A. Sparsa

Incidence and Mortality of Bullous Pemphigoid in France

A major increase in the incidence of BP has been recently reported in the United Kingdom. In addition, there are some controversies about the over-mortality of BP patients. The primary objective was to reevaluate the incidence of BP in France as compared with that we estimated 15 years ago. The secondary objective was to assess mortality of BP patients. BP incidence was retrospectively estimated from all BP cases diagnosed between January 2000 and December 2005 in three French regions with a total population of 3.858 million inhabitants. BP mortality was assessed from a prospective cohort accrued during the same time period. A total of 502 incident BP patients (mean age: 82.6±8.8 years) were identified. Overall estimated incidence was 21.7 cases per million persons per year (95%CI:19.8-23.7 cases per million persons per year), which is about 3-fold higher than the incidence that we estimated 15 years ago. In the population aged 70 years or above, BP incidence was 162 cases per million per year (95%CI:147-177 cases per million per year). The overall 1-year survival rate was 62% (95% CI: 56-67%). The risk of death for BP patients was more than six times greater than that for the general population (SMR:6.60; 95%CI:5.47-7.90). The incidence of BP in France has increased 3-fold in the last 15 years. BP is associated with high mortality.

Risk factors for bullous pemphigoid in the elderly: a prospective case-control study.

A rise in the incidence of bullous pemphigoid (BP) was documented recently in Europe, and the main risk factors for BP remain unknown. We conducted a multicenter case-control study to evaluate risk factors for BP. We identified 201 incident BP cases and 345 controls individually matched for age, gender, center, and place of residence (home, nursing home, or extended-care facility). We used univariate and multivariate logistic regression analyses to compare drugs used for over 3 months, comorbidities, and physical and cognitive impairments between cases and controls. Mean age of BP patients was 84.2 (±8.7) years. Factors independently associated with BP by multivariate analysis were major cognitive impairment (odds ratio (OR), 2.19; 95% confidence interval (95% CI), 1.24-3.87), bedridden condition (OR, 2.19; 95% CI, 1.23-3.89), Parkinsons disease (OR, 2.16; 95% CI, 1.09-4.27), unipolar or bipolar disorder (OR, 5.25; 95% CI, 1.21-22.86), and chronic use of spironolactone (OR, 2.30; 95% CI, 1.20-4.46) or phenothiazines with aliphatic side chains (OR, 3.70; 95% CI, 1.21-11.34). Chronic analgesic use was associated with a lower risk of BP (OR, 0.49; 95% CI, 0.30-0.81). Thus, risk factors for BP include neurological disorders, particularly dementia and Parkinsons disease, psychiatric disorders (unipolar and bipolar disorders), bedridden condition, and chronic use of several drugs.

Role of drug exposure in aphthous ulcers: a case–control study

Background Drug‐induced aphthous ulcers have been the subject of several isolated and heterogeneous case reports for the last three decades.

Treatment of ocular cicatricial pemphigoid with the tumour necrosis factor alpha antagonist etanercept.

Sir, Cicatricial pemphigoid (CP) is a rare autoimmune subepithelial blistering disease that predominantly affects the mucous membranes with scarring (1). The disease is characterized by the involvement of muco-cutaneous sites, among them ocular invo-lvement, which can lead to blindness. Mild forms of the disease may be controlled by anti-inflammatory drugs, such as dapsone, whereas severe forms, and particularly ocular involvement, usually require the use of immunosuppressive therapies (2). As tumour necrosis factor (TNF)-α plays a role in the fibrosing process, its targeting strategies are promising in the management of ocular CP. Here we report a case of ocular CP treated successfully with the TNF-α antagonist etanercept.

Ischemic Digital Ulcers Affect Hand Disability and Pain in Systemic Sclerosis

Objective. Ischemic digital ulcers (DU) are frequent and severe complications of systemic sclerosis (SSc). The purpose of our study was to assess the effect of DU on hand disability and pain in patients with SSc. Methods. The Evaluation of the Impact of Recurrent Ischemic DU on Hand Disability in Patients with SSc (ECLIPSE) is a prospective, multicenter, noninterventional study with a 2-year followup. Patients with SSc who experienced at least 1 DU in the previous year and received bosentan therapy were included between October 2009 and March 2011. This cohort is described at the time of inclusion. Results. There were 190 patients (132 females) from 53 centers. Mean age ± SD was 43 ± 15 years at SSc diagnosis and 53 ± 15 years at inclusion. In 105 patients (56.2%), DU were the first non-Raynaud symptoms of SSc. The mean time interval between the occurrence of Raynaud phenomenon and the first DU episode was 6.6 ± 9.1 years. The mean numbers of active DU and fingers affected per patient for both hands were 2.3 ± 1.8 and 2.2 ± 1.6, respectively. Presence of active DU at inclusion was significantly associated with pain and impaired hand function: Visual Analog Scale for pain (0 to 10) was 6.2 ± 2.6 versus 2.5 ± 2.4 (p < 0.0001) and Cochin Hand Function Scale for hand disability (0 to 90) was 38 ± 20 versus 25 ± 19 (p < 0.0001), respectively. Conclusion. DU represent a major sign of SSc, often affecting multiple fingers and both hands. They are significantly associated with pain and hand disability.

Predictive Clinical Features of Eczema Craquelé Associated with Internal Malignancy

Objective: To describe a series of hospitalized patients with eczema craquelé (EC) and the possible correlations between clinical features of EC and cancer in an open prospective observational study. Patients and Interventions: The study population comprised 68 consecutive patients included between January 1, 1999 and December 31, 2000 who were followed up through December 2004. All patients who had localized or generalized EC were included. Patients underwent complete clinical examinations, routine laboratory tests, chest x-rays, abdominal ultrasound, and cutaneous biopsies performed on EC. Main Outcome Measures and Results: Rates of EC associated with cancer, clinical features of eczema, rate of recalcitrant eczema, relationship to other clinical prognostic factors, and paraneoplastic evolution were evaluated. Cancer was diagnosed in 32 patients (47%). We observed a significant difference in the presenting clinical signs of EC between patients with malignant tumors and patients without cancer. In patients with malignancies, EC was widespread on the trunk and we noted deep red and inflammatory fissures. In all cases, EC led to the discovery of malignancy or recurrence of cancer. Conclusion: Widespread EC, topical corticosteroid resistance, and deep red and inflammatory fissures were significantly correlated with neoplasia.

High prevalence and risk factors of thromboembolism in stage IV melanoma

Background  Patients with cancer are at a high risk of thromboembolism (TE), which contributes to morbidity and mortality. Several case reports of thromboembolic events have been reported in patients with melanoma in the literature.

Use of bosentan for digital ulcers related to systemic sclerosis: a real-life retrospective French study of 89 patients treated since specific approval

Objectives: Ischaemic digital ulcers (DUs) are a common complication of systemic sclerosis (SSc). This study aimed to characterize patients with SSc and ongoing DUs treated with the endothelin receptor antagonist bosentan in clinical practice in France. Method: An observational, retrospective, longitudinal study was conducted in 10 French expert centres. Medical records from randomly selected adult SSc patients who received treatment with bosentan for DU prevention from March 2007 to December 2010 were analysed. The primary objective was to determine the profile of patients at treatment initiation. Secondary objectives were to monitor bosentan dosing, treatment schedule, and reasons for treatment termination. Results: The study included 89 patients (mean age 52 years, 69% female, 44% diffuse cutaneous SSc). At bosentan treatment initiation, the mean duration of Raynaud’s phenomenon was 15 ± 12 years, and the mean time since first episode with DU was 6.5 ± 7 years. Most patients had a history of at least two episodes with DUs, separated by < 12 months (61%), and had received intravenous iloprost (63%). Previous DU complications included auto-amputation (8%), surgical amputation (6%), osteitis (6%), and gangrene (4.5%). Active smokers (25%) had a history of significantly more surgical amputation (p = 0.004) and osteitis (p = 0.004) than non-smokers. At least one active DU at bosentan initiation was detected in 82% of patients. Bosentan was used according to prescription guidelines and was well tolerated; six patients (7%) withdrew from treatment because of raised liver enzymes. Conclusions: Patients treated with bosentan for DU prevention in France have severe, refractory, ongoing ulcerative disease. Active smoking was correlated to a history of DU complications. Tolerance of bosentan was comparable to previous studies.

Traitement de la maladie de Paget extramammaire par photothérapie dynamique topique

BACKGROUND The usual treatment for extramammary Pagets disease (EMPD) is surgery, but this approach may have grave functional and physical consequences, as well as high recurrence rates. Topical photodynamic therapy (PDT) offers an optional approach for EMPD; it has a high complete response rate and there is no dose restriction. The aim of this study was to evaluate the efficacy and safety of PDT in the treatment of EMPD. PATIENTS AND METHODS This series of patients was seen at a single centre between 1 December 2005 and 31 December 2010. All patients with histologically confirmed EMPD were included. Patients received two courses of PDT 21 days apart: 3 hours after topical application of methyl aminolevulinic acid emulsion, they underwent illumination with red light (570-670 nm) at a dose of 37 J/cm(2) for 10 minutes. In the event of relapse, a further cycle was given at week 6. RESULTS Eight patients (seven female, one male) of a mean age of 69 years were included. After two series of two illuminations, seven patients were in complete clinical remission at 3 months and one patient was in partial remission. Five patients were still in complete clinical remission at 6 months. All patients had relapsed after a mean 8.4 months (4-14 months). The limiting factor appears to be pain occurring during illumination. Patients reported satisfaction with the disappearance of symptoms and a notable improvement in quality of life. DISCUSSION The complete clinical response rate to PDT at month 6, after two series of two illuminations, was equivalent to that for surgery. Although the recurrence rate was high, this treatment may be repeated without functional or physical consequences. PDT resulted in disappearance of pain and improved quality of life.

Acquired ichthyosis with pravastatin

1 Mashiah J, Brenner S. The acceptable addiction. Rapid response. e-published Feb 2003, Brit Med J, E journal. 2 Galindo PA, Borja J, Mur P, Feo F, Gomez E, Garcia R. Anaphylaxis to paracetamol. Allergol Immunopathol 1998; 26: 199–200. 3 Mahboob A, Haroon T. Drugs causing fixed eruptions: a study of 450 cases. Int J Dermatol 1998; 37: 833–839. 4 Wohl Y, Goldberg I, Sharazi I, Brenner S. A case of paracetamol induced acute generalized exanthematous pustulosis in a pregnant woman localized in the neck region. Skinmed 2004; 3: 47–49. 5 Halevy S, Cohen AD, Grossman N. Clinical implications of in vitro drug-induced interferon gamma release from peripheral blood lymphocytes in cutaneous adverse drug reactions. J Am Acad Dermatol 2005; 52: 254–261.