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Psoriasis is well known to affect negatively daily activities, occupational and sexual functioning. We investigated whether the improvement of skin lesions obtained with the new biological drugs comes with by a better Quality of Life (QoL) as well.Thirty patients with moderate to severe psoriasis treated with either anti-CD11 (13) or anti-TNF-α molecules (17) were studied. The severity of skin lesions was evaluated, before the biological therapy (T0) and after 6 months of treatment (T1), by the Psoriasis Area and Severity Index (PASI) and the Body Surface Area (BSA). QoL was evaluated by the Dermatology Life Quality Index (DLQI) and by the Psoriasis Quality of Life (PsoriQoL).DLQI and PsoriQoL mean scores shifted significantly from 16.5 to 2 respectively. At T0, PASI did not correlate significantly with DLQI. DLQI nor PsoriQoL correlated significantly with age and with the duration of the disease. PASI correlated significantly with DLQI of patients with arthropathy only, but did not with DLQI of patients without arthropathy. At T1, PASI was not correlated significantly with DLQI nor with PsoriQoL.The patient affected by psoriasis suffer an impairment of their QoL which seems to be independent from many obvious factors, such as age, duration of the disease and, mainly, its severity. QoL does not seem to be influenced significantly by arthropathy. Other factors, of both genetic or environmental nature, could play a determinant role in depriving psoriatics of a good QoL.

Desmosomes guarantee the integrity of the epidermis, by functioning both as an adhesive complex and as a cell-surface attachment site for the keratin intermediate filaments of the cytoskeleton. Considerable progress has been made in our knowledge of desmosomes and their components. The structure and function of many of the desmosomal molecules have been determined, and a number of the molecular interactions between desmosomal proteins have been elucidated. Desmosomal proteins are major antigens in pemphigus. Each type of pemphigus has its own antigenic targets, but in the last few years it has been shown that certain autoantibody populations are not restricted to just one form of pemphigus. The production of autoantibodies against multiple intracellular and extracellular desmosomal proteins, whose pathogenic role remains to be elucidated, suggests an overlapping distribution of antibody specificities among different forms of pemphigus.

The aim of this study was to provide practical recommendations for optimizing the use of conventional and biological systemic treatments for moderate-severe chronic plaque psoriasis, particularly in case of transitioning and switching.A total number of 147 dermatologists from 33 different countries including Italy achieved consensus in providing practical recommendations for the use of conventional and biological treatments for moderate to severe psoriasis based on systematic literature review and/or expert opinion.In general, the continuous treatment regimen should be preferred in order to achieve a complete and long-term control of psoriasis. However, the treatment could be stopped or the dose reduced in case of complete disease clearance. A conventional drug could be associated to biological treatment in selected cases. Transitioning and/or switching could be considered in case of inefficacy or intolerance. A period of wash up is required if transitioning or switching is due to safety issues.This study provides practical suggestions for the optimal use of conventional and biological treatments for chronic plaque psoriasis.

Vasculitis in connective tissue disease (CTD) is quite rare, it is reported in approximately 10% of patients with CTD; systemic lupus erythematosus (SLE) shows the highest association rate. Vessels of any size may be involved, but mainly small vessels vasculitis is reported. At present the classification of these vasculitis is unsatisfactory. According to the 2012 revised International Chapel Hill Consensus Conference, vasculitides secondary to CTD are a well identified entity and are classified under the category of "vasculitis associated with systemic disease". However only lupus vasculitis and rheumatoid vasculitis are explicitly listed, while the remaining are generically included under the heading "others". Petechiae, purpura, gangrene and ulcers are the most frequent cutaneous manifestations that should investigated in order to rule out potentially dangerous systemic involvement, especially if cryoglobulinemic or necrotizing vasculitis are suspected. This review will focus on the cutaneous involvement in CTD associated vasculitis.

Four different Marine Rapid Environmental Assessment (MREA) procedures are compared with a focus on underwater acoustic performance. Co-located oceanographic-acoustic data were collected during the summer of 2015 in the Northwestern Mediterranean in the framework of a sea trial led by the NATO Centre for Maritime Research and Experimentation. The data were used to link MREA procedures and ocean-acoustic validation in a seamless framework. The MREA procedures consider Conductivity Temperature Depth (CTD) data, operational products from the Copernicus Marine Service, and two dynamical downscaling systems (with and without data assimilation). A portion of the oceanographic data are used for the assimilation procedure, and the remaining portion is withheld from the assimilation system for use as an independent verifying dataset. The accuracy of modelled acoustic properties is evaluated using the sound speed estimates from the different MREA methodologies as inputs to an acoustic model, and then comparing the modelled and observed acoustic arrival intensities and temporal structure. In 95% of the studied cases, the assimilative dynamical downscaling approach provides acoustic results equaling or exceeding in skill those modelled with the sound speed extracted from CTD casts. Acoustic assessment results indicate that our implementation of dynamical downscaling has skill at oceanographic scales of 4 km, about ten times larger than the ocean model horizontal resolution.

Pityriasis rosea (PR) is a common, self-limiting exanthematous disease associated with a systemic reactivation of human herpesvirus 6 (HHV-6) and/or HHV-7. It usually occurs in the second or third decade of life whereas it is uncommon in patients younger than 10 years. We studied the clinical features and virological parameters of 31 children with PR, comparing them with those in adults. Our findings indicate that PR presents different characteristics between children and adults, mainly consisting of time lapse between herald patch and generalized eruption, duration of the exanthem, oropharyngeal involvement and persistence of HHV-6 and HHV-7 plasma viremia. Overall, these results suggest that, following HHV-6 and/or HHV-7 systemic active infection, the pathogenetic mechanisms involved in PR may at least partly be different in children and adults.

Background: Scleromyxedema is associated with a monoclonal gammopathy and other comorbidities. Its prognostic and therapeutic features are poorly documented because most reports deal with single cases or small series. Objective: We sought to describe the characteristics of patients with scleromyxedema regarding demographics, clinical characteristics, comorbidities, therapeutic interventions, and course. Methods: We conducted a retrospective and prospective multicenter study. Results: We identified 30 patients with scleromyxedema (17 men and 13 women). The mean age at diagnosis was 59 years. The mean delay between disease onset and diagnosis was 9 months. Monoclonal gammopathy was detected in 27 patients. Extracutaneous manifestations were present in 19 patients including neurologic (30%), rheumatologic (23.3%), and cardiac (20%) manifestations. Two patients developed hematologic malignancies. The most common therapies included oral steroids and intravenous immunoglobulins. Although corticosteroids were ineffective, intravenous immunoglobulins (alone or in combination with other drugs) induced complete remission in 4 and partial remission in 9 patients with a mean treatment duration of 2 years. In all, 21 patients were followed up for a mean period of 33.5 months, at which time 16 patients were alive, 12 with and 4 without skin disease. Five patients died: 2 with dermatoneuro syndrome and 1 each with myeloid leukemia, Hodgkin lymphoma, and myocardial insufficiency. Limitations: This is mainly a retrospective study. Conclusions: Our study confirms that scleromyxedema is a chronic and unpredictable disease with severe systemic manifestations leading to a guarded prognosis. There is no specific definitive treatment. Our data support the contention that intravenous immunoglobulin is a relatively effective and safe treatment. The response is not permanent and maintenance infusions are required

Activation of blood coagulation has been demonstrated in bullous pemphigoid (BP), a rare autoimmune blistering disease, potentially leading to a prothrombotic state. In order to evaluate the incidence of venous thromboembolism (VTE) in BP, a cohort study was carried out on 432 BP patients (59 % females; median age 76 years, interquartile range [IQR]: 68-82). At diagnosis, autoimmune bullous skin disorder intensity score (ABSIS) was calculated. VTE incidence was standardised with rates of the general population. Multivariable Cox proportional hazard model was used to estimate the hazard ratio of VTE according to ABSIS and concomitant risk factors. During a median follow-up of 4.2 years, 31 objectively-diagnosed VTE events were recorded. The incidence rate of VTE (per 1000 patient-years) was 17.2 overall (95 % confidence interval [CI]: 11.1-23.2), 56.7 (95 % CI: 33.0-80.4) during acute phase (22 VTE) and 6.3 (95 % CI: 2.8-11.3) during remission (9 VTE). The standardised incidence ratio was 4.06 (95 % CI: 2.73-5.65), higher during the acute phase (14.86, 95 % CI: 9.20-21.88) than during remission (1.48, 0.66-2.63). The adjusted hazard ratio of VTE was 2.74 (95 % CI: 1.07-7.04) for ABSIS > 48 vs ABSIS < 28, and 2.56 (95 % CI: 1.00-6.70) in patients with >= 2 concomitant risk factors. In conclusion, BP patients have a 15-fold increased VTE risk during acute phase, proportional to disease severity and heightened by concomitant risk factors. Activation of blood coagulation has been demonstrated in bullous pemphigoid (BP), a rare autoimmune blistering disease, potentially leading to a prothrombotic state. In order to evaluate the incidence of venous thromboembolism (VTE) in BP, a cohort study was carried out on 432 BP patients (59 % females; median age 76 years, interquartile range [IQR]: 68-82). At diagnosis, autoimmune bullous skin disorder intensity score (ABSIS) was calculated. VTE incidence was standardised with rates of the general population. Multivariable Cox proportional hazard model was used to estimate the hazard ratio of VTE according to ABSIS and concomitant risk factors. During a median follow-up of 4.2 years, 31 objectively-diagnosed VTE events were recorded. The incidence rate of VTE (per 1000 patient-years) was 17.2 overall (95 % confidence interval [CI]: 11.1-23.2), 56.7 (95 %CI: 33.0-80.4) during acute phase (22 VTE) and 6.3 (95 %CI: 2.8-11.3) during remission (9 VTE). The standardised incidence ratio was 4.06 (95 %CI: 2.73-5.65), higher during the acute phase (14.86, 95 %CI: 9.20-21.88) than during remission (1.48, 0.66-2.63). The adjusted hazard ratio of VTE was 2.74 (95 %CI: 1.07-7.04) for ABSIS > 48 vs ABSIS

Background: Antibodies to stratified epithelia characterize chronic ulcerative stomatitis, an entity that very closely resembles erosive lichen planus both clinically and histologically. These antibodies are directed against a 70-kd antigen. Objective: Our aim was to verify whether antibodies to stratified epithelia are present in patients with common lichen planus. Patients and methods: One hundred thirty-eight patients with various forms of lichen planus were studied. Indirect immunofluorescence was performed on both monkey esophagus and HEp2-2000 cells. Immunoblotting was done with cultured keratinocytes used as the source antigen. Results: Nineteen patients had antibodies to stratified epithelia (in 9 directed against an antigen of 70 kd). Forty-eight patients had circulating antibodies detected by indirect immunofluorescence on both monkey esophagus and HEp2-2000 cells (in 7 directed against an antigen of 70 kd). Indirect immunofluorescence was positive only on HEp2-2000 cells in 21 patients. Indirect immunofluorescence was negative in 50 patients on both HEp2-2000 cells and monkey esophagus. None of the last 71 patients had antibodies directed to an antigen of 70 kd. Limitations: This is a serological study; results from direct immunofluorescence studies would be interesting. Conclusion: Antibodies to stratified epithelia directed to an antigen of 70 kd are not exclusive to chronic ulcerative stomatitis, but are also present in some patients with lichen planus. © 2007 American Academy of Dermatology, Inc.

Several cases of psoriasis associated with bullous diseases have been reported in the literature. Bullous pemphigoid is the most frequent association observed, while pemphigus-related disorders are less frequently described. The authors report a case of a patient with long-standing psoriasis who developed pemphigus foliaceous and discuss the possible relationship between the diseases.