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Sarcoidosis is a multi-system granulomatosis of unknown etiology, defined by the presence of epithelioid and gigantocellular granulomas, without caseous necrosis. Ocular sarcoidosis manifests mainly as bilateral granulomatous anterior uveitis. Occlusion of the central retinal vein in sarcoidosis is a rare manifestation, which is the particularity of our observation.

Introduction. Pure extra-thoracic sarcoidosis is rare and poorly characterized in the literature. Herein we present features of pure extra-thoracic sarcoidosis, in comparison with sarcoidosis with intra-thoracic involvement, in a population from the south of Tunisia.

Introduction. The prognosis of dermatomyositis (DM)/ polymyositis (PM) in adults is partly related to their association with neoplasia. The aim of our study was to report the epidemiologic, clinical, paraclinical, therapeutic and evolutionary aspects of DM associated with malignancy in patients from Sfax, south eastern of Tunisia. Methods. A retrospective cohort study of patients with DM or PM admitted in Dermatology and Internal Medicine Departments of Hedi Chaker University Hospital of Sfax between 1996 and 2015. Cases of DM or PM associated with malignancy were retained. Results. Seventeen cases (13.5%) of DM or PM associated with malignancy were noted. Fourteen patients had DM and 3 patients had PM. The Sex ratio M / F was 0.3 and the mean age at diagnosis was 56.5 years. In DM patients, malignancy preceded the myositis in 64.2% of cases. In PM patients, only one patient was known to have breast cancer and the myositis revealed the cancer for 2 others. Treatment consisted of corticosteroids associated with methotrexate in 4 cases. Outcome was fatal in 5 cases (29.4%), due to the underlying cancer in 3 cases. Swallowing disorders related to DM/PM were responsible for death in 2 cases. Conclusion. There are no specific clinical or biological features in paraneoplastic DM. In our series, breast neoplasm represented the first cancer associated with DM. Cancers of nasopharynx, colon and urinary tract had the second position.

Optic neuritis (ON) may be associated to a range of autoimmune or infectious diseases. We report herein a case of ON induced by Rickettsia conorii. A 53-year-old woman presented with a recent decrease in visual acuity and headache. ON was diagnosed on the basis of ophthalmologic examination and flash visual evoked potentials. Etiological investigation made in our department eliminated first autoimmune disorders (vasculitis and connective tissue diseases). Rickettsial optic neuritis was confirmed by detection of specific antibodies in serum and the negativity of other serologic tests. An association between corticosteroids and cyclines was prescribed with improvement of visual acuity.

Renal involvement is rarely reported and little is known about the renal morpho-logic changes in the idiopathic hypereosinophilic syndromes. We reported a case of a 52-year-old man with idiopathic hypereosinophilic syndrome associated with membranous glomerulopathy. The eosinophilia and the proteinuria greatly improved with corticosteroid treatment. We review the literature regarding the nephropathy of this hematologic disorder.

L’ostéome ostéoïde est une tumeur osseuse bénigne qui affecte les adultes jeunes et se localise préférentiellement au niveau des os longs. La localisation intra-articulaire est rare et atteint le plus souvent la hanche. La symptomatologie clinique est alors atypique et peut faire errer le diagnostic constituant un défi diagnostique pour les cliniciens. Nous rapportons deux observations d’ostéome ostéoïde intra-articulaires de la hanche chez deux hommes âgés 24 et 45 ans, révélés par des douleurs de la hanche gauche de type inflammatoire évoluant depuis un an et un an et demi respectivement. Chez les deux patients, le tableau atypique de l’ostéome ostéoïde a été à l’origine d’un retard diagnostic. La tomodensitométrie est dans cette indication l’examen le plus spécifique qui a permis d’évoquer le diagnostic d’ostéome ostéoïde. Une fois le diagnostic est posé, l’exérèse chirurgicale à ciel ouvert a permis la guérison avec disparition totale des douleurs. L’examen histologique a confirmé le diagnostic final d’ostéome ostéoïde intra-articulaire dans les deux cas.

Introduction: Extra-intestinal mainly articular features of inflammatory bowel disease (IBD) are common and may precede the IBD. Several well-documented reports have revealed that axial manifestations are the most frequent type of inflammatory arthritis which develops in patients with IBD. The frequent association of a number of autoimmune diseases in the same patient has also been described. However, RA associated with IBD is rare and the underlying mechanism remains unknown. Case Presentation: This report describes a 53-year-old woman with ulcerative colitis (UC) and who was diagnosed 14 years after the onset of IBD with RA, based on clinical, radiological and immunologic findings. Conclusion: Because of the paucity of data in this field, we reviewed the cases of overlap RA-IBD and we discussed in this article its treatment stemming from few reports. [ABSTRACT FROM AUTHOR]

Liposarcoma, a rare malignant soft tissue tumour, is a particular form of sarcoma that develops from fatty tissue. It is most commonly seen in men and often occurs on the lower extremities. We present the case of a 71 year old patient who presented with a progressive soft tissue mass on the right thigh for 7 months. Clinical examination revealed a firm, slightly painful mass on the inner side of the right thigh measuring 10 × 15 cm not adherent to the underlying bony planes. Magnetic resonance imaging (MRI) of the thigh showed a well-limited intramuscular fatty mass measuring 96 × 80 mm axially and 180 mm in height with T1 and T2 hyper signal with a drop in signal on the sequences with suppression of the fat and cancellation of the signal on the STIR sequence. It is crossed by enhanced septa and vascular structures. This MRI appearance initially suggested a well-differentiated liposarcoma of the right thigh. This diagnostic hypothesis was confirmed by anatompathological and immunohistochemical examination of a biopsy of the tumour. Treatment consisted of surgical removal of the tumour followed by radiotherapy. [ABSTRACT FROM AUTHOR]

Objective Biclonal gammopathies (BGs) are rare situations characterized by the production of 2 monoclonal proteins. There are no available data on BGs in North Africa. We aimed to estimate the prevalence of BGs in our population and describe their clinical and laboratory features. Methods We conducted a 31-year retrospective study including patients with persistent double monoclonal bands based on the results of immunofixation/immunoelectrophoresis. Results A total of 35 patients with available clinical data (sex ratio, M/F = 1.53; mean age, 70 ± 10.87 years [range, 45–90 years]) were included. The main associated conditions were multiple myeloma (MM) (40%), BG of undetermined significance (BGUS) (34%), and lymphoproliferative diseases (23%). Only one-third of the patients had 2 monoclonal spikes on serum protein electrophoresis. The most common paraprotein combinations were immunoglobulin (Ig)G-IgG (25%) and IgG-IgA (23%) with different light chains in one-half of the cases. The mean follow-up was 25.6 months (median, 12 months). No BGUS evolved into a malignant disease. Conclusion BGs are rare in clinical laboratory routine but must be accurately identified by the pathologist. Our cohort is characterized by a high prevalence of BGUS compared with MM. [ABSTRACT FROM AUTHOR]

Relatively recently, the concept that immunoglobulin G4 (IgG4)‐related disease is a distinct chronic inflammatory disorder rather than a subset of Sjögren's syndrome has been accepted. IgG4‐related disease (IgG4‐RD) is a fibro‐inflammatory systemic immune‐mediated condition that can affect the pancreas, salivary glands, and lymph nodes. Almost every organ may be impacted synchronously or metachronously by this illness, which causes the development of sclerotic masses of varying sizes. Numerous other rheumatic diseases can present with characteristics of IgG4‐RD, making it difficult to distinguish between them. However, cases of IgG4‐RD involving the bilateral orbits, and pancreas with biological abnormalities are rare. We present a case of an 18‐year‐old female with vitiligo since the age of 3 years who presented with acute pancreatitis and acalculous cholecystitis, bilateral orbital masses, palpebral edema, and eosinophilia. The patient was diagnosed with IgG4‐RD in keeping with clinical presentation and the elevated serum IgG4 level and after elimination of other differential diagnoses. The patient's symptoms gradually relieved after glucocorticoid therapy. This case presents an uncommon combination of clinical features infrequently reported in the literature. Multi‐organ IgG4‐RD is a multisystemic mass, commonly creating diagnostic challenges for clinicians. Furthermore, and more importantly, it highlights the need to keep a differential of IgG4‐RD in mind, to aid in the early and correct treatment of the disease. [ABSTRACT FROM AUTHOR]

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