Definitions and outcome measures for mucous membrane pemphigoid: recommendations of an international panel of experts.

Mucous membrane pemphigoid encompasses a group of autoimmune bullous diseases with a similar phenotype characterized by subepithelial blisters, erosions, and scarring of mucous membranes, skin, or both. Although knowledge about autoimmune bullous disease is increasing, there is often a lack of clear definitions of disease, outcome measures, and therapeutic end points. With clearer definitions and outcome measures, it is possible to directly compare the results and data from various studies using meta-analyses. This consensus statement provides accurate and reproducible definitions for disease extent, activity, outcome measures, end points, and therapeutic response for mucous membrane pemphigoid and proposes a disease extent score, the Mucous Membrane Pemphigoid Disease Area Index.

Therapy with rituximab for autoimmune pemphigus: results from a single-center observational study on 42 cases with long-term follow-up.

BACKGROUND: Rituximab induces depletion of B cells and has shown efficacy in antibody-mediated autoimmune disorders. In studies on small series of patients with pemphigus, rituximab administration results in significant improvement. However, differences in inclusion criteria, treatment protocols, and follow-up make it difficult to derive uniform conclusions. OBJECTIVES: We sought to test the efficacy and tolerability of rituximab as adjuvant therapy to corticosteroids in the treatment of pemphigus. METHODS: In all, 42 patients with pemphigus were treated with rituximab and followed up for up to 5 years. No additional immunosuppressive agents were used. Steroids were rapidly tapered. Outcomes were the proportion of patients who achieved a complete response on or off therapy, the rate of discontinuation of corticosteroid within 6 months, length of remission, time to relapses, and occurrence of adverse events. RESULTS: In all, 36 of 42 patients (86%; 95% confidence interval 75%-96%) achieved a complete response on or off therapy and discontinued steroids within 6 months from induction therapy. Six patients had a complete response off therapy with an additional infusion of rituximab 6 months after initial treatment. Twenty patients experienced a total of 34 relapses; the time to relapse was 8 to 64 months. Every relapse was treated with rituximab (500 mg) without corticosteroids, which induced a new complete response. No serious adverse events were observed. LIMITATIONS: Lack of a control group is a limitation. CONCLUSIONS: Rituximab therapy induces prolonged clinical remission in patients with pemphigus. Coadministration of other immunosuppressive agents is not necessary. Relapses can be managed with additional infusions administered on demand.

Definitions and outcome measures for bullous pemphigoid: recommendations by an international panel of experts.

Our scientific knowledge of bullous pemphigoid (BP) has dramatically progressed in recent years. However, despite the availability of various therapeutic options for the treatment of inflammatory diseases, only a few multicenter controlled trials have helped to define effective therapies in BP. A major obstacle in sharing multicenter-based evidences for therapeutic efforts is the lack of generally accepted definitions for the clinical evaluation of patients with BP. Common terms and end points of BP are needed so that experts in the field can accurately measure and assess disease extent, activity, severity, and therapeutic response, and thus facilitate and advance clinical trials. These recommendations from the International Pemphigoid Committee represent 2 years of collaborative efforts to attain mutually acceptable common definitions for BP and proposes a disease extent score, the BP Disease Area Index. These items should assist in the development of consistent reporting of outcomes in future BP reports and studies.

Quality-of-life assessment in patients with pemphigus using a minimum set of evaluation tools.

BACKGROUND: Pemphigus is a rare but severe autoimmune disease caused by autoantibodies directed against desmosomes, and clinically characterized by bullae and painful erosions of the skin and mucous membranes. The two major subtypes, vulgaris and foliaceus, are distinguished by the depth of the cleavage plane in the epidermis. Very few studies have investigated the quality of life (QoL) of patients with pemphigus, all reporting a strong impact on physical and emotional status. OBJECTIVES: We sought to achieve an exhaustive description of health status in a large sample of patients with pemphigus, assess the impact on QoL, and define a minimum set of QoL tools for clinical practice. METHODS: In all, 139 patients with pemphigus enrolled at our bullous skin diseases department from February 2007 to February 2008 were given the Medical Outcome Study 36-item short form health survey questionnaire to assess the health status, the Skindex-29 to evaluate the impact of dermatologic-specific aspects, and the 12-item General Health (GH) Questionnaire to detect patients with psychological problems. Clinical severity of the disease was assessed by a dermatologist by the Physician Global Assessment index and the Ikeda index. RESULTS: A strong impact of pemphigus on health status was observed, especially in women and older patients, and in patients with mucocutaneous involvement. A significant association between disease severity and lower Medical Outcome Study 36-item short-form questionnaire values was also observed. Patients with pemphigus showed a markedly impaired overall QoL compared with healthy control subjects on all 3 Skindex-29 scales (symptoms mean scores 37 vs 8, in patients and control subjects, respectively; emotions 37 vs 14; functioning 33 vs 4; P < .001); disease severity was also significantly associated with Skindex-29 scores, on all 3 scale scores for both Physician Global Assessment and Ikeda values (P < .05). GH Questionnaire positivity, reflecting probable minor psychiatric nonpsychotic conditions, such as depression and anxiety, was detected in 39.7% of patients. LIMITATIONS: The small sample size in the different treatment groups prevented a more detailed analysis, failing to highlight an association between treatment type and QoL impairment. CONCLUSIONS: In this study we described a strong impact of pemphigus on patients' QoL both for the dermatology-specific and the GH aspects. The prevalence of patients with GH Questionnaire positivity was also very high (almost 40%). The introduction of the proposed minimal set of QoL evaluation tools would provide additional useful information to guide clinicians in the treatment of these patients.

Strabismus in Bronzino's paintings: a hallmark of a realistic painter?

The present article analyses eleven paintings of Bronzino, one of the major painters of the late Italian Mannerism, in which the sitters are portrayed with deviating eyes. The reasons why Bronzino may have included a truant eye in his subjects are herein discussed. We consider the 'wandering' eye as a hallmark of Bronzino's style. The inclusion of strabismus may be part of the Mannerism tendency of using exaggerated hallmarks but pursuing at the same time an increasing realism that was typical of the 15th and 16th century movements.

Consensus statement on definitions of disease, end points, and therapeutic response for pemphigus.

Our scientific knowledge of pemphigus has dramatically progressed in recent years. However, despite the availability of various therapeutic options for the treatment of inflammatory diseases, only a few multicenter controlled trials have helped to define effective therapies in pemphigus. A major obstacle in comparing therapeutic outcomes between centers is the lack of generally accepted definitions and measurements for the clinical evaluation of patients with pemphigus. Common terms and end points of pemphigus are needed so that experts in the field can accurately measure and assess disease extent, activity, severity, and therapeutic response, and thus facilitate and advance clinical trials. This consensus statement from the International Pemphigus Committee represents 2 years of collaborative efforts to attain mutually acceptable common definitions for pemphigus. These should assist in development of consistent reporting of outcomes in future studies.

Clinical and instrumental evaluation of the efficacy of an emollient cream and a cleansing cream in the management of mild to moderate adulthood atopic dermatitis.

BACKGROUND: Atopic dermatitis (AD) is a chronic, recurrent, inflammatory skin disorder which may persist or directly start in adults. This is an open-label prospective study to clinically and instrumentally evaluate the effects of an emollient glycerin and paraffin-based cream and a gentle refatting cleanser in the management of mild to moderate adulthood AD. METHODS: Fifty adult patient with mild to moderate AD were recruited at the Professional Dermatology and Allergology Outpatient Clinic of the San Gallicano Dermatological Institute of Rome, between November 2016 and January 2018. The patients applied the emollient cream twice daily for 2 months. To assess the efficacy of the cream, two different areas of treatment were identified in each patient's limbs. The outcome was evaluated at 30 days (T1) and 60 days (T2) of treatment comparing to baseline (T0) by means of clinical evaluation, Dermatology Life Quality Index (DLQI) questionary, transepidermal waterloss (TEWL) and corneometry measurements. RESULTS: Clinical evaluation showed significative improvement of skin xerosis, fissuring, itching and erythema. Consistently, a significative reduction of TEWL and an improvement in skin hydration was also detected. A significative improvement of DLQI score was also detected. CONCLUSIONS: Study treatment was well tolerated and showed significative improvement of clinical and instrumental parameters evaluated. The topical daily use of an emollient glycerin and paraffin-based cream and a gentle refatting cleanser seems to be a useful tool in the treatment of mild to moderate adulthood AD improving quality of life.