C. Bernier

Long-term effectiveness and safety of interleukin-1 receptor antagonist (anakinra) in Schnitzler's syndrome: A french multicenter study

The aim of this study is to assess the long-term effectiveness and safety of IL1Ra in Schnitzler syndrome (SchS). Between 2010 and 2012, we performed a nationwide survey among French internal medicine departments to identify SchS patients. We retrospectively analyzed the long-term efficacy and safety of IL1Ra and the outcome of patients that did not receive this treatment. Forty-two patients were included in the study, 29 of whom received IL1Ra. The mean age at disease onset was 59.9years. Disease manifestations included urticaria (100%), fever (76%), bone/joint pain (86%), bone lesions (76%), anemia (67%), and weight loss (60%). The monoclonal gammopathy was overwhelmingly IgM kappa (83%). The mean follow-up was 9.5years (range: 1.6-35). Two patients developed Waldenströms macroglobulinemia and one developed AA amyloidosis. All of the 29 patients who received IL1Ra responded dramatically. After a median follow-up of 36months (range: 2-79), the effectiveness remained unchanged. All patients remained on anti-IL-1 therapy. Twenty-four patients (83%) went into complete remission and five (17%) into partial remission. Three patients experienced grade 3-4 neutropenia. Six patients developed severe infections. No lymphoproliferative diseases occurred while on IL1Ra. When last seen, all patients without anakinra had an active disease with variable impact on their quality of life. Their median corticosteroids dosage was 6mg/d (range: 5-25). IL1Ra is effective in SchS, with a sharp corticosteroid-sparing effect. Treatment failures should lead to reconsider the diagnosis. Long-term follow-up revealed no loss of effectiveness and a favorable tolerance profile. The long-term effects on the risk of hemopathy remain unknown.

Therapeutic Patient Education in Atopic Dermatitis: Worldwide Experiences

Therapeutic patient education (TPE) has proven effective in increasing treatment adherence and improving quality of life (QoL) for patients with numerous chronic diseases, especially atopic dermatitis (AD). This study was undertaken to identify worldwide TPE experiences in AD treatment. Experts from 23 hospitals, located in 11 countries, responded to a questionnaire on 10 major items. Patients in TPE programs were mainly children and adolescents with moderate to severe AD or markedly affected QoL. Individual and collective approaches were used. Depending on the center, the number of sessions varied from one to six (corresponding to 2 to 12 hours of education), and 20 to 200 patients were followed each year. Each centers education team comprised multidisciplinary professionals (e.g., doctors, nurses, psychologists). Evaluations were based on clinical assessment, QoL, a satisfaction index, or some combination of the three. When funding was obtained, it came from regional health authorities (France), insurance companies (Germany), donations (United States), or pharmaceutical firms (Japan, Italy). The role of patient associations was always highlighted, but their involvement in the TPE process varied from one country to another. Despite the nonexhaustive approach, our findings demonstrate the increasing interest in TPE for managing individuals with AD. In spite of the cultural and financial differences between countries, there is a consensus among experts to integrate education into the treatment of eczema.

Therapeutic patient education in children with atopic dermatitis: position paper on objectives and recommendations

Poor adherence is frequent in patients with atopic dermatitis (AD), leading to therapeutic failure. Therapeutic patient education (TPE) helps patients with chronic disease to acquire or maintain the skills they need to manage their chronic disease. After a review of the literature, a group of multispecialty physicians, nurses, psychologists, and patients worked together during two international workshops to develop common recommendations for TPE in AD. These recommendations were structured as answers to nine frequently asked questions about TPE in AD: What is TPE and what are its underlying principles? Why use TPE in the management of AD? Who should benefit from TPE in AD? How can TPE be organized for AD? What is the assessment process for TPE in AD? What is the evidence of the benefit of TPE in AD? Who are the people involved in TPE? How should TPE be funded in dermatology? What are the limits of the TPE process?

Different patterns of skin manifestations associated with parvovirus B19 primary infection in adults

BACKGROUND Skin involvement is reported during primary parvovirus B19 infection in adults. OBJECTIVES We sought to describe the cutaneous presentations associated with parvovirus B19 primary infection in adults. METHODS We conducted a descriptive, retrospective, multicenter study. The patients included (>18 years old) had well-established primary infections with parvovirus B19. RESULTS Twenty-nine patients were identified between 1992 and 2013 (17 women, 12 men). The elementary dermatologic lesions were mostly erythematous (86%) and often purpuric (69%). Pruritus was reported in 48% of cases. The rash predominated on the legs (93%), trunk (55%), and arms (45%), with a lower frequency of facial involvement (20%). Four different but sometimes overlapping patterns were identified (45%): exanthema, which was reticulated and annular in some cases (80%); the gloves-and-socks pattern (24%); the periflexural pattern (28%); and palpable purpura (24%). LIMITATIONS The limitations of this study were its retrospective design and possible recruitment bias in tertiary care centers. CONCLUSION Our findings suggest that primary parvovirus B19 infection is associated with polymorphous skin manifestations with 4 predominant, sometimes overlapping, patterns. The acral or periflexural distribution of the rash and the presence of purpuric or annular/reticulate lesions are highly suggestive of parvovirus B19 infection.

Élaboration d’un référentiel d’éducation thérapeutique dans le psoriasis

INTRODUCTION Psoriasis is a chronic inflammatory skin disease which can cause significant impairment of quality of life, absenteeism at work and significant psychological distress. This justifies the elaboration of a multidisciplinary education program for patients. The objective of this work was to develop the content of a therapeutic education program in psoriasis, which may serve as a basis for teams wishing to develop psoriasis therapeutic education in their community. PATIENTS AND METHODS A group of 15 health professionals (dermatologists, dermatology nurses, and psychologist) and four psoriasis patients representatives of the psoriasis patient association (Association pour la lutte contre le psoriasis) participated in the development of this program. Health professionals all had an experience in therapeutic patient education in psoriasis through prior participation in a multicenter open pilot study, evaluating a therapeutic education program in psoriasis. Based on the previous experience, preparatory work in subteams was initiated to prepare draft objectives and content of the program. A two-day meeting was then organized to discuss in depth content of the therapeutic education program and elaborate recommendations. The meeting structure combined subteam work and plenary sessions. The following program was elaborated: two individual sessions and three group sessions. The groups have worked for two days, according to a predefined pattern: interview guide of educational diagnostic, content of collective workshops and knowledge questionnaire. All these documents were validated in plenary session. The methodology used for the development of this program followed the recommendations of the HAS in the field of chronic disease. RESULTS In the end, were retained three collective workshops, preceded by a consultation of individual educational diagnosis and knowledge questionnaire followed by an evaluation session at the end of the program. The interview guide for educational diagnosis and the knowledge basis questionnaire have been defined. Three themes of group workshops were defined: (1) understanding the disease, (2) understanding the mechanism of onset of disease and treatments available, (3) how to live with psoriasis in everyday life. For each workshop, were defined learning objectives, skills to acquire and how to get there. DISCUSSION We describe here a framework of educational therapy program in psoriasis comprising educational objectives, skills to acquire, basic disease knowledge, suitable for patients with psoriasis. The content was tailored to patient language and knowledge based on feedback from participating patients. The list of skills may be adapted to patients individual needs. This program serves primarily as a working basis for the caregiver, to standardize practices in terms of therapeutic education in psoriasis in France.

Érythème cutané télangiectasique après implantation d’une pompe à morphine

BACKGROUND Reticulated telangiectasic erythema is a cutaneous complication reported after in the implementation of pacemakers and cardiac defibrillators. Here we report a case occurring after the implantation of a morphine pump. PATIENTS AND METHODS Reticulated and pigmented telangiectasic erythema was observed at the morphine pump implantation site in a 56-year-old man presenting chronic lower back pain. DISCUSSION To date, cutaneous telangiectasic erythema has been reported following implantation of cardiac defibrillators and pacemakers although the physiopathogenesis of the condition is poorly understood. An identical case was recently described following implantation of morphine pump, as in our case. We discuss hypotheses concerning the possible physiopathogenesis of such lesions.