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. Pan African Medical Journal 2012; 12:5

Fréquence, implication clinique et valeur pronostique de la lymphopénie au cours du lupus érythémateux systémique : étude cas témoin

Le lupus érythémateux systémique (LES) est une maladie auto-immune dotée d'un grand polymorphisme clinique et caractérisée par la production d'une grande variété d'autoanticorps. Sa définition repose sur les critères de l'ACR dont fait partie la lymphopénie. Afin de déterminer s'il existe une corrélation entre la présence de la lymphopénie d'une part et les manifestations cliniques, immunologiques et l'activité du lupus érythémateux systémique d'autre part, nous avons réalisé une étude rétrospective, comparative portant sur 148 cas de LES colligés dans un service de médecine interne entre 2006 et 2012. Ces patients ont été subdivisés en 2 groupes : Groupe 1 avec lymphopénie (taux de lymphocytes < 1500/mm3 ) etgroupe 2 sans lymphopénie (taux de lymphocytes > 1500/mm3 ). Les 2 groupes ont été comparés en fonction de la présentation clinique et immunologique et l'activité de la maladie mesurée par le SLEDAI (Systemic Lupus Erythematosus Disease Activity Index). L'âge moyen des patients (134 femmes et 14 hommes) était de 35,64 ans. L'atteinte hématologique était présente dans 81,1 % des cas avec une lymphopénie dans 69,2% des cas. Une association statistiquement significative était notée entre la lymphopénie et l'atteinte rénale (p=0,025), l'atteinte cardiaque (p=0,004), l'anémie hémolytique (p=0,020), la présence d'anticorps anti DNA (p=0,046), le traitement par cyclophosphamide (p=0.035) et l'activité de la maladie (p<0,01). En revanche il n'y avait pas de corrélation entre la présence de lymphopénie et les atteintes cutanées, articulaires et neuropsychiatriques. La lymphopénie est une manifestation fréquente du lupus systémique. Notre étude a démontré que sa présence est  associée à plusieurs manifestations cliniques graves dont les atteintes cardiaque et rénale. Ceci pourrait faire d'elle un outil utile dans l'évaluation du pronostic de la maladie.Key words: Lupus érythémateux systémique, lymphopénie, lymphocyte

Hypertrophic pachymeningitis as an unusual cause of headache and sphincter dysfunction in systemic lupus erythematosus

Hypertrophic pachymeningitis (HP) is an uncommon condition characterised by focal or diffuse thickening of the dura mater. An increasing number of cases have been reported of its association with underlying connective tissue diseases. It is a rare complication in systemic lupus erythematosus (SLE) and might be the initial and sole clinical manifestation. We report a case of a 21-year-old man presenting with febrile meningeal syndrome and sphincter dysfunction. Physical examination showed malar rash and joint pain. Biological assessment revealed a regenerative normocytic normochromic anaemia, a leucopenia and a lymphopenia. The 24-hour urine protein was positive at 0.6 g. Immunological evaluation revealed positive antinuclear, anti-Sm and anti-dsDNA antibodies. Brain and spinal magnetic resonance imaging showed hypertrophic pachymeningitis. Cerebrospinal fluid biochemistry was within normal limits. Renal biopsy revealed a mesangial proliferative lupus nephritis. The diagnosis of SLE with neurologic and renal involvement was established, and the patient was treated with intravenous methylprednisolone pulse, followed by oral prednisone in association with azathioprine and hydroxychloroquine. Considering the persistence of symptoms and MRI lesions after 6 months, a treatment with rituximab was initiated with good evolution

Syndrome de la personne raide associé à une dermatite herpétiforme: à propos d´un cas

Le syndrome de la personne raide (SPR) est une maladie rare affectant le système nerveux central et qui peut être d´origine auto-immune, paranéoplasique ou idiopathique. Sa présentation classique typique est caractérisée par une rigidité progressive du tronc et des membres, associée à des spasmes. Le diagnostic est soutenu par l'existence d'une activité musculaire continue et spontanée en détection à l'électroneuromyogramme, la présence d'anticorps anti-acide glutamique décarboxylase (anti-GAD) sériques, et une réponse aux benzodiazépines. Nous rapportons le cas d'un patient de 46 ans ayant une forme classique de syndrome de la personne raide auto-immune associée à une dermatite herpétiforme

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

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

Wet-cupping in the treatment of recalcitrant oral and genital ulceration of Behçet disease: A randomized controlled trial

615-618The recalcitrant oral ulceration (OU) and genital ulceration (GU) of Behçet disease (BD) can be very distressing and no standard therapy has been established yet. To determine the efficacy of Wet-cupping (W-C) as adjuvant treatment of oral and genital ulceration of BD, 24 patients were included in this prospective and comparative study. All patients included in the study had a recalcitrant oral and/or genital ulceration BD to conventional drug treatment. Treatment with W-C decreased significantly the number of episodes per month, number of ulcers per episode and duration of the episode after 06 months of conventional treatment associated with the W-C of OU and GU compared with the group treated only with conventional treatment. In patients with OU and GU, the difference in improvement ratings between therapy with W-C group and the group treated only with conventional treatment was statistically significant, 91,7%  of patients in intervention group had a complete response to oral aphtose, versus 25% of control group. For aphtosis genital response was complete in 100% cases in Intervention group, versus 25% in control group. Our results showed that combining W-C plus conventional treatment shows great promise as an effective treatment for oral and genital ulceration in patients with Behçet disease

Differential diagnosis of COVID-19 in symptomatic patients at the University Hospital Center Mohammed VI, Marrakesh

Introduction:&nbsp;coronavirus disease 2019 caused by severe acute respiratory syndrome coronavirus 2 was first reported in Wuhan, China. Clinical spectrum of this disease has nonspecific symptoms shared by many other frequent infectious diseases of the respiratory tract and other respiratory tract diseases. This study explains the importance of differential diagnosis between COVID-19 and other lung diseases. Methods:&nbsp;we analyzed in this study, the demographic features, clinical presentations, laboratory data and radiologic findings of the COVID-19 patients in comparison to those with other respiratory infections or diseases. Results:&nbsp;the mean age of all patients was 38.04 years; 35 patients were later confirmed to be positive for SARS-CoV-2 infection. The most common symptoms reported by both groups included nonproductive cough and myalgia. Two of the non-COVID-19 patients were having below 92% oxygen saturation and low systolic blood pressure. The patients shared relatively similar laboratory findings except 3% of the non-COVID-19 patients who had lympho-neutropenia and 22.6% had high levels of C-reactive protein. Pulmonary tuberculosis and autoimmune disease respiratory disorder were suspected in 2 of the non-COVID-19 patients respectively. Conclusion:&nbsp;we emphasize the importance of good screening protocols, rapid detection of SARS-CoV-2 and other most common respiratory pathogens, which may help for a better control of COVID-19 spread and avoid delayed care of other lung diseases

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 syndrom

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