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BACKGROUND: Facial (FP) and genital psoriasis (GP) significantly affect patients' quality of life. Despite the advances in treatments, limited data on efficacy and safety are available on these difficult-to-treat areas. Guselkumab is an interleukin (IL)-23 inhibitor which has been proven effective in treating patients with moderate-to-severe plaque psoriasis. OBJECTIVES: The aim of this interim analysis was to report the efficacy and safety of guselkumab in the treatment of patients with FP and/or GP. MATERIALS AND METHODS: GULLIVER is a 52-week Italian observational study to evaluate the effectiveness and safety of guselkumab in a real-life setting in patients with FP and/or GP. Adult patients with facial and/or genital moderate-to-severe psoriasis (sPGA score ≥ 3) were included. The primary endpoint of this analysis was the percentage of patients achieving a facial or genital sPGA score of 0 (clear) or 1 (almost clear), at Week 12. The change in the score of the facial or genital sPGA components in patients with a score ≥3 for each sPGA component was assessed. PASI score in patients with a baseline PASI above or below 10 was evaluated. RESULTS: Overall, 351 patients were included in the study; 83.3% of FP and 76.5% of GP patients achieved the primary endpoint. Similar response rates were observed for the facial or genital sPGA components in patients with a baseline facial or genital sPGA score ≥3 in each component. Among patients with a baseline PASI score >10, mean PASI score improved from 19.0 (SD 8.3) to 2.2 (SD 4.8). Forty-four AEs were observed in 32 patients; two mild and transient AEs (fatigue and nausea) were considered treatment related. No SAEs were observed. CONCLUSIONS: Guselkumab, showing to be effective and safe in treating FP and GP, may be a valid therapeutic option for patients with psoriasis localized in these difficult-to-treat areas.
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OBJECTIVES: Several studies indicated leukocyte telomere length (LTL) as a biomarker of multiple sclerosis (MS) evolution. This study aimed to investigate LTL in women with multiple sclerosis (MS) compared to that in healthy women (HW) across different reproductive phases, and to evaluate its relationship with MS activity. METHODS: Blood samples were collected from women with MS and HW during the fertile phase, pregnancy, and puerperium. LTL was determined using quantitative fluorescence in situ hybridization (Q-FISH). RESULTS: Blood samples from 68 women with MS (22 during fertile life, 23 during pregnancy, and 23 post-partum) and 52 HW (23 during fertile life, 20 during pregnancy, and 9 post-partum) were analyzed. During pregnancy, LTL in MS women and HW was 84.7 ± 10.5 and 77.6 ± 11.5, respectively (p < 0.005). Regression analysis showed that shorter LTL was associated with pregnancy in HW (p = 0.021); this relationship was not observed in MS women, for whom shorter LTL was related to a higher EDSS (p = 0.036). A longitudinal analysis was performed in eight MS women, showing LTL shortening from pregnancy to puerperium (p = 0.003), which was related to MS reactivation (p = 0.042). CONCLUSION: Our results highlight the possible associations between LTL, reproductive biological phases, and MS activity after delivery.
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Esclerose Múltipla , Gravidez , Feminino , Humanos , Esclerose Múltipla/genética , Hibridização in Situ Fluorescente , Período Pós-Parto , Leucócitos , TelômeroRESUMO
INTRODUCTION: Bullous pemphigoid is the most common autoimmune bullous dermatosis. In recent years several studies have tried to identify the main factors of the disease related with an increased risk of death. The aim of this multicenter Italian study was to assess the risk score of death considering epidemiologic, clinical, immunological, and therapeutic factors in a cohort of patients affected by bullous pemphigoid and try to identify the cumulative survival up to 120 months. METHODS: We retrospectively reviewed the medical records of patients with bullous pemphigoid who were diagnosed between 2005 and 2020 in the 12 Italian centers. Data collected included sex, age at the time of diagnosis, laboratory findings, severity of disease, time at death/censoring, treatment, and multimorbidity. RESULTS: A total of 572 patients were included in the study. The crude mortality rate was 20.6%, with an incidence mortality rate of 5.9 × 100 person/year. The mortality rate at 1, 3, 5, and 10 years was 3.2%, 18.2%, 27.4% and 51.9%, respectively. Multivariate model results showed that the risk of death was significantly higher in patients older than 78 years, in presence of multimorbidity, anti-BP180 autoantibodies >72 U/mL, or anti-BP230 > 3 U/mL at diagnosis. The variables jointly included provided an accuracy (Harrel's Index) of 77% for predicting mortality. CONCLUSION: This study represents the first nationwide Italian study to have retrospectively investigated the mortality rates and prognostic factors in patients with bullous pemphigoid. A novel finding emerged in our study is that a risk prediction rule based on simple risk factors (age, multimorbidity, steroid-sparing drugs, prednisone use, and disease severity) jointly considered with two biomarkers routinely measured in clinical practice (anti-BP230 and anti-BP180 autoantibodies) provided about 80% accuracy for predicting mortality in large series of patients with this disease.
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Penfigoide Bolhoso , Humanos , Penfigoide Bolhoso/diagnóstico , Colágenos não Fibrilares , Estudos Retrospectivos , Autoantígenos , Prognóstico , AutoanticorposRESUMO
BACKGROUND: The anti-tumour necrosis factor (TNF)-α adalimumab is the only licenced biologic for moderate-to-severe hidradenitis suppurativa (HS). No predictors of response have been identified so far. OBJECTIVES: To identify clinical parameters predicting response to adalimumab and confirm its efficacy/safety. METHODS: The data of 389 patients with HS treated with adalimumab in 21 Italian centres were reviewed. Sex, age at onset/diagnosis/baseline, body mass index, smoking, phenotype, previous treatments, concomitant antibiotics and 'therapeutic delay', defined as the time from HS onset to adalimumab initiation, were assessed. Response to adalimumab and its impact on quality of life (QoL) were evaluated using the Hidradenitis Suppurativa Clinical Response (HiSCR) and the Dermatology Life Quality Index (DLQI) or the Visual Analogue Scale for pain (VAS pain), respectively. Logistic regression analysis was performed. RESULTS: The therapeutic delay correlated to lack of response to adalimumab at week 16 [odds ratio (OR) 1·92 for therapeutic delay > 10 years; 95% confidence interval (CI) 1·28-2·89; P = 0·0016). HiSCR was achieved in 43·7% and 53·9% patients at week 16 and 52, respectively. Significant reductions in both DLQI and VAS pain were found between week 16 vs. baseline (P < 0·0001 for both) and week 52 vs. baseline (P < 0·0001 for both). Previous immunosuppressants inversely correlated to HiSCR at week 52 (OR = 1·74, 95% CI 1·04-2·91, P = 0·0342). CONCLUSIONS: Inverse correlation between therapeutic delay and clinical response was found, supporting early adalimumab use and providing evidence for a 'window of opportunity' in HS treatment. Adalimumab efficacy and safety were confirmed, along with patients' QoL improvement. Immunosuppressants could negatively influence the response to adalimumab inducing a switch to non-TNF-α-driven pathways.
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Hidradenite Supurativa , Adalimumab/uso terapêutico , Anti-Inflamatórios , Hidradenite Supurativa/tratamento farmacológico , Humanos , Qualidade de Vida , Estudos Retrospectivos , Índice de Gravidade de Doença , Resultado do TratamentoRESUMO
PURPOSE: The serum metabolic changes occurring during the transition from hypothyroidism to euthyroidism are not known. This study aimed to determine the metabolomic profile in hypothyroid patients before (HypoT0) and after (HypoT1) euthyroidism achieved through levothyroxine (L-T4) treatment. METHODS: Eighteen patients with overt primary hypothyroidism were recruited for the study. All patients were treated with L-T4 to achieve euthyroidism. Thyrotropin (TSH), free thyroxine (FT4), free triiodothyronine (FT3) and metabolomics profiles were measured before and after 3 months of treatment. The euthyroid control group consisted of 28 healthy volunteers. Metabolomics analysis was performed using Nuclear Magnetic Resonance (NMR) spectroscopy. RESULTS: 1H NMR-based metabolomics profiling of patients with newly diagnosed hypothyroidism (HypoT0) showed significantly higher levels of citrate, creatinine, glycerol, myo-inositol and serine, and lower levels of proline and taurine compared to controls. Interestingly, some metabolic changes were persistent three months after pharmacological treatments, despite normal serum TSH and thyroid hormone concentrations (HypoT1). When an Orthogonal Partial Least Squares Discriminant Analysis (OPLS-DA) model was built to evaluate possible differences in the metabolic profile between HypoT0 and HypoT1, the data obtained were not significantly different. CONCLUSION: These results suggest that metabolic changes in the patients with hypothyroidism may persist after normalization of serum levels of FT3, FT4, and TSH, which currently represent the gold standard in laboratory testing for diagnosis and evaluation of thyroid pathology. So, the metabolomics approach may contribute to integrate classical hormone assays and to determine the euthyroid status achievement with greater efficacy.
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Terapia de Reposição Hormonal/métodos , Hipotireoidismo , Metabolômica/métodos , Glândula Tireoide , Tireotropina/sangue , Tiroxina , Biomarcadores/sangue , Monitoramento de Medicamentos/métodos , Feminino , Humanos , Hipotireoidismo/sangue , Hipotireoidismo/tratamento farmacológico , Hipotireoidismo/metabolismo , Estudos Longitudinais , Masculino , Metaboloma , Pessoa de Meia-Idade , Testes de Função Tireóidea/métodos , Glândula Tireoide/metabolismo , Glândula Tireoide/fisiopatologia , Tiroxina/administração & dosagem , Tiroxina/sangue , Tri-Iodotironina/sangueAssuntos
Anti-Inflamatórios não Esteroides/uso terapêutico , Betacoronavirus/isolamento & purificação , Infecções por Coronavirus/complicações , Cuidados Críticos/organização & administração , Dermatite Esfoliativa/tratamento farmacológico , Pneumonia Viral/complicações , Psoríase/tratamento farmacológico , Talidomida/análogos & derivados , COVID-19 , Infecções por Coronavirus/virologia , Dermatite Esfoliativa/complicações , Humanos , Masculino , Pessoa de Meia-Idade , Pandemias , Pneumonia Viral/virologia , Psoríase/complicações , SARS-CoV-2 , Talidomida/uso terapêuticoAssuntos
Betacoronavirus , Infecções por Coronavirus/epidemiologia , Dermatologia/organização & administração , Controle de Infecções/organização & administração , Pneumonia Viral/epidemiologia , Psoríase/terapia , COVID-19 , Infecções por Coronavirus/prevenção & controle , Humanos , Itália , Pandemias/prevenção & controle , Pneumonia Viral/prevenção & controle , SARS-CoV-2RESUMO
Steatocystoma multiplex (SM) is a rare autosomal dominant disease characterized by multiple asymptomatic intradermal true sebaceous cysts of variable size. These lesions may occasionally rupture, become inflamed, painful and heal with scarring [SM suppurativa (SMS)], thus sharing overlapping features with hidradenitis suppurativa (HS). The question of whether SMS simply mimics HS or if the two diseases may be associated remains open. We report three patients (2 M/1 F; age: 47, 27 and 40 years) affected by SMS showing inflammatory lesions on the areas typically affected by HS. Repeated skin biopsy from each patient showed true sebaceous cysts in some specimens and findings compatible with HS, without sebaceous glands evidence in others, supporting coexistence of the two diseases. Adalimumab at the initial dose of 160 mg (day 1), followed by 80 mg (day 15), and maintained at the dose of 40 mg every week from day 29 on, improved both HS and SMS lesions, including also non-inflammatory cystic lesions.
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Adalimumab/uso terapêutico , Anti-Inflamatórios/uso terapêutico , Hidradenite Supurativa/tratamento farmacológico , Esteatocistoma Múltiplo/tratamento farmacológico , Adulto , Feminino , Hidradenite Supurativa/complicações , Hidradenite Supurativa/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Esteatocistoma Múltiplo/complicações , Esteatocistoma Múltiplo/patologiaAssuntos
Carcinoma de Células Escamosas/patologia , Carcinoma de Células Escamosas/cirurgia , Progressão da Doença , Líquen Plano Bucal/patologia , Neoplasias Labiais/patologia , Neoplasias Labiais/cirurgia , Carcinoma de Células Escamosas/etiologia , Transformação Celular Neoplásica , Cicatriz/cirurgia , Seguimentos , Humanos , Hidroxicloroquina/uso terapêutico , Líquen Plano Bucal/complicações , Líquen Plano Bucal/tratamento farmacológico , Neoplasias Labiais/etiologia , Masculino , Pessoa de Meia-Idade , Invasividade Neoplásica , Procedimentos de Cirurgia Plástica , Resultado do TratamentoRESUMO
BACKGROUND: The clinical and dermoscopic changes of melanocytic nevi under the effects of photo-epilation are poorly known. OBJECTIVE: Prospective study on clinical and dermatoscopic effects of photoe-pilation on melanocytic nevi. METHODS: Eighteen patients exposed to photo-epilation gave consent to a follow-up programme from 2008 to 2017 at the Videodermatoscopic Office of the Dermatology Clinic of Cagliari (Italy), with clinical and dermoscopic assessment. RESULTS: A mean 2-year follow-up was recorded for 73 lesions. The most frequent clinical change was nevi colour, clearer than basal pictures, sometimes with increased pigmentation at the periphery, or residual islands of pigmentation, with vanishing borders. Acute burn with crusting was noted in few cases. Only one case showed progressive growth. Corresponding dermoscopic findings were bleaching of nevi, followed by pigment network disruption, appearance of white structureless areas and grey-blue globules. Complete regression occurred in 32.8% of the lesions. The growing nevus was characterized by peripheral globules. Excision for histopathological examination showed a compound nevus with mild atypia. CONCLUSIONS: Although malignant modifications of nevi after photo-epilation have not been described, the clinical and dermoscopic changes we observed suggest to extend the follow for more than 24 months, as the potential long-term adverse effects of photo-epilation on melanocytic nevi are unknown.
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Dermoscopia/métodos , Remoção de Cabelo/métodos , Terapia a Laser/métodos , Nevo Pigmentado/diagnóstico por imagem , Adulto , Feminino , Humanos , Masculino , Nevo Pigmentado/patologia , Estudos ProspectivosRESUMO
BACKGROUND: Hidradenitis Suppurativa (HS) is a chronic, highly debilitating disease. Few questionnaires have been developed to identify potential HS cases based on simple questions. A visual representation may help in characterising these lesions. OBJECTIVE: To develop and validate a visually assisted questionnaire for HS detection. METHODS: This was an observational diagnostic study on a series of patients with a first diagnosis of HS and a corresponding series of age and gender-matched controls consecutively observed in two Italian centres. The questionnaire was developed based on a critical appraisal of the relevant literature and on expert consensus. Measures of accuracy and reproducibility were assessed. RESULTS: 57 patients with HS and 57 controls were included in the study (mean age 32.9 ± 12.3 years). Based on at least one affirmative answer to the proposed questionnaire items, the accuracy was 95.6%, with a sensitivity of 98.2% and a specificity of 93.0%. Reproducibility was almost perfect on all the tested items (Cohen's kappa ≥ 0.85). LIMITATIONS: The questionnaire was tested only in experimental conditions. CONCLUSION: The questionnaire could be a useful tool for HS screening in the general population. Further studies are needed to confirm its performance in a real-world setting.
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Hidradenite Supurativa/diagnóstico , Qualidade de Vida , Autoavaliação (Psicologia) , Inquéritos e Questionários , Adulto , Fatores Etários , Estudos de Casos e Controles , Intervalos de Confiança , Feminino , Hidradenite Supurativa/fisiopatologia , Hidradenite Supurativa/psicologia , Humanos , Itália , Masculino , Pessoa de Meia-Idade , Valores de Referência , Medição de Risco , Sensibilidade e Especificidade , Índice de Gravidade de Doença , Fatores Sexuais , Perfil de Impacto da Doença , Estatísticas não Paramétricas , Adulto JovemAssuntos
Vesícula/complicações , Epidermólise Bolhosa/complicações , Doenças Periodontais/complicações , Transtornos de Fotossensibilidade/complicações , Dermatoses do Couro Cabeludo/complicações , Idoso , Antibacterianos/administração & dosagem , Antibacterianos/uso terapêutico , Clobetasol/administração & dosagem , Clobetasol/uso terapêutico , Quimioterapia Combinada , Ácido Fusídico/administração & dosagem , Ácido Fusídico/uso terapêutico , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Humanos , Masculino , Staphylococcus aureus Resistente à Meticilina/efeitos dos fármacos , Staphylococcus aureus Resistente à Meticilina/isolamento & purificação , Testes de Sensibilidade Microbiana , Rifampina/administração & dosagem , Rifampina/uso terapêutico , Dermatoses do Couro Cabeludo/tratamento farmacológico , Dermatoses do Couro Cabeludo/microbiologia , Resultado do TratamentoRESUMO
Bullous morphoea is a rare variant of localized scleroderma whose pathogenesis has been widely discussed. We retrospectively reviewed the records of all histopathologically confirmed cases of morphoea followed from 2005 to 2015 at the Dermatology Clinic and Pathology Institute of the University of Cagliari, Sardinia, Italy. Among 137 patients with morphoea, 2 cases of the bullous variant were identified, which were successfully treated with methotrexate. Thus, the bullous form comprised 1.4% of all cases of morphoea, which is much lower than the 7.5% previously reported. In one of the cases, histopathological examination revealed a peculiar 'stretching' pattern of basal keratinocytes attached to the epidermal roof of the bulla, together with increased lymphatic vessels, which were either collapsed or dilated, stressing the role of lymphatics and possibly of excessive skin trauma and friction in the development of bullous lesions.