Mortality and prognostic factors in patients with bullous pemphigoid: a retrospective multicenter Italian study.

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.

Secukinumab in multi-failure psoriatic patients: the last hope?

Psoriasis is a multi-systemic chronic inflammatory disease that affects about 1.5-3% of the general population, of which almost 20% suffer from a moderate-severe form. Those patients can be treated with a systemic agent and in case of scarce response or contraindications, they may require a biologic therapy, such as tumor necrosis factor or interleukin-12/23 inhibitors. When also these agents fail, clinicians face a true therapeutic challenge. We report a case series of multi-failure 16 patients, successfully treated with secukinumab, a human monoclonal antibody that selectively neutralizes interleukin-17 A and is recently approved for the treatment of plaque psoriasis, psoriatic arthritis, and ankylosing spondylitis.

Sex and the PASI: patients affected by a mild form of psoriasis are more predisposed to have a more severe form of erectile dysfunction.

BACKGROUND: Psoriasis is a multi-systemic disease involving the skin and joints, but it is also characterized by endothelial dysfunction, which may cause sexual impotence and erectile dysfunction (ED), an embarrassing disease frequently neglected by dermatologists. OBJECTIVE: The principal objective was assessing the relationship between the severity of psoriasis and the severity of ED. We also investigated whether severity of psoriasis was related to International Index of Erectile Function-5 (IIEF-5) score, whether genital lesions worsened the IIEF-5 score, whether ED was related to factors such as diabetes, smoking and hypertension, and finally the overall the psychological factors felt by the patient. METHODS: We administered two questionnaires (one of which was the IIEF-5, a validated score to assess erectile dysfunction) to three groups of patients: 60 with mild psoriasis, 60 with severe psoriasis (assessed by Psoriasis Area Severity Index, PASI) and a control group including 60 patients without the disease. RESULTS: In the group of mild psoriasis, the patients who suffered from ED were the 56.67%, while in the group of severe psoriasis, ED affected the 46.68% of subjects. In the control group, ED was reported by the 23.33% of patients. The average IIEF-5 score was 18.81 for patients with mild psoriasis and 20.31 for patients with severe form. The difference in the average IIEF-5 scores between psoriatic (mild and severe cases) and control group was not statistically significant. Most patients with sexual dysfunction had also genital lesions; diabetes, smoking and hypertension were not related to lower IIEF-5 scores. The overall psychological profile of psoriatic patients was worse than that of the controls. CONCLUSION: We concluded that ED was related to psoriasis, in particular to mild forms. Moreover, since ED is a marker of cardiovascular events, also related to negative impact on the quality of life, physicians should always investigate the presence of ED in clinical practice.

Detection of D-penicillamine in skin lesions in a case of dermal elastosis after a previous long-term treatment for Wilson's disease.

BACKGROUND: Skin adverse events associated with D-Penicillamine (DPA) are common and multi-faceted, although the presence of DPA or its metabolites has never been documented in the skin, because of inherent difficulties in determining its tissue levels. Thus, the association between DPA and DPA-related dermatoses has been only hypothesized on the basis of careful history, clinical observation and typical histopathological findings. OBJECTIVE: To detect DPA in biopsy specimens in a unique case of 25-year-late-onset elastosis perforans serpiginosa and pseudo-pseudoxanthoma elasticum associated with a history of long-term high dose DPA, by applying a recently described analytical method to assess the presence of DPA in skin. METHODS: We used a reliable analytical method based on high-performance liquid chromatography coupled with amperometric detection to look for the presence of DPA in skin biopsy specimens. RESULTS: A chromatographic peak corresponding to DPA was evidenced in some affected skin samples collected from the patient. CONCLUSION: We documented the effective presence and the persistence after 25 years of DPA in the skin of a woman affected by elastotic cutaneous change due to a long-term therapy with DPA. This report provides further evidence of the relationship between DPA deposit in affected skin and clinical manifestation.