Refractory Depressive Disorder as the First Manifestation of Adolescent Sjögren's Syndrome, Successfully Treated with Rituximab.

Background: The psychiatric manifestations of Sjögren's syndrome are often overlooked despite their prevalence. They can be revelatory of the disease and include anxiety, depression, dementia and, rarely, psychosis. Case description: We report a case of 18-year-old female in whom a major depressive syndrome revealed primary Sjögren's disease, with a favourable outcome after treatment with rituximab. Conclusion: The diagnostic of Sjögren's syndrome should be considered in patients who present with unexplained and refractory neuropsychiatric symptoms, even in the absence of sicca symptoms. LEARNING POINTS: Psychiatric manifestations secondary to Sjögren's syndrome are not rare but often overlooked.They can be indicative of the disease and precede systemic signs by years.The diagnostic of Sjögren's syndrome should be considered in patients who present with unexplained and refractory neuropsychiatric symptoms, even in the absence of sicca symptoms.

Haemorrhagic Pericardial Effusion as the Presenting Symptom of Scurvy.

Introduction: Vitamin C deficiency (or scurvy) usually takes weeks to become apparent as cutaneous signs and impaired wound healing. Haemorrhagic pericarditis remains a rare complication of scurvy, which has never been reported as an isolated condition. We report the case of a haemorrhagic pericarditis revealing a vitamin C deficiency in a 56-year-old patient. Case description: A 56-year-old woman presented with a 2-week history of worsening chest pain and dyspnoea, with no significant medical history. Upon admission, the patient exhibited tachycardia, tachypnoea, low blood pressure, elevated jugular venous pressure, muffled heart sounds and multiple petechiae on her lower limbs. An ultrasound revealed a large pericardial effusion, and an emergency pericardiocentesis was performed, which yielded haemorrhagic fluid without atypical cells. An initial workup including haemoculture, PT and PTT, tuberculosis workup, autoantibodies, tumour markers and infectious disease was negative. A whole-body CT scan showed no evidence of tuberculosis or lymphoma. Additional testing showed that her vitamin C level was <3 umol/L. Following stabilisation, high-dose vitamin C therapy was initiated. Subsequently, she showed continued clinical improvement and remained asymptomatic upon her discharge. Discussion: While uncommon, it is crucial to investigate vitamin C deficiency when confronted with an unexplained haemorrhagic pericardial effusion, particularly in patients with risk factors. Conclusion: Our case highlights the significance of early detection of this condition in promptly addressing the diverse complications of scurvy, thereby enhancing the prognosis of a potentially fatal condition. LEARNING POINTS: Haemorrhagic pericarditis could be an initial indication of scurvy.Vitamin C deficiency must be included in the differential diagnostic of haemorrhagic tamponade, even in the absence of a typical signs and symptoms of scurvy.

[Stiff person syndrome associated with dermatitis herpetiformis: a case report]. / Syndrome de la personne raide associé à une dermatite herpétiforme: à propos d'un cas.

Stiff person syndrome (SPS) is a rare disease affecting the central nervous system which can be autoimmune, paraneoplastic or idiopathic in origin. Its typical classic presentation is characterized by progressive stiffness of the trunk and limbs, associated with spasms. The diagnosis is supported by the existence of continuous and spontaneous muscle activity on electroneuromyogram detection, the presence of serum anti-GAD antibodies, and a response to benzodiazepines. We report the case of a 46-year-old patient with a classic form of autoimmune stiff person syndrome associated with dermatitis herpetiformis.

[Drug-induced toxidermia in a patient receiving hydroxychloroquine as systemic therapy for primary Gougerot-Sjögren syndrome: a case report]. / Toxidermie médicamenteuse sous hydroxychloroquine pour traitement systémique d'un syndrome de Gougerot-Sjögren primaire: à propos d'un cas.

We here report the case of a 41-year-old female patient with maculopapular rash occurring a week after receiving hydroxychloroquine 400 mg for primary Gougerot-Sjögren syndrome with articular involvement. The patient had more than 1-year history of idiopathic minimal glomerular lesion, effectively treated with corticosteroid therapy. Maculopapular rashes resolved after hydroxychloroquine treatment was stopped and the patient was given hydrocortisone and desloratadine. Our case highlights the importance of prescribing low dose hydroxychloroquine in subjects with a history of kidney disease as well as of raising awareness and educating patients about side effects of hydroxychloroquine.

Myasthenia Gravis Revealing Hodgkin's Lymphoma.

INTRODUCTION: Myasthenia gravis is a rare autoimmune disease caused by autoantibodies directed against the synapses of the neuromuscular junction. PATIENT AND METHODS: We report the case of a young patient with myasthenia gravis associated with Hodgkin's lymphoma. RESULTS: A 22-year-old patient presented with a 2-month history of severe weakness associated with muscle fatigability and intermittent ptosis without dysphonia or respiratory signs. Clinical examination revealed generalized myasthenia. The EMG did not show post-synaptic block, and anti-acetylcholine receptor antibodies were elevated at 3 nmol/l (normal ≤0.3 nmol/l). CT of the thorax showed an anterior medial mass. Immunohistochemistry of the mass revealed mixed cellularity Hodgkin's lymphoma. CONCLUSION: The association of lymphomas with myasthenia gravis has been rarely reported. LEARNING POINTS: Myasthenia gravis is an autoimmune disease of the neuromuscular junction and can be associated with malignant haemopathy.The coexistence of myasthenia and Hodgkin's lymphoma is very rare.This association is a therapeutic challenge.

Necrotizing Fasciitis of the Breast Underlying an Autoimmune Disease.

Necrotizing fasciitis is a serious soft tissue infection that causes necrosis of the subcutaneous tissues and the muscle fascia. It is associated with a high mortality rate of around 25%. Necrotizing fasciitis of the breast is a rare entity. It is a rapidly progressive life-threatening condition which can lead to sepsis with multiple organ failure. We describe a case of necrotizing fasciitis of the right breast in a 48-year-old patient diagnosed with systemic sclerosis-rheumatoid arthritis overlap syndrome. LEARNING POINTS: Necrotizing fasciitis of the breast is a very challenging diagnosis due to its rarity and similarity with simple breast infection.It is a life-threatening condition which can lead to systemic shock and multiple organ dysfunction syndrome and death.It may occur in patients with rheumatic diseases treated with corticosteroids and immunosuppressant drugs.

Sacral pain as the initial symptom in primary Hodgkin's lymphoma of bone.

Progressive painless enlargement of peripheral lymph nodes is the most common presentation of Hodgkin's lymphoma. The bone involvement is seen particularly in the later stages, but very rarely in early stages of the disease. Primary osseous Hodgkin lymphoma is exceedingly rare. Here, we report a case of Primary osseous Hodgkin lymphoma in a 35-year-old male presenting with osteolytic lesions of iliac and sacral bone without other symptoms. Histopathology examination revealed Hodgkin's disease. All other investigations (including computed tomography of the chest, abdomen and pelvis, bone marrow aspiration) were negative for occult disease. Polychemotherapy was started and the symptoms resolved. The case report highlights the importance of clinical suspicion of primary Hodgkin lymphoma of the bone, especially those involving the pelvis bones.

Periorbital edema as initial manifestation of chronic cutaneous lupus erythematosus.

Periorbital edema occurs frequently in dermatomyositis, but it has rarely been noted in systemic systemic lupus erythematosus. We describe a patient who developed bilateral periorbital edema and erythema as the sole manifestation of systemic lupus erythematosus.

Cerebral thrombosis complicating Crohn's disease: two cases.

Cerebrovenous thrombosis is quite rare in infammatory bowel disease. There are only a few reports of this association in the literature. We report 2 cases of patients with Crohn's disease (CD) who developed cerebral thrombophlebitis confirmed by neuroimaging. The first case was a 35-year-old man with a history of CD who presented with acute confusion. Brain magnetic resonance imaging demonstrated a left temporoparietal infarction and thrombosis of the left lateral sinus. Coagulation studies showed a marked protein S deficiency. His condition improved significantly after initiation of anticoagulant therapy. The second case was a 38-year-old woman who was admitted for a sudden loss of consciousness with tetraplegia. Brain computed tomography revealed a profound cerebrovenous thrombosis. She died within a few days after admission. Inflammatory bowel disease carries an increased risk of venous and arterial thrombosis. Although the pathogenic mechanisms of this predisposition are unclear, a possible role of inherited risk factors for thrombosis in determining this predisposition has been suggested. In these cases, both fibrinolysis and coagulation are activated as well.

Recurrent meningitis revealing a Behçet's disease.

The aim of the present study was to describe a case of Behçet's disease revealed by a recurrent meningitis and to review literature on these two conditions. We describe the case of a 25-year-old man who presented four episodes of recurrent meningitis without any locoregional cause and developed oral and genital ulcerations few months later. Behçet's disease is a chronic, multisystemic disorder with variable prevalence in different geographical areas. Its neurological manifestations are well recognized. Both central and peripheral nervous systems can be involved. Recurrent meningitis in Behçet's disease is exceptional. To our knowledge, only two cases reported recurrent meningitis as initial manifestation of Behçet's disease. This case report underscores another facet of neurological manifestations of Behçet's disease.