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Tabolli S.,Health Services Research Unit | Pagliarello C.,University of Parma | Paradisi A.,Health Services Research Unit | Cianchini G.,IDI IRCCS | And 2 more authors.
British Journal of Dermatology | Year: 2014

Background Studies conducted using different tools have invariably observed that physical and mental components of health status are seriously compromised in patients with pemphigus. An improvement in quality of life (QoL) has been commonly observed over the treatment period. Objectives The aim of the study is to verify whether the patients' wellbeing is affected by pemphigus also in absence of cutaneous and mucosal lesions. Materials and methods The clinical records of 203 patients were analysed. A total of 47 patients were without bullae/erosions and reported a score = 0 for both the Patient Global Assessment and the Ikeda index. In order to assess the QoL we used the Skindex-17 and the 12-item General Health Questionnaire (GHQ-12). Results Patients without bullae/erosions had a better QoL when compared with patients with active lesions. This difference, with a reduction of approximately 30% of the Skindex-17 scores in the patients without lesions, was statistically significant, for both the symptoms and the psychosocial scales. The proportion of patients at risk of anxiety/depression (GHQ-positive cases) was 44% lower in patients without lesions compared with patients with lesions. In a multiple linear regression model the presence of bullae/erosions negatively influences QoL with an average increase of Skindex-17 symptoms and psychosocial scale scores of 11·7 and 10·6 points, respectively. Female patients had a statistically significantly worse QoL than males on the symptoms but not on the psychosocial Skindex-17 scales. Conclusions While patients without lesions reported a better QoL than patients with bullae/erosions, their Skindex-17 scores remained elevated. Dermatologists should be aware that a clearing of the skin manifestations does not mean 'perfect health' for the patient. What's already known about this topic? Pemphigus has a severe impact on quality of life. Patients with pemphigus have an increased risk of anxiety and depression. Following treatment, pemphigus may have long periods of quiescence. What does this study add? Quality of life is significantly impacted also in patients without bullae/erosions, and in women it is worse than in men. High percentages of patients are at risk of anxiety and depression even when the disease is quiescent. The burden of disease continues to be present even during periods of relative wellness. © 2014 British Association of Dermatologists. Source

Zulian F.,University of Padua | Martini G.,University of Padua | Vallongo C.,University of Padua | Vittadello F.,University of Padua | And 11 more authors.
Arthritis and Rheumatism | Year: 2011

Objective Juvenile localized scleroderma is a chronic progressive fibrotic disorder of the skin that causes permanent disability and aesthetic damage. This study was undertaken to assess the safety and efficacy of methotrexate (MTX) in the treatment of juvenile localized scleroderma. Methods In this double-blind study, patients with active juvenile localized scleroderma were randomized (2:1) to receive oral MTX (15 mg/m2, maximum 20 mg) or placebo once weekly, for 12 months or until treatment failure. Both groups received oral prednisone (1 mg/kg/day, maximum 50 mg) for the first 3 months. A target lesion was evaluated clinically, with infrared thermography and using a computerized scoring system with skin score rate (SSR) evaluation. Response to treatment was defined as the absence of new lesions, SSR â1, and a decrease in lesion temperature of at least 10% compared to baseline. Treatment failure was defined as the occurrence of new lesions, SSR >1, or increased lesion temperature. All analyses were done on the intent-to-treat population. Results Of the 85 patients screened, 70 (ages 6-17 years) were randomized (46 to the MTX group, 24 to the placebo group). The mean disease duration was 2.3 years. After an initial response in all patients, disease relapsed in 15 MTX-treated patients (32.6%) and 17 placebo-treated patients (70.8%) (P ;lt& 0.005). New lesions appeared in 3 MTX-treated patients (6.5%) versus 4 placebo-treated patients (16.7%). The mean SSR decreased from 1 to 0.79 in the MTX group and increased from 1 to 1.1 in the placebo group, and the mean target lesion temperature decreased by 44.4% in the MTX group versus 12.1% in the placebo group. Twenty-six patients in the MTX group (56.5%) and 11 patients in the placebo group (45.8%) developed mild side effects related to treatment. None of the side effects were severe enough to necessitate treatment discontinuation. Conclusion Our findings indicate that MTX is efficacious in the treatment of juvenile localized scleroderma and is well tolerated. Copyright © 2011 by the American College of Rheumatology. Source

Serra V.,University of Rome Tor Vergata | Castori M.,University of Rome La Sapienza | Paradisi M.,IDI IRCCS | Bui L.,University of Rome Tor Vergata | And 3 more authors.
American Journal of Medical Genetics, Part A | Year: 2011

Heterozygous mutations in TP63 cause a wide spectrum of autosomal dominant developmental disorders variably affecting skin, limbs, and face. TP63 encodes p63, a protein expressed in two main isoforms (Tap63 and ΔNp63) with critical roles in both cell differentiation and development. Some analyses suggest a relationship of the mutation site to the observed clinical picture, although this link is inconsistent. This suggests an appreciable phenotypic continuity within the TP63-related disorders. We report a 3-month-old boy ascertained for congenital scalp erosion and mild features of ectodermal dysplasia. His mother showed full-blown characteristics of Rapp-Hodgkin syndrome plus intense abdominal and popliteal freckling. Molecular investigation identified the novel TP63 mutation c.1697delG. We used a luciferase reporter assay to compare the effects on the p63 transactivation (TA) activity of c.1697delG with that of the p.Arg280Cys and p.Gln634X mutations, associated with ectrodactyly-ectodermal dysplasia-cleft lip/palate syndrome and isolated split hand/foot malformation, respectively. These results demonstrated complex behavior of c.1697delG in the TA of genes involved in epidermal differentiation and development and shed further light in the physiopathology of TP63-related disorders. © 2011 Wiley Periodicals, Inc. Copyright © 2011 Wiley Periodicals, Inc. Source

Carbone T.,Laboratory of Experimental Immunology | Nasorri F.,Laboratory of Experimental Immunology | Pennino D.,Laboratory of Experimental Immunology | Donnarumma M.,University of Naples Federico II | And 4 more authors.
European Journal of Dermatology | Year: 2010

Lichen planus is an inflammatory disease of the skin and mucous membranes characterized by vacuolization of basal keratinocytes associated with a prominent junctional lymphocyte infiltrate which comprises T lymphocytes, NK cells, myeloid and plasmacytoid dendritic cells. Basal keratinocyte damage is considered as being a consequence of a lymphocytic cytotoxic attack, mostly mediated by perforin+CD8+ T lymphocytes. NK cells have been described to infiltrate inflamed skin and significantly contribute to the amplification of immune-mediated skin diseases, thanks to their cytotoxic activity and the release of pro-inflammatory cytokines. Here, we investigated the characteristics and functional properties of NK lymphocytes involved in lichen planus. Double staining immunohistochemistry showed a considerable number (6.42 ± 2.2% of the total cellular infiltrate) of CD3 -CD56+ cells in early lichen planus lesions, mostly distributed in the papillary dermis and at the epidermal-dermal interface. Skin NK cells isolated from lichen planus lesions belong to the CD56 highCD16- subset, are highly positive for perforin and natural cytotoxic receptors NKG2D and NKp44, and, in accordance with their phenotype, are negative for KIRs receptors CD158a and CD158b. Skin CD56 highCD16- NK cells display a CCR6+CXCR3 +CCR5+ChemR23+ chemokine receptor asset for homing into inflamed skin. In terms of cytokine release, skin CD56 highCD16- NK cells are able to secrete IFN-γ, TNF-α and hardly release IL-22, IL-17 and IL-4. Overall, our data propose a proinflammatory role of NK lymphocytes in lichen planus. Source

Mazzotti E.,IDI IRCCS | Mozzetta A.,Servizio di Psicologia Clinica e Psicoterapia Psicosomatica | Antinone V.,Servizio di Psicologia Clinica e Psicoterapia Psicosomatica | Alfani S.,Laboratorio Of Ricerca Sui Servizi Sanitari | Abeni D.,Laboratorio Of Ricerca Sui Servizi Sanitari
Journal of the European Academy of Dermatology and Venereology | Year: 2011

Background Pemphigus vulgaris is a chronic disease not currently curable. Physical involvement and the impact of chronic therapies can lead patients to changes in psychological and relational areas. Objectives To provide a measure of psychological distress, dysfunctional investment in one's appearance, and their relationship. Methods Self-administered questionnaires (ASI, HADS) were given to 74 pemphigus inpatients. Results Strong associations between psychological distress and dysfunctional investment in one's appearance were observed [adjusted odds ratio (OR) = 7.36, 95% confidence intervals (CI) 1.20-45.11; OR 5.38, 95% CI 0.81-35.87, respectively, for appearance stereotyping and body-image vulnerability], together with a perceived high disease severity (OR 6.03, 95% CI 1.90-23.46). Conclusions Our results are compatible with the idea that some forms of psychological distress could be in part due to one's perception of his/her own body image. © 2010 European Academy of Dermatology and Venereology. Source

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