Syndrome de Sweet : étude rétrospective de 47 cas

Purpose

Also called acute febrile neutrophilic dermatosis, Sweet's syndrome is an inflammatory disorder with a prominent cutaneous expression. It is characterized by a variety of manifestations, clinical and histological findings. The objective of this study was to describe their clinical, pathological and therapeutic characteristics.

Methods

We report on a series of 47 patients who presented a Sweet's syndrome, collected in our institution in Tunis between 1997 and 2011.

Results

The patient population consisted of 11 men and 36 women. The mean age was 47 years with extreme ranging from 28 to 74 years. An associated disorder was observed in ten patients: inflammatory disease (three cases), inflammatory bowel disease (two cases), tuberculosis (three cases) and diabetes (three cases). One case of pregnancy was observed. Cutaneous lesions consisted of erythematous plaques or nodules. Lesions were located mainly on the upper or lower extremities. All biopsy specimens demonstrated a dermal infiltrate composed predominantly of neutrophils. Fibrinoid necrosis and intramural inflammation were observed in eight cases.

Conclusion

The skin disorder can precede, follow, or appear concurrent with the diagnosis of an associated disease which requires careful surveillance.     

Efficacité du rituximab dans un cas de syndrome de Lambert-Eaton non paranéoplasique séronégatif

Introduction

Lambert-Eaton myasthenic syndrome is a rare and autoimmune presynaptic disorder of the neuromuscular junction, due in 85% of cases to autoantibodies directed against voltage-gated calcium channels. It is a paraneoplastic disorder in 50 to 60% of cases. Diagnosis involves a proximal muscle weakness and areflexia, associated with a significant increment after post-exercise stimulation in electrophysiological study. Symptomatic treatment is based on 3,4-diaminopyridine. No etiological treatment has proven its efficacy in both paraneoplastic and non-paraneoplastic Lambert-Eaton myasthenic syndrome.

Case report

We report a 41-year-old man who presented with a seronegative non-paraneoplastic Lambert-Eaton myasthenic syndrome in whom conventional immunosuppressive treatments (corticosteroids, azathioprine) failed, and who eventually improved after treatment with rituximab.

Conclusion

Rituximab was an effective and well-tolerated treatment in this case of seronegative non-paraneoplastic Lambert-Eaton myasthenic syndrome. Its indication should be discussed when conventional immunosuppressive therapy fails in both seropositive and seronegative patients.     

Lichen plan buccal érosif associé à un syndrome de Good

Introduction

Good syndrome is characterized by thymoma, hypogammaglobulinemia, low number of peripheral B cells, and variably, peripheral CD4 T cell lymphopenia and inverted CD4/CD8 T cell ratio, associated with infections and autoimmune diseases.

Case report

We report an 85-year-old woman who presented with oral erosive lichen planus and thymic tumor. Improvement of oral erosive lichen planus was observed after resection of a benign thymoma, but was ineffective on the immunodeficiency syndrome. Only 11 patients with Good syndrome and lichen planus have been previously reported and are reviewed.

Conclusion

The association of oral erosive lichen planus with Good syndrome is rare, but does not seem to be fortuitous.     

Lymphome intravasculaire révélé par un syndrome d’activation macrophagique

Introduction

Intravascular large B cell lymphoma is a neoplastic cell proliferation leading to the occlusion of the lumen of small vessels. This is a rare haematological malignancy, which is difficult to diagnose because of a heterogeneous clinical presentation.

Case report

We report a 62-year-old man who presented a macrophage activation syndrome as the presenting manifestation of an intravascular lymphoma. This association is frequently marked by a greater severity and clinical care requires an early and appropriate treatment.

Conclusion

Due to the polymorphism and the systemic presentation of intravascular large B cell lymphoma, the internist may be confronted with this disease, which is considered to be more severe if associated with a macrophage activation syndrome. Awareness of the intravascular large B cell lymphoma is important because the prognosis depends on the rapidity of the initiation of chemotherapy associated with rituximab.     

Un cas de syndrome de Hoigné lors d’une injection intraveineuse de ceftriaxone

Introduction

Hoigne's syndrome is characterized by the development of acute clinical manifestations which are mainly psycho-sensorial. Classically, these features immediately follow the injection of procaine penicillin G.

Case report

We report a 59-year-old man who presented with psycho-organic manifestations that occurred just after the intravenous injection of ceftriaxone; to our knowledge, this is the first case of Hoigne's syndrome reported after an injection of this antibiotic.

Conclusion

The pathophysiologic basis of this syndrome is still unknown. It is important to keep in mind its clinical characteristics, which may mimic immuno-allergic symptoms. It should be differentiated from anaphylactic manifestations because Hoigne's syndrome allows the continuation of the treatment.     

Fasciite avec éosinophilie (syndrome de Shulman) : à propos de 11 patients

Purpose

Eosinophilic fasciitis or Shulman's disease is a rare condition of unknown etiology.

Methods

We report a retrospective case series of 11 patients with eosinophilic fasciitis (seven men and four women, including a single pediatric case) and perform a systematic literature review to determine the main features of this disease.

Results

Mean age of the patients was 46 years. Subcutaneous induration of limbs observed in all the patients was the major presenting symptom. The induration was atypically located in the chest area in two patients. Blood eosinophilia was absent in five cases. Histological fasciitis was demonstrated in all patients and eosinophilic infiltration was present in seven patients. Relapse of subcutaneous induration was observed in only one patient who gradually developed systemic sclerosis.

Conclusion

Diagnosis of eosinophilic fasciitis should be considered in the presence myalgia and subcutaneous induration of limbs, blood eosinophilia and hypergammaglobulinemia. Treatment is based on systemic corticosteroids.     

Hépatite E aiguë associée à un syndrome de Guillain-Barré chez un patient immunocompétent

Introduction

Hepatitis E virus (HEV) infection is now recognized to be an emerging autochthonous disease in several countries. There have been several reports of neurological manifestations associated with HEV infections. Immunocompromised patients seem to be particularly vulnerable.

Case report

We report a 73-year-old man who presented with an acute polyradiculopathy and an acute hepatitis. HEV RNA was positive in serum and cerebrospinal fluid. Serum antiganglioside antibodies were also detected. Liver function tests returned to normal rapidly and HEV RNA was undetectable 4 weeks after initial testing. The neurological features improved gradually with the use of intravenous immunoglobulins.

Conclusion

We report a case of Guillain-Barré syndrome related to acute hepatitis E in an immunocompetent patient. The outcome was favorable after intravenous immunoglobulins administration. HEV screening should be systematic in patients who present with an acute polyradiculopathy and abnormal liver function tests.     

Syndrome MYH9 et anémie hémolytique auto-immune : une association fortuite ?

Introduction

The MYH9 syndrome is a group of rare autosomal dominant platelet disorders associating in most of the cases a macrothrombocytopenia and characteristic leukocyte inclusions. Clinical features may include renal, visual, or hearing impairment. The bleeding tendency is usually moderate.

Case report

We report a 28-year-old-man, with an auto-immune haemolytic anaemia associated with a MYH9 syndrome.

Conclusion

To our knowledge, this is the first report of such an association.     

Pseudomaladie de Still et néoplasie : une observation de mélanome et revue de la littérature

Introduction

A malignancy must be carefully excluded before ruling in the diagnosis of adult onset Still's disease (AOSD). However, an occult or poorly symptomatic malignancy can easily be overlooked.

Case report

We report a 50-year-old female patient who presented with features of adult onset Still's disease (AOSD), in fact heralding a malignant melanoma with fatal outcome since discovered lately, at a metastatic stage. In retrospect, the only significant atypical feature was cholestatic hepatitis, which soon disappeared upon institution of glucocorticoid treatment. The literature review identified 27 additional cases of AOSD-like disease associated with malignancy published since 1980 including solid cancer in 61% of the cases (especially breast and lung) and haematological malignancies in 39% of the cases (especially malignant lymphoma). The interval between OASD-like symptoms and malignancy averaged 8 months, and AOSD most often preceding malignancy. Although idiopathic AOSD and neoplastic AOSD-like disease are often indistinguishable initially, some features could point toward the latter: an onset of AOSD after the age of 40 years, the presence of atypical clinical, biological, or immunological features in less than one third of the cases, and a poor response to NAIDS or systemic glucocorticoids in 61% of the cases.

Conclusion

Making the differential diagnosis of malignancy-associated AOSD in a timely fashion remains a primary goal, even in the most typical cases and those showing good initial therapeutic response.     

Syndrome des antisynthétases et adénocarcinome pulmonaire : une association fortuite ?

Introduction

Dermatomyositis and polymyositis are sometimes associated with neoplasia. Conversely, a link between antisynthetase syndrome and neoplasia has not been clearly demonstrated.

Case report

We report a 54-year-old smoker male patient who presented with an antisynthetase syndrome with anti-Jo1 and anti-Ro-52 antibodies. An adenocarcinoma of the lung was diagnosed at the same time.

Conclusion

Two recent studies showed that patients with an antisynthetase syndrome associated with anti-Jo1 antibodies have more severe prognosis than antisynthetase syndrome associated with other antibodies (i.e. PL7/PL12). The risk of cancer occurrence seems to be increased when the anti-Jo1 antisynthetase syndrome is associated with anti-Ro-52 antibodies. To date, there is no demonstrated association between antisynthetase syndrome and neoplasia.     

Syndrome d’encéphalopathie postérieure réversible induit par un décongestionnant nasal contenant de la pseudo-éphédrine

Introduction

Posterior reversible encephalopathy syndrome is a clinico-radiological entity characterized by neurologic symptoms in association with usually reversible bilateral posterior hemispheric oedema on neuroimaging. Many pathological conditions and treatments have been associated with this syndrome.

Case report

We report a 19-year-old woman, followed-up for hypocomplementemic urticarial vasculitis, who presented with a posterior reversible encephalopathy syndrome induced by the intake of an over-the-counter cold remedy containing pseudoephedrine. Clinical manifestations and radiological abnormalities resolved after anti-hypertensive therapy and withdrawal of sympathomimetic drug.

Conclusion

The diagnosis of posterior reversible encephalopathy syndrome should be considered in patients with compatible clinical and radiological presentation because of its potential reversibility with an appropriate management. Intake of drugs, including over-the-counter cough and cold drugs, should be looked for in the history as well as autoimmune disorders.     

Maladie de Kikuchi-Fujimoto : une présentation atypique

Introduction

Histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease) is a rare clinical entity characterized by the association of enlarged lymph nodes in the posterior cervical region and fever. The disease is more frequent in young women.

Case report

We report a 41-year-old African patient who presented with atypical features of Kikuchi's disease including cutaneous lupus, haemophagocytosis, and lymphocytic meningitis. The ethnic origin and the clinical presentation were initially suggestive of tuberculous meningitis. However, microbiological analyses remained negative, histological findings were suggestive of Kikuchi's disease and HHV6 DNA integration was documented in our patient.

Conclusion

Kikuchi's disease should be suspected in an African patient when lymphocytic meningitis is associated with enlarged cervical lymph nodes, hemophagocytosis and HHV6 DNA integration.     

Tratamiento antirretroviral de inicio en pacientes infectados por el virus de la inmunodeficiencia humana en España: decisiones con relación a características inmunovirológicas específicas (estudio PERFIL-es)

Introduction

The purpose of Perfil-es study was to identify the proportion of patients starting ARV treatment based on NNRTIs or PI/r, and to identify the variables involved in the therapeutic decision-making in standard clinical practice.

Methods

An observational restrospective study performed in 65 Spanish hospitals.

Results

Was a total of 1,687 starts: 53% with NNRTI-based regimen and 42% with PI/r, and of the 642 patients analyzed, 72% had a CD4 count < 350 cells/μl.

Conclusion

The initiation of ARV treatment is still late in Spain. NNRTIs are the more frequent choice, although PI/r plays an important role.     

Les myocardites aiguës compliquant les maladies inflammatoires chroniques de l’intestin : à propos de deux observations

Introduction

Acute myocarditis is one of the less common extra-intestinal manifestations of inflammatory bowel diseases (IBD). It may reveal IBD or occur during the follow-up.

Case reports

We report two patients who presented an acute myocarditis associated with ulcerative colitis that reveal the disease in one of them. Clinical features at presentation were non specific and both patients presented with chest pain. Diagnosis was obtained with cardiac MRI. Outcome was favourable in the two patients.

Conclusion

Etiologies of myocarditis are various and the diagnosis of extra-intestinal manifestations related to IBD remains a diagnosis of exclusion. Cardiac MRI is an important diagnostic tool. Colchicine and acétylsalicylic acid are commonly prescribed in a first therapeutic approach.     

Valoración de anticuerpos con reactividad cruzada patógeno-huésped en pacientes con diferentes estadios de cardiopatía chagásica crónica

Introduction and objectives

Trypanosoma cruzi infection has been shown to induce humoral autoimmune responses against host antigens tissues. Particularly, antibodies cross-reacting with myocardial antigens may play a role in the development of the severe forms of chronic Chagas disease. The aim of this study was to determine the association between clinical stage of the disease and the presence of autoantibodies in patients with chronic Chagasic disease.

Methods

We performed a cross-sectional study in T. cruzi-seropositive patients divided into 3 groups according to the classic classification of chronic Chagas heart of Storino et al. All participants underwent complete clinical examination and their sera were used to measure autoantibody levels.

Results

All patients had detectable levels of anti-p2β and anti-B13 autoantibodies but none had anti-Na-K-ATPase antibodies. No association was observed between electrocardiographic conduction disturbances and autoantibody levels. Patients with chronic Chagas disease stage III had the highest levels of anti-B13 antibodies and a high risk of mortality score, showing a clear association between disease stage and this score.

Conclusions

Anti-B13 antibodies were significantly higher in chronic Chagas disease stage III patients, suggesting that these antibodies may be involved in disease progression and that they might be a useful marker of poor prognosis in terms of heart compromise. Our results also reveal an important correlation between the level of anti-B13 autoantibodies and symptomatic heart failure and/or dilated cardiomyopathy.Full English text available from:www.revespcardiol.org/en     

Syndrome d’Evans : attention aux diagnostics par excès

Introduction

Evans syndrome (ES) is characterized by the coexistence of an autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP). Despite being frequently evocated in the simultaneous presence of anemia and thrombocytopenia, this rare disease only accounts for 0.8 to 3.7% of patients with ITP or AIHA.

Case reports

We report three suspected cases of ES, diagnosed in the presence of thrombocytopenia and hemolytic anemia association, with a positive direct Coombs test in two patients. Standard ES treatment failure and occurrence of aditional features subsequently led to correct diagnosis to thrombotic thrombocytopenic purpura, myelodysplastic syndrome with AIHA, and ITP with hemorrhagic anemia, respectively.

Conclusion

Bicytopenias, even in an immunological context, are not sufficient to ascertain ES diagnosis. Our cases illustrate the diagnostic difficulties that may arise in daily practice, and induce over-diagnosis of this rare disease.     

Patrón de inicio del seguimiento clínico en los pacientes con infección por el virus de la inmunodeficiencia humana en España

Objetive

To describe linkage to care among new HIV diagnoses in Spain; and to estimate factors associated to linkage to care within three months after diagnosis.

Methods

The distribution of the time elapsing between the date of HIV diagnosis and the date of first determination of CD4 (considered to be the date of linkage to care) was calculated among new HIV diagnoses in 2010 in the seven Autonomous Regions participating, where data on date of CD4 count was available. Linkage to care was considered «correct» if done within three months after diagnosis. Factors associated to correct linkage to care were estimated using logistic regression.

Results

A total of 1769 new HIV diagnoses were included. Of them, 83.1% had evidence of linkage to care within a year, and 75.7% were linked within three months after diagnosis. Being an injectable drug user (IDU) was the only factor inversely associated with linkage to care within 3 months (OR = 0.3; 95% CI: 0.2-0.6).

Conclusion

In Spain linkage to care after HIV diagnosis is good, but there is still room for improvement, especially among IDUs.