The Schnitzler syndrome is characterized by a chronic urticarial eruption with a monoclonal IgM gammopathy. The other signs of the syndrome include intermittent elevated fever, joint and/or bone pain with radiologic evidence of osteosclerosis, palpable lymph nodes, enlarged liver and/or spleen, elevated erythrocyte sedimentation rate, and leukocytosis. The mean delay to diagnosis is more than 5 years, and this syndrome is of concern to internists and many medical specialists. Patients with this syndrome are often initially considered to have lymphoma or adult-onset Still disease, which are the main differential diagnoses. However, hypocomplementic urticarial vasculitis, systemic lupus erythematosus, cryoglobulinemia, acquired C1 inhibitor deficiency, hyper IgD syndrome, chronic infantile neurologic cutaneous and articular (CINCA) syndrome, and Muckle-Wells syndrome should also be excluded, because diagnosis relies on a combination of clinical and biologic signs and there is no specific marker of the disease. The disease pursues a chronic course, and no remissions have yet been reported. Disabling skin rash, fever, and musculoskeletal involvement are the most frequent complications. Severe anemia of chronic disease is another serious complication. The most harmful complication, however, is evolution to an authentic lymphoplasmacytic malignancy, which occurs in at least 15% of patients. This hematologic transformation can occur more than 20 years after the first signs of the disease, thus patients deserve long-term follow-up. Treatment is symptomatic and unsatisfactory. The skin rash is unresponsive to treatment, and nonsteroidal antiinflammatory drugs, antihistamines, dapsone, colchicine, and psoralens and ultraviolet A (PUVA) therapy give inconstant results. Fever, arthralgia, and bone pain often respond to nonsteroidal antiinflammatory drugs. In some patients, these symptoms and/or the presence of severe inflammatory anemia require steroids and/or immunosuppressive treatment, which ameliorate inflammatory symptoms but do not change the course of the skin rash.

BackgroundStringent control of proteolytic activity represents a major therapeutic approach for wound-bed preparation.ObjectivesWe tested whether a protease-modulating polyacrylate- (PA-) containing hydrogel resulted in a more efficient wound-bed preparation of venous leg ulcers when compared to an amorphous hydrogel without known protease-modulating properties.MethodsPatients were randomized to the polyacrylate-based hydrogel (n = 34) or to an amorphous hydrogel (n = 41). Wound beds were evaluated by three blinded experts using photographs taken on days 0, 7 and 14.ResultsAfter 14 days of treatment there was an absolute decrease in fibrin and necrotic tissue of 37.6 ± 29.9 percentage points in the PA-based hydrogel group and by 16.8 ± 23.0 percentage points in the amorphous hydrogel group. The absolute increase in the proportion of ulcer area covered by granulation tissue was 36.0 ± 27.4 percentage points in the PA-based hydrogel group and 14.5 ± 22.0 percentage points in the control group. The differences between the groups were significant (decrease in fibrin and necrotic tissue P = 0.004 and increase in granulation tissue P = 0.0005, respectively).ConclusionIn particular, long-standing wounds profited from the treatment with the PA-based hydrogel. These data suggest that PA-based hydrogel dressings can stimulate normalization of the wound environment, particularly in hard-to-heal ulcers.

EditorThe prevalence of smoking in rosacea patients has only been examined twice 1,2 with different results. In this study, we aimed to evaluate if rosacea is a disorder predominantly affecting non-or ex-smokers.A multicentre case-control study was performed in the dermatology departments of Lorraine's hospitals, France, between January and June 2008. All patients were adults clinically diagnosed with rosacea by a dermatologist using the criteria of Wilkin et al. 3 The control group consisted of dermatological patients without rosacea. Each rosacea case was matched by age (±5 years), gender and dermatology department with one control case. All participants gave informed oral consent to be included in the study.Data collection was based on an anonymous questionnaire. The questionnaire included: (i) the patient profile items; (ii) smoking habits; and (iii) rosacea subtype and severity. 2 We defined smoking status as follows: (i) non-smokers never exposed to tobacco; (ii) smokers, active or ex-smokers; (iii) exsmokers; and (iiii) ex-smokers for more than 1 year (to avoid the short-term effects of tobacco).Univariate analysis was performed with the odds ratio (OR) method with a 95% confidence interval (CI). Pearson's chi 2 test was used to compare data. P-values of 0.05 or less were considered significant.Among 240 questionnaires, 34 questionnaires were excluded because of the absence of a matched control, incomplete responses or cases of rosacea induced by medication to avoid bias caused by drug-induced modifications. A resulting 206 questionnaires were analysed (Table 1).The prevalence of smoking among cases and controls was not different (P = 0.26). No significant difference was found between active smokers and those never exposed to tobacco (P = 0.60). The proportion of ex-smokers at the time of the study was larger in the case group than in the control group, but the difference was not significant (Table 2). When ex-smokers were defined as patients who stopped smoking for more than 1 year, the difference became significant ( Table 2).The prevalence of smoking differed with respect to the stage of the disease: 17% of smokers were present among the 46 cases of subtype 1 rosacea, 9% in subtype 2, 30% in subtype 3 and none in subtype 4. No difference could be evaluated regarding the subtypes of rosacea and smoking status because of the size of the groups.

Anthrax is an acute bacterial infection caused by Bacillus anthracis. The infection is cutaneous in about 95% of human cases and respiratory in about 5%. Approximately 2000 cases of cutaneous anthrax are reported annually worldwide. This disease became exceptional in Europe thanks to strict veterinarian monitoring. The last human cases of anthrax indicated in France were in 1997. We report 3 new related cases of naturally acquired cutaneous anthrax that occurred in France in 2008. The unique features of these cases include the atypical clinical presentation and the contribution of the rapid and specific diagnosis techniques by polymerase chain reaction.In cutaneous forms of anthrax, although the local course is not influenced by the treatment, antibiotic therapy is necessary to control any bacterial distribution. A case of exposure similar to that of a confirmed human case or an exposure identified by epidemiologic inquiry should usually result in chemoprophylaxis. Chemoprophylaxis for the close relations of a patient or for health workers is unnecessary since person-to-person transmission has not been reported.

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