Manifestations cutanées associées aux gammapathies monoclonales

Whatever their aetiology, monoclonal gammopathies can be associated to several clinical features. Mechanisms are various and sometimes unknown. Skin is frequently involved and may represent a challenging diagnosis. Indeed, skin manifestations are either the presenting features and isolated, or at the background of a systemic syndrome. Our objective was to review the various skin manifestations that have been associated with monoclonal gammopathies.     

Traitement des calcinoses sous-cutanées des connectivites

Calcinosis cutis constitutes a heterogeneous group of chronic disorder. It can be associated with disturbance of calcium and/or phosphate metabolism (metastatic, tumor calcinosis, calciphylaxis) but may also develop without any metabolic disorder, in particular during the course of connective tissue diseases. Among these, the most common are dermatomyositis and the limited form of systemic sclerosis. The physiopathology of calcinosis cutis is poorly known. It can cause pain, chronic ulcerations, infections, which are sources of sometimes major disability. Treatment of calcinosis is challenging because no drug has been shown to be reliably effective in stopping the progression or decreasing dystrophic calcifications in controlled trials. Calcium blocker and colchicine are generally prescribed as the first line systemic therapy. In the localized forms of small lesions, surgical excision is often effective and sometimes preceded by local treatments (laser therapy, extracorporeal shock wave lithotripsy, topical sodium thiosulfate, etc.) or systemic treatment (minocycline, warfarine). When calcinosis is disseminated, it may require additional treatments (aluminium hydroxyde, bisphosphonates) possibly associated with surgery in case of large lesions. Time to response may be prolonged from weeks to months. The calcinosis cutis can lead to secondary infection, pain and functional disability that have to be prevented.     

Manifestations cutanées des thérapies ciblées

Many cutaneous adverse events have been identified with recently developed targeted treatments. Some of them are common and specific, like paradoxical psoriasiform eruptions with anti-TNFα, papulopustular eruptions and paronychias with EGFR inhibitors and peculiar hand-foot skin reactions with multitargeted kinase inhibitors sorefenib and sunitinib. Patients treated with these recently available biologics need a careful monitoring.     

Manifestations systémiques extracutanées du psoriasis pustuleux généralisé

Generalized pustular psoriasis of the von Zumbusch type is a severe form of psoriasis characterized by disseminated pustular skin lesions with high fever and hyperleukocytosis. We report a 32-year-old woman who presented a generalized pustular psoriasis with extra-cutaneous manifestations of the disease that included pulmonary involvement, aseptic arthritis, jaundice, and liver abnormalities. The extra-cutaneous manifestations of generalized pustular psoriasis should be known to physicians caring for patients with psoriasis in order to avoid diagnostic delay.     

Poussées de dermatite atopique : définitions et causes

Atopic dermatitis (AD) is by definition a chronic pruritic skin disease characterised by a history of flares and remissions. Genetic studies have recently demonstrated a link of causality between an impaired permeability barrier defect and both AD and mucosal allergic disorders, putting the skin stratum corneum at the centre of the pathophysiology of these disorders. The respective importance of flare factors is difficult to sort out in published papers. Some of them, such as temperature, irritants and aeroallergens have a demonstrated impact on the permeability barrier anomaly, based on epidemiological or experimental evidence. However, a better methodology and terminology are needed to identify the most significant flare factors and promote an efficient prevention.     

Valves aortiques percutanées

As the French population is aging, a growing number of elderly patients with symptomatic severe aortic stenosis are going to require therapeutic management. Approximately 30 to 40 % of these patients do not undergo surgical treatment and a high proportion is considered to be at high risk for surgery. The transluminal aortic valve implantation technique has been increasingly used over the past few years, thus providing a solution to a major therapeutic issue in our industrialized countries. Thanks to major randomized studies such as the PARTNER US trial, this treatment has been approved for high-risk patients. Expertise and new devices are now opening the way to intermediate risk population.     

L’infarctus du myocarde au Maroc : les données du registre FES-AMI

Background

Acute myocardial infarction is the most dangerous complication of coronary atherothrombosis. There are several disparities in regard to its management around the world. The aim of this study is to analyze the specificities of management of acute myocardial infarction in Morocco.

Methods

FES-AMI (Fès Acute Myocardial Infarction) is a prospective monocentric registry conducted in cardiology department of Hassan II university hospital in Fès. In this registry, we enrolled patients with acute myocardial infarction who presented within 5 days after symptom onset.

Results

From January 2005 to August 2015, we enrolled 1835 patients. Seventy-five percent of patients were males and mean age was 60 years old. Fifty-one percent of patients were smokers, 27% were hypertensives and 14% were diabetics. Sixty-six percent of patients had more than 2 risk factors. Time from symptom onset to hospital admission was less than six hours for 40% of the patients. Thirty-six percent of patients were admitted more than twelve hours after the onset of chest pain. Only 37% of patients received reperfusion therapy, 31% with in-hospital thrombolysis and 6% with primary angioplasty. In-hospital mortality was 7.6%.

Conclusion

The patients enrolled in our registry have late presentation of acute myocardial infarction and less rate of reperfusion therapy. Furthermore, the majority of our patients have multiple risk factors and this result underlines the failure of preventive interventions.