopathie hypothyroïdienne d'origine auto-immune A propos de deux cas.

While muscular manifestations are common of hypothyroidism, hypothyroid myopathy is most often limited to myalgia, muscular stiffness and cramps with, in some patients, elevated levels of muscle enzymes. We report two cases of rhabdomyolysis related to hypothyroid myopathy. One of the patients developed acute renal failure. Thyroid hormone replacement therapy improved thyroid and renal function with involution of rhabdomyolysis. Hypothyroidism appears to be an authentic cause of rhabdomyolysis and should be carefully ruled out in all patients with elevated serum levels of muscle enzymes.

[An historical case of malignant hyperparathyroidism with unusual metastatic sites]. / A propos d'une hyperparathyroïdie maligne historique à localisations métastatiques inhabituelles.

We report a historical case of hyperparathyroidism in a young patient hospitalized for an array of osteolytic foci and incomplete fracture associated with a swollen neck, revealing a very special form of a metastatic parathyroid carcinoma with unusual multiple locations and exceptional medullary flooding. Carcinoma of the parathyroid gland produces a malignant hypersecreting tumor particularly difficult to diagnose. Treatment of this rare tumor is primarily surgical. The preoperative syndrome is unusually severe primary hyperparathyroidism. Intraoperatively, the size of the tumor and its local extension to surrounding tissue are highly suggestive. Confirmation requires pathological analysis of the operative specimens and can be further supported by the clinical course of local recurrence or metastasic spread. Specific immunohistochemical techniques have recently been shown to be contributive. The diagnosis is strengthened in the presence of associated Schantz and Castelman criteria. Foci of local extension can be identified preoperatively with ultrasound, (99m)Tc-sestamibi scintigraphy and MRI of the neck and mediastinum. The prognosis depends mainly on the possibility of achieving complete resection at the initial surgery. In some cases, very aggressive complementary postoperative radiotherapy is likely to improve locoregional control of the tumor. Chemotherapy alone or in combination with radiation has not demonstrated its effectiveness. The disease course and control can be monitored by regular assay of serum calcium and the parathormone.

[Tuberculous tenosynovitis: an uncommon manifestation]. / Ténosynovite tuberculeuse: une localisation rare.

INTRODUCTION: Synovial sheath involvement of the foot is a rare location of tuberculosis. Its diagnosis is often delayed because of the indolent and chronicity of the clinical features. OBSERVATION: We report a case of bilateral tuberculous tenosynovitis of the anterior tibial and the common extensor of the toes, with uneventful outcome with antituberculous therapy. CONCLUSION: Tuberculous tenosynovitis is uncommon but should be kept in mind in chronic tenosynovitis.

[Acute respiratory distress revealing severe pulmonary leptospirosis]. / Détresse respiratoire aiguë révélatrice d'une leptospirose pulmonaire sévère.

We return a clinical case of leptospirose revelated by a complicated febrile harp pneumopathie of a sharp respiratory distress syndrome having required a transfer in resuscitation. The goal of our article is to recall that it is necessary to think systematically about a pulmonary shape of leptospirose facing an atypical pneumopahie.

A controlled study of sacroiliitis in Behçet's disease.

The aim of the study was to evaluate the prevalence of sacroiliitis in a group of patients with Behçet's disease (BD). Pelvic X-rays of 27 patients with BD responding to the International Study Group of BD and 30 controls (15 AS and 15 sciatica) were read blind and sacroiliac involvement was graded according to the New York criteria. In a second step, patients or controls with equivocal sacroiliitis had a sacroiliac CT scan. Two patients with BD (7.4%) and all patients with AS had evident bilateral sacroiliitis (at least grade 2). One patient with BD and two patients with sciatica had equivocal sacroiliitis (grade 1). CT confirmed sacroiliitis in the two patients with BD and eliminated inflammatory sacroiliitis in the three other patients with equivocal sacroiliitis showing mild degenerative lesions. A review of the literature showed that sacroiliitis and AS are rarely associated with BD. There remains insufficient evidence to suggest that sacroiliitis is an intrinsic feature of BD and that BD belongs to the group of SpA.

Idiopathic retroperitoneal fibrosis and ankylosing spondylitis. A new case report.

The case of a 52-year-old man with retroperitoneal fibrosis and ankylosing spondylitis is described. Inflammatory low back pain and acute renal insufficiency prompted a computed tomography scan of the abdomen with contrast agent injection. A fibrous sheath surrounding the aorta and attracting the ureters toward the midline was seen, strongly suggesting retroperitoneal fibrosis. The diagnosis of ankylosing spondylitis was based on the presence of inflammatory low back pain responsive to nonsteroidal anti-inflammatory drugs, syndesmophytes at the lumbar and cervical spine, bilateral sacroiliitis, and presence of the HLA-B27 antigen. Prednisone therapy in a daily dosage of 1 mg/kg induced a marked improvement. Only nine cases of concomitant retroperitoneal fibrosis and ankylosing spondylitis have been reported. These two conditions share similarities in some of the etiologic factors and anatomic localizations, suggesting that both may stem from a predisposition to fibrotic diseases.

[Hyperthyroidism and atrioventricular block. Pathogenic hypothesis. Apropos of a case and review of the literature]. / Hyperthyroïdie et bloc auriculo-ventriculaire. Hypothèse pathogénique. A propos d'une observation et revue de la littérature.

The authors report the case of a 26-year old male patient who had Graves' disease with a first degree atrioventricular block (AVB) and intermittent episodes of a second degree AVB of the Lucciani-Wenckebach type. These disorders of conduction had features characteristic of a nodal block and disappeared after treatment of the hyperthyroidism. The pathogenesis of atrioventricular conduction disorders in hyperthyroidism remains controverted. The authors put forward the following hypothesis: under the influence of thyroid hormones in excessive amounts, the autonomic nervous system would act by reciprocal excitation and exacerbate a patent or latent hypervagotonia which was pre-existent to the hyperthyroidism. This hypothesis needs to be tested by intracardiac electrophysiological studies with atrial stimulation.

[Biermer's disease and venous thrombosis. Report of two cases]. / Maladie de Biermer et thrombose veineuse. A propos de deux observations.

INTRODUCTION: The main issue in venous thrombotic events is their etiology. Several clinical and/or biological data can be helpful in that research. In the two cases we report here, a macrocytic anemia led to the diagnosis of probably acquired hyperhomocysteinemia. FIRST CASE: a 24-year-old man was admitted for severe anemia and a superior vena cava syndrome. Biological data showed megaloblastic anemia and mild features of blood destruction that were explained by a pernicious anemia. Second case: a 35-year-old man had two deep venous thrombotic events in one year (involving the right leg, then the left leg); biological findings showed a macrocytic mild anemia that was diagnosed as a pernicious anemia. In both of the patients, deep venous thrombosis was mainly explained by a hyperhomocysteinemia that was a consequence of vitamin B12 deficiency. The two patients improved under anticoagulant treatment combined with subcutaneous vitamin B12. CONCLUSION: Pernicious anemia can cause acquired hyperhomocysteinemia, which is considered a risk factor for deep venous thrombosis. Thus, the connectivity of these conditions should remain in the practitioner's mind, especially when thrombosis occurs along with a macrocytic anemia.

[Sinus dysfunction in Basedow's disease. A case report]. / Dysfonction sinusale au cours d'une maladie de Basedow. A propos d'une observation.

UNLABELLED: INTRODUCTION AND EXEGESIS: The authors report a case of sinus node dysfunction that occurred in a 22-year-old patient with Graves' disease and disappeared after thyroidectomy. CONCLUSION: Bradycardia is uncommon in hyperthyroidism, the sinus node dysfunction would be due to thyrotoxicity-related myocarditis.

[McDuffie hypocomplementemic urticarial vasculitis. Two cases and review of the literature]. / Vascularite urticarienne hypocomplémentémique de McDuffie. Deux observations et revue de la littérature.

INTRODUCTION: Hypocomplementemic urticarial vasculitis (HUV) described by McDuffie is a rare entity recently individualized among vasculitis. We report two new cases. EXEGESIS: Case 1: a 41-year-old woman presented in 1994 with inflammatory polyarthralgia, diffuse urticaria, fever, and weight loss. Biology showed proteinuria, positive rheumatoid factor with hypocomplementemia and negative immunological tests. Skin and renal biopsies showed leukocytoclastic vasculitis and extramembranous glomerulopathy, respectively. Outcome within steroid therapy was marked by alternating clinical improvement and relapses. Case 2: a 39-year-old woman presented in 1994 with inflammatory polyarthritis, diffuse urticaria, Raynaud phenomenon, cough and dyspnea. Chest x-rays and CT scan showed interstitial fibrosis and echocardiography revealed pericarditis. Biology showed positive rheumatoid factor with hypocomplementemia and negative antinuclear antibodies. Skin biopsy showed leukocytoclastic vasculitis. Corticosteroids and cyclophosphamide improved the patient's condition. McDuffie HUV is a disease with varied systemic manifestations. Its existence is still contested by some authors. Treatment is still empirical and depends on the clinical features. It is based primarily on corticosteroids. CONCLUSION: McDuffie HUV is a defensible entity among urticarial vasculitis because of its particular clinical and biological features.

[Cardiothyrotoxicosis in the young adult in Basedow disease: report of 30 cases]. / Les cardiothyréoses de l'adulte jeune au cours de la maladie de Basedow: à propos de 30 observations.

The authors report on 30 cases of cardiothyrotoxicosis in the young adult with Basedow's disease. Cardiac arrhythmia represented the most frequent clinical form of dysfunction, mainly atrial fibrillation. Conductive disorders came second, with seven cases of first-degree atrioventricular block [AVB], one case of second-degree AVB, and two cases of sinusoidal bradycardia. Three cases of ballooning of the mitral valve were detected by echocardiography. Myocardial hypertrophy was found in one case. The authors discuss the various physiopathological hypotheses regarding conduction and myocardial hypertrophy anomalies. No cases of severe cardiac insufficiency or coronaropathy were noted, which is explained by the absence of cardiopathic antecedents and the young age of the patient population. Treatment is more complicated in the case of a preexisting cardiac event.

[Hemoptysis revealing pulmonary artery aneurysm associated with intracardiac thrombosis: a delicate anticoagulation situation (a case report of Behçet's disease)]. / Hémoptysie révélant un anévrisme de l'artère pulmonaire associé à une thrombose intracardiaque: une situation délicate d'anticoagulation (à propos d'un cas de maladie de Behçet).

Behçet's disease is a vasculitis affecting both arteries and veins. Cardiac involvement is less well known. The association of an aneurysm of the pulmonary artery and intracardiac thrombosis is rare, and a therapeutic challenge. We report the case of a 26-year-old patient hospitalized for moderately abundant hemoptysis and New York Heart Association (NYHA) class III dyspnea, which illustrates the difficulty encountered when using anticoagulants in this complex situation.

[Ocular involvement in polyarteritis nodosa: two cases]. / Atteinte oculaire au cours de la périartérite noueuse. Deux observations.

INTRODUCTION: Polyarteritis nodosa (PAN) is a systemic vasculitis with polymorphic manifestations. Ocular involvement is rare; we report two such cases. CASES: The first case was a 56-year-old man with PAN treated with corticosteroids. A year and a half later, during a disease outbreak associated with decreased visual acuity, ocular examination objectified a localized pigment epithelial detachment in the right eye. The outcome was favorable with corticoid bolus followed by azathioprine. The second case was a 41-year-old man presenting with PAN and decreased visual acuity. Ophthalmologic examination showed inferotemporal branch vein occlusion in the right eye with serous retinal detachments in the left eye. The outcome was favorable with intravenous cyclophosphamide followed by azathioprine. DISCUSSION: The incidence of ocular involvement in PAN varies from 10 to 20%. It is most often associated with vasculitis of retinal and choroidal arteries. Fluorescein angiography is the gold standard of diagnosis. Multifocal acute ischemia of the choriocapillaris is very pathognomonic. Immunosuppressant drugs are effective and should be reserved for severe forms.