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A 52‐year‐old woman developed progressive infiltrated purple and hyperpigmented cutaneous lesions in the face, thighs, armpits, chest, and abdomen evolving forone year. Histopathological examination showed large histiocytes exhibiting intact inflammatory cells in their cytoplasm (emperipolesis). Immunohistochemical analyses showed that the histiocyte population was positive for S100 and CD68, but negative for CD1a. Based on the clinical, histopathological, and immunohistochemical findings, we made the diagnosis of Rosai Dorfman disease (RDD). Our patient didn’t manifest any other extra-cutaneous involvement and all the biological and radiological investigations were normal. This form of pure cutaneous RDD (P-CRDD) with multifocal lesions has been rarely reported. RDD is very rare and hardly recognized in the absence of lymphadenopathy. The diagnosis of this entity involves a combination of histology and immunohistochemistry. To date, there is no standard treatment.

Abstract Acquired hemophilia A (AHA) is a potentially life‐threatening hemorrhagic disorder with many etiologies. We report the first case in the literature describing the association of AHA with adult‐onset Still's disease (AOSD).

Rosai-Dorfman disease (RDD) is an uncommon lymphoproliferative disorder; RDD with oropharyngeal involvement is extremely rare, especially in adults. A 65-year-old woman with a complaint of progressive dyspnoea since 2016 presented with laryngeal involvement of RDD. A laryngoscopy examination revealed two solid, polypoid masses in the subglottic region, and a laryngeal biopsy concluded chronic in ammation without signs of malignancy. A second biopsy of axillary lymph nodes was performed, supporting the diagnosis of histiocytosis. The patient was treated with corticosteroids and then lost to follow-up. In 2019, she suffered from dyspnoea and a hoarse voice. Laryngoscopy examination showed a polypoid lesion causing airway obstruction at 70% and thickening of the lateral wall of the cavum. Physical examination found left axillary and submandibular adenopathy, and computed tomography revealed thickening of the supraglottic larynx narrowing the laryngeal pathway. Lymphadenectomy with immunohistochemical analysis revealed typical protein positive S-100 histiocytes and emperipolesis. The patient was treated with high doses of corticosteroids for six weeks then these were progressively decreased. The outcome was favourable; the laryngeal lesion disappeared after two weeks of treatment.

One of the most common interstitial lung diseases in antisynthetase syndrome is nonspecific interstitial pneumonia (NSIP). A 49-year-old woman presented with slow progression exertional dyspnea, myalgia, and arthralgia. The radiological findings indicated an NSIP pattern. Autoantibodies were found to be positive, but no lung biopsy was performed. Even though corticosteroid therapy significantly improved the patient's dyspnea, the patient developed mechanic's hands, the anti-synthetase antibody (PL12) became positive, and creatine phosphokinase (CPK) levels increased. As a result, the antisynthetase syndrome was established. The patient follow-up after three years revealed an improvement in symptoms under corticosteroid therapy.

Abstract Guillain–Barré syndrome (GBS) and polymyositis (PM) are two rare autoimmune diseases, one of which affects the peripheral nervous system and other the muscle. We report the case of a young woman with no previous medical history who was hospitalized with an ascending paralysis associated with acute respiratory failure due to a GBS. The patient was treated with plasmapheresis with unfavorable outcome and permanent proximal muscular disability. The diagnosis of an associated PM was retained based on biological myolysis and the results of electromyography and muscular biopsy. To our knowledge, this association of GBS and PM has been reported only once in the literature. The search for syndromic associations in the presence of an autoimmune helps to avoid diagnostic errors.

Abstract Agranulocytosis is a rare acute condition characterized by severe a

Abstract Pulmonary artery aneurysm must be evoked in front of any hemoptysis in a patient with Behçet disease as it requires urgent immunosuppressive therapy and often surgery.

Hughes-Stovin syndrome is a very rare condition with no defined diagnostic criteria. We present the case of a 26-year-old man who had haemoptysis revealing Hughes-Stovin syndrome. We will consider the aetiology, therapeutic and evolutionary aspects of this disease.

Les pneumopathies infiltrantes diffuses (PID) constituent une manifestation fréquente des connectivites. Elles peuvent être révélatrices de la maladie ou survenir au cours du suivi. Le syndrome des anti-synthétases (SAS) est une connectivite auto-immune complexe et hétérogène. Des anticorps de type «anti synthétases», en particulier l´anticorps anti-Jo-1 caractérise ce syndrome. Le pronostic du SAS étant conditionné par la survenue d´une PID et de sa sévérité dictant ainsi la prise en charge thérapeutique du SAS. Nous rapportons l´observation d´une patiente âgée de 57 ans se présentant avec un tableau d´une PID aigue fébrile révélant le diagnostic d´un SAS. L´évolution a été favorable sous boli de corticoïdes associés au cyclophosphamide.

L´atteinte neurologique pseudotumorale au cours de la maladie de Behçet est rare. Quelques cas ont été rapportés dans la littérature. L´imagerie par résonance magnétique, montre un aspect pseudotumoral de localisation capsulothalamique. Le traitement repose sur la corticothérapie associée à un traitement immunosuppresseur type cyclophosphamide avec une bonne évolution clinico-radiologique sous traitement. Nous rapportons le cas d´une maladie de Behçet révélée par une atteinte neurologique pseudotumorale chez un homme âgé de 42 ans sans antécédents particuliers. Il présentait une hémiparésie d´installation aigue. L´imagerie par résonnance magnétique (IRM) montrait une masse capsulolenticulaire pseudotumorale se rehaussant après injection du gadolinium. L´examen clinique trouvait un aphte buccal avec une cicatrice d´aphte génital. Le test pathergique était positif. Il était traité par une corticothérapie et un traitement immunosuppresseur type cyclophosphamide avec une bonne évolution clinique et radiologique.

Le lupus érythémateux chronique est une dermatose auto-immune touchant habituellement le visage et le cuir chevelu. La localisation palpébrale sous la forme d´une blépharite chronique est rare. Nous rapportons un cas de blépharite lupique au cours d´un lupus discoïde chez une femme âgée de 42 ans.

Le pyoderma gangrenosum est une affection rare, se présentant classiquement par des ulcérations cutanées inflammatoires et douloureuses. Il peut être associé à une maladie générale, inflammatoire intestinale, ou rhumatismale comme la polyarthrite rhumatoïde (PR). Nous rapportons ici un cas de pyoderma gangrenosum survenu chez un patient suivi pour une polyarthrite rhumatoïde et rappelons les caractéristiques épidémiologiques, cliniques, thérapeutiques et évolutives de cette dermatose.

La fibromatose plantaire ou « maladie de Ledderhose » ou « Morbus Ledderhose » est caractérisée par une fibrose nodulaire progressive de l’aponévrose plantaire superficielle due à une prolifération de fibroblastes. C’est une pathologie rare et bénigne du fascia dont l’étiopathogénie reste incertaine. Nous rapportons ici un cas de fibromatose plantaire survenue chez une patiente traitée par inhibiteurs du TNFα pour une polyarthrite rhumatoïde et rappelons les caractéristiques épidémiologiques, cliniques, thérapeutiques et évolutives de cette affection.

Background: Multiple sclerosis and other demyelinating processes are sometimes difficult to differentiate from the neurological involvement in autoimmune diseases. Distinguishing multiple sclerosis from other lesions due to autoimmune diseases is crucial to avoid unsuitable or delayed treatments. Methods: Charts of 6 patients diagnosed with mimicking multiple sclerosis between 1996 and 2014 were retrospectively assessed. Results: The mean age at diagnosis was 35±7 years. The most commonly neurological manifestation at onset was paraparesis due to transverse myelopathy and uni/bilateral optic neuropathy. All our patients suffered from recurrent episodes of optic neuritis with a mean lag time of 12 months. Other initial presenting neurological manifestations in our patients included ataxic gait and pyramidal syndrome. Systemic symptoms occurred a long time before or after their initial neurological presentation. All patients had numerous T2 hyperintense lesions in the periventricular white matter and spinal cord with contrast enhancement. The antibodies tests revealed the presence of significant amounts of anti-nuclear antibodies. The anti-phospholipid antibodies were negative in all patients. All patients were treated with corticosteroid therapy and neurological features were cleared in all cases. Conclusion: Multiple sclerosis, other myelitis and optic neuritis, are sometimes difficult to differentiate from CNS involvement in autoimmune disease. Indeed, the clinical presentation, immunological profile and MRI lesions may be similar.

Tuberous sclerosis or tuberous sclerosis complex (TSC) is an autosomal dominant inherited neurocutaneous disorder that variably affects the brain, skin, kidneys, heart, and other organs. It is characterized by skin and renal lesions in addition to central and peripheral nervous system tumors, with neurological and psychiatric findings. We report such a rare case of tuberous sclerosis in a 25-year-old female who presented with abdominal pain and hypertension. Physical examination showed dermatological signs that included hypopigmented maculae, shagreen plaque, angiofibromas on the centrofacial areas, periungual fibromas on toes, and molluscum pendulum around the neck. Abdominal ultrasonography revealed bilateral renal angiomyolipoma. Brain magnetic resonance imaging showed subependymal nodules and cortical tubers. She also presented retinal and oral lesions. Our patient has a definitive diagnosis of TSC. Hypertension was related to the renal involvement of TSC, and the patient benefitted from oral angiotensin- converting enzyme inhibitors with a favorable outcome.

Le purpura rhumatoïde (PR) est une vascularite des petits vaisseaux secondaire à des dépôts d'immunoglobulines de type IgA. À travers une étude rétrospective colligeant 14 cas de PR de l'adulte en se basant sur les critères de classification du PR de EULAR/ PRINTO/PRES durant une période de 18 ans (1996 à 2014) dans le service de médecine interne du CHU Hédi Chaker de Sfax dans le sud tunisien, les caractéristiques épidémiologiques, cliniques, thérapeutiques et évolutives du PR chez nos patients adultes seront précisées. L'âge moyen des patients était de 33 ans (extrêmes: 17-64 ans) avec une prédominance masculine (sex-ratio de 1.8). Le purpura vasculaire pétéchial était constant. Les manifestations articulaires (arthralgies et/ou arthrites) étaient notées chez 9 patients (64,2%). L'atteinte digestive était présente chez 13 patients (92.8%). L'atteinte rénale était notée chez 11 patients (78.5%) révélée par une protéinurie de rang néphrotique dans 7 cas, une hématurie microscopique dans 9 cas, une hypertension artérielle dans 4 cas et une insuffisance rénale dans un cas. Le type histologique le plus fréquent était la glomérulonéphrite proliférative endocapillaire diffuse (36.3%). La corticothérapie à forte dose était instaurée chez 7 patients ayant une atteinte rénale proliférative initiée par des bolus de solumédrol dans 4 cas et associée au cyclophosphamide dans un cas. Deux patients avec une atteinte digestive sévère ont reçu une corticothérapie. Après un suivi moyen de 18,5 mois (4-36 mois), l'évolution était favorable dans tous les cas sans rechutes et l'insuffisance rénale chronique était notée dans un seul cas.

Adult-onset Still's disease (AOSD) has been recognized as a cause of fevers of unknown origin. Malignancies are the most important differential diagnoses of AOSD which has been rarely reported in association with cancer. The present paper undertakes the study of a 69-year-old Tunisian woman with AOSD according to the diagnostic criteria of Yamaguchi. She was treated by prednisone, then associated with methotrexate. 18 months later, she developed a squamous cell carcinoma treated with chemotherapy and radiotherapy.

Patiente âgée de 48 ans était hospitalisée pour une cholestase asymptomatique hépatique. Elle rapportait une histoire personnelle et familiale d'récidivante. Le bilan biologique révélait une anémie ferriprive et une cholestase modérée. Les sérologies virales ainsi que les anticorps anti tissu hépatique étaient négatifs. Le scanner abdominal objectivait de multiples shunts artério-veineux dans la région sous-capsulaire du foie. Le diagnostic d'une atteinte hépatique dans le cadre d'un Rendu Osler était retenu. Un traitement martial était prescrit et une surveillance biologique et morphologique du foie était entreprise.

Mucocele is an uncommon pathology of the vermiform appendix estimated to be seen in 0.2-0.3%. The term mucocele means dilation of the appendix due to mucus, caused either by a benign or a malignant process. Herein, we report the case of a 77-year-old man with Calcinosis, Raynaud’s phenomenon, Esophageal dysfunction, Sclerodactyly and Telangiectasias syndrome, a limited form of Scleroderma, who had presented an abdominal cyclical pain and in which abdominopelvic computed tomography scan concluded to the diagnosis of appendiceal mucocele. Surgery and histopathology confirmed the diagnosis of mucinous cystadenoma. This association appendiceal mucocele and scleoderma has not been previously reported. The clinical and radiological features of this unusual complication are reviewed.

L'ostéchondromatose synoviale est une métaplasie du tissu synovial. Elle engendre de petites masses cartilagineuses qui font saillie à la face interne de la synoviale, puis, se pédiculisent et enfin s'en détachent en développant des chondromes libres. On en distingue deux formes d'ostéchondromatose , la forme primitive, qui est rare, et la forme secondaire plus fréquente dont l'origine est généralement l'arthrose. Nous rapportons un cas d'ostéchondromatose synoviale de l'articulation carpo-métacarpienne du pouce chez une patiente atteinte d'un lupus érythémateux systémique.

L'hypercalcémie hypocalciurique familiale (HHF) est une maladie bénigne à transmission autosomique dominante, caractérisée par une hypercalcémie persistante béhigne, une hypocalciurie, et des concentrations de parathormone (PTH) normales ou modérément élevées, sans complication secondaire à l'hypercalcémie. Nous rapportons l'observation d'un patient ayant présenté une chondrocalcinose articulaire révélatrice d'une HHF. A travers cette observation nous essayons de décrire les aspects épidémiologiques, les caractéristiques cliniques, et paracliniques de cette association.

Hughes and Stovin first reported a syndrome consisting of multiple pulmonary artery aneurysms and venous thrombosis in 1959. Here, we encountered a 42-year old woman who had hemoptysis revealing a Hughes- Stovin syndrome. Helical computed tomography showed multiple pulmonary artery aneurysms with pulmonary thromboembolism. The patient was treated with steroid therapy, cyclophosphamide and anticoagulation with a good response.

L'ONA n'est pas une maladie spécifique, mais c'est l'aboutissement de diverses conditions pathologiques dont la plupart altère la circulation sanguine. Elle peut compliquer toutes les maladies systémiques et auto-immunes essentiellement le lupus érythémateux systémique. Sa survenue au cours de l'artérite de Takayasu a été exceptionnellement rapportée. Nous rapportons l'observation d'une femme âgée de 69 ans, et suivie pour artérite de Takayasu qui se présentait pour des douleurs de la hanche gauche. Un scanner de la hanche gauche avait confirmé le diagnostic d'une ostéonecrose aseptique de la tête fémorale gauche stade 3.

'Les myopathies inflammatoires (MI) sont des connectivites rares caractérisées par l'association d'un d'ficit musculaire et la présence d'un infiltrat inflammatoire au sein du muscle squelettique. Les MI regroupent trois entités principales : la polymyosite (PM), la dermatomyosite (DM) et la myosite à inclusions [1,2]. Elles sont dot'es d'un grand polymorphisme clinique et voélutif. Des d'sordres immunitaires cellulaires et humoraux sont probablement à l'origine de ces connectivites. Des progrés considérables ont à t à réalisés ces derniéres ann'es dans la compréhension de leur pathogénie. La DM se distingue par une atteinte cutan'e caractéristique. Elle est caractérisée par une atteinte primitive péri vasculaire de mécanisme humoral [2,3].

L'artérite de Takayasu ou maladie de Takayasu (MT) et la polyarthrite rhumatoïde (PR) et sont deux maladies inflammatoires chroniques et leur association a été rapportée dans la littérature à travers quelques observations de cas sporadiques. Nous rapportons une nouvelle observation d'une telle association. Une patiente âgée de 44 ans, diagnostiquée avec une polyarthrite rhumatoïde à facteur rhumatoïde positif, qui a développé des céphalées avec des vertiges de caractère permanent. L'examen révélait un pouls radial et huméral abolis à droite, un souffle carotidien bilatéral et une tension artérielle imprenable à droite. L'artériographie a confirmé la présence d'une atteinte de l'arc aortique type MT. Le diagnostic d'une maladie de Takayasu associée à une polyarthrite rhumatoïde était retenu. La patiente était traitée par une corticothérapie (prednisone à la dose de 0,5 mg/kg par jour) et un traitement de fond par Méthotrexate avec une bonne réponse initiale. A travers notre observation et une revue de la littérature, les caractéristiques épidémiologiques, étiopathogéniques, cliniques, thérapeutiques et évolutives de cette association seront discutées.

Celiac disease (CD) is a chronic digestive disease that results in hypersensitivity to the gliadin fraction of Gluten. Malabsorption syndrome may be responsible for weight loss, diarrhea, osteomalacia, and vitamins deficiency. Herein we report a patient with coeliac disease (CD) who presented with osteomalacia and psoriasis without classical symptoms of CD. A 25-year-old North African Tunisian white woman was admitted to the hospital because of a 1-year history of bone pain, weight loss and weakness. She had cutaneous psoriasis on dermatologic examination. She had also anemia, hypocalcemia and pathological fracture. She was diagnosed to have osteomalacia on the basis of clinical, biological and radiological findings. Further investigations revealed the presence of antiglutaminase antibodies, and histopathologic findings of the duodenal biopsy were consistent with celiac disease. The patient showed a fast response to gluten-free diet, and full recovery with calcium and vitamin D replacement. Coeliac disease is frequently misdiagnosed leading to major complications such as osteolamacia. In the other hand, osteomalacia can still be the presenting feature of undiagnosed celiac disease. Association between osteomalacia and cutaneous psoriasis is rarely reported.

The incidence of myositis in patients with systemic lupus erythematosus (SLE) is low among different series. Here we attempt to describe the main features of SLE/myositis overlap syndrome. We retrospectively reviewed the medical records of 174 patients with SLE seen over 15-year period. All the patients fulfilled the revised American Rheumatology Association criteria for SLE. Patients who met The Bohan and Peter criteria for definite myositis were included in this study. Among those patients, six patients had an associated myositis (3.4% overall). They were 6 women with a mean age of 29 years (20-41 years). At the initial evaluation, 3 patients (50%) were complained from myalgia, and all patients had symmetrical muscle weakness (proximal muscle weakness in 6 cases with distal muscle weakness in 2 cases). The muscle disease was severe in 1 case. Involvements of muscles of the pharynx and upper esophagus were noted in 4 patients (66.6%). The creatine kinase (CK) levels were elevated in 4 cases with a mean rate of 2153.5 UI/L. The electromyogram (EMG) revealed signs of myositis in 5 cases. Muscle biopsy, performed in 5 patients, revealed an inflammatory myopathy changes in 4 cases. Antinuclear antibodies (ANA) were positive in all cases. All our patients were treated with high doses of corticosteroids with favorable outcome. Relapse of SLE disease had occurred in 2 patients. The association SLEmyositis is rare with heterogeneous presentation. Through our observations and literature data we will specify the characteristics of this association.

Objective: To assess the rate, risk factors of adverse events (AEs) during long-term corticosteroid therapy in elders. Patients and methods: We enrolled a retrospective study of our patients over 65 year-old treated with a high corticosteroid regimen for greater than 4 weeks. Results: Seventy one elders were assessed. The most common condition was giant cell arteritis. The most common AEs were metabolic disorders (45 cases, 63.4%). There were 14 patients (19.7%) with corticosteroid induced diabetes mellitus. Corticosteroid therapy induced arterial hypertension in 10 patients (14.1%). In addition, 25 patients (35.2%) developed at least one infection. Prevailing infectious sites were skin (15 cases, 21%), lung (11 cases, 15.5%), and urinary tract (7 cases, 9.9%). Moreover, 18 patients (25.4%) developed osteoporosis. Our analysis showed that the prevalence of osteoporosis and diabetes was significantly higher in women (p = 0,001). Similarly, corticosteroid induced diabetes showed a significant correlation with blood glucose and creatinine at admission (p = 0.01). Infections were strongly correlated to the duration of high corticosteroid regimen (p = 0.035). Our analysis revealed that subjects with lymphopenia were more likely to develop urinary infections (p = 0,039). Conclusion: Long-term GC use is associated with an increased rate of serious AEs in elderly. Our study highlights different AEs in elders. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]