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.

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.

Analysis of current data on the use of topical rapamycin in the treatment of facial angiofibromas in tuberous sclerosis complex.

BACKGROUND: Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous syndrome causing hamartomatous growths in multiple organs. Facial angiofibromas occur in up to 80% of patients and can be highly disfiguring. Treatment for these lesions has historically been challenging. Recently, topical rapamycin has been proposed as an effective option to treat angiofibromas but a commercially available compound has not yet been developed. OBJECTIVES: The aim of this review is to analyse the current data on the use of topical rapamycin in the treatment of angiofibromas in TSC, focusing on the risk-benefit profile. METHODS: A retrospective review of the English-language literature was conducted. RESULTS: Sixteen reports describing the use of topical rapamycin in the treatment of angiofibromas in TSC were considered, involving a total of 84 patients. An improvement of the lesions has been shown in 94% of subjects, particularly if the treatment was started at early stages. Several different formulations (ointment, gel, solution and cream) with a wide range of concentrations (0.003%-1%) were proposed. Only 4 local adverse side-effects were reported after the use of rapamycin solution. CONCLUSION: Topical rapamycin can be considered a safe option for the treatment and the prevention of facial angiofibromas in younger patients, but the best formulation has not been established. Our review demonstrates that ointment and gel should be preferred, but it is not clear which concentration is optimal. Long-term and comparative studies between topical rapamycin and ablative techniques are required to establish which treatment has a better outcome and lower recurrence rate.

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.

Intima-media thickness in an Italian psoriatic population: correlation with lipidic serum levels, PASI and BMI.

BACKGROUND: Recent findings have shown that psoriasis is frequently associated with atherosclerosis. Intima-media thickness (IMT) is a surrogate marker of atherosclerosis damage. Psoriasis patients had impaired endothelial function and thicker IMT of the carotid artery, compared with the healthy control subjects. OBJECTIVES: The aim of our study is to evaluate the prevalence of subclinical atherosclerosis in patients affected by cutaneous psoriasis without arthritis, considering the IMT as a subclinical feature of atherosclerosis. METHODS: Intima-media thickness of the common carotid artery was measured using High-resolution B-mode ultrasound in 40 psoriasis patients and 40 control patients matched for age and gender. We also measured triglycerides, PASI, total cholesterol, LDL cholesterol and BMI. Pearson's partial correlation test was used to determine the correlation between each variable. RESULTS: Intima-media thickness in subjects with psoriasis was statistically higher than those in the control subjects (1.465 mm, P value < 0.001). A positive strong correlation between IMT and age (r = 0.464 P < 0.01), total cholesterol (r = 0.466 P < 0.01), LDL cholesterol (r = 0.518 P < 0.01), triglycerides (r = 0.285 P = 0.01) and PASI (r = 0.515 P < 0.01) was observed. There was no statistically significant correlation between IMT and BMI (r = 0.132 P = 0.244). CONCLUSIONS: Our study showed a significant increase in IMT in cutaneous psoriatic patients with moderate to severe psoriasis compared with a control group. IMT seems to be uncorrelated with BMI value. It can be assumed that the assessment of IMT, through a reliable and non-invasive technique such as ultrasound, appears particularly useful in the clinical evaluation of patients with psoriasis who may benefit from early intervention.

Efficacy and Safety of bimekizumab in elderly patients: real-world multicenter retrospective study - IL PSO (Italian Landscape Psoriasis).

Purpose of the article: The aim of this multicenter observational study is to report data from real world on the use of bimekizumab in patients aged ≥ 65 years with moderate-to-severe plaque psoriasis. Elderly patients are poorly represented in clinical trials on bimekizumab for plaque psoriasis, and real-world studies are important to guide clinical choices.Materials and methods: A retrospective multicenter study was conducted in 33 dermatological outpatient clinics in Italy. Patients aged ≥ 65 years, with moderate-to-severe plaque psoriasis and treated with bimekizumab were enrolled. No exclusion criteria were applied. Bimekizumab was administered following the Italian Guidelines for the management of plaque psoriasis and according to the summary of product characteristics, in adult patients who were candidates for systemic treatments. Overall, 98 subjects were included, and received bimekizumab up to week 36. Clinical and demographic data were collected before the initiation of treatment with bimekizumab. At baseline and each dermatological examination (4, 16, and 36 weeks), clinical outcomes were measured by the following parameters: (1) PASI score; (2) site-specific (scalp, palmoplantar, genital, nail) Psoriasis Global Assessment (PGA). At each visit, the occurrence of any adverse events (AEs) was recorded, including serious AEs and AEs leading to bimekizumab discontinuation.Results: The mean PASI score was 16.6 ± 9.4 at baseline and significantly decreased to 4.3 ± 5.2 after 4 weeks (p < 0.001), and 1.1 ± 1.7 after 16 week (p < 0.001). This level of improvement was maintained after 36 weeks (p < 0.001). PASI ≤2 was recorded in 36 (36.7%) at week 4, 68% and 69.4% at week 16 and 36, respectively. By week 16, 86/98 (87.8%) patients reached PASI75, 71/98 (72.4%) obtained PASI90, and 52/98 (53.1%) PASI100. Binary logistic regression tests showed a significant association of PASI100 by week 4 with lower PASI at baseline. PASI 100 at 16 or 36 weeks was not associated with baseline PASI, obesity, age, gender, previously naïve state, and presence of psoriatic arthritis. Patients naïve to biologics at baseline had similar response to bimekizumab as non-naïve subjects.Conclusions: Bimekizumab is a suitable option for elder patients as it is effective, tolerated and has a convenient schedule.

Interdigital psoriasis of the feet (psoriasis alba): not a distinct form of psoriasis.

BACKGROUND: Interdigital psoriasis (IP) of the feet is often missed and is commonly mistaken for interdigital fungal infection. OBJECTIVE: To assess the characteristics and the clinical presentation of IP, in order to better understand if IP should be considered a distinct form of psoriasis or not. METHODS: We performed a 1-year observational study on 164 psoriatic patients, affected by moderate to severe cutaneous psoriasis and undergoing systemic therapy, examining each patient between the digits of both feet. In every suspected case of IP, differential diagnosis with interdigital fungal infection was excluded by direct microscopic examination of skin scrapings, by culture and by skin biopsy. RESULTS: We suspected IP in 7 of the 164 patients. IP was confirmed in 6 patients and in the other one a diagnosis of tinea pedis was made. CONCLUSION: IP proved to be not rare or atypical since IP localized between the toes usually presents as characteristic whitish and sodden plaques or patches. Such a diagnosis should be considered in all patients presenting characteristic lesions especially if these have a negative fungal culture, are resistant to antimycotic treatment and involve patients with a history of psoriasis.

Nail digital dermoscopy (onychoscopy) in the diagnosis of onychomycosis.

BACKGROUND: Distal subungual onychomycosis and traumatic onycholysis are the most common causes of toenail abnormalities, and differential diagnosis is often impossible without mycology. OBJECTIVES: To identify and describe dermoscopic signs specific for distal subungual onychomycosis that could facilitate its diagnosis and differentiation from traumatic mycologically negative onycholysis and to determine the sensitivity and specificity of these dermoscopic features. METHODS: We performed a retrospective study at the Outpatient Consultation for Nail Diseases of the Department of Dermatology of the University of Bologna. Dermoscopic digital images of 57 consecutive patients who underwent global photography, videodermoscopy and mycological examination for onycholysis of a single toenail between 1 December, 2010 and 30 June, 2011, were evaluated and compared. Digital dermoscopic images of onycholysis of the great toenail were evaluated for the presence of peculiar dermoscopic features. The presumptive dermoscopic diagnosis was compared with results of mycology. RESULTS: Evaluation of videodermoscopic images allowed us to identify three recurring peculiar dermoscopic features, two of which were present only in distal subungual onychomycosis (jagged proximal edge with spikes of the onycholytic area and longitudinal striae) and one only in traumatic onycholysis (linear edge - without spikes - of the onycholytic area). CONCLUSIONS: We found distinctive dermoscopic signs that are exclusive to distal subungual onychomycosis and to traumatic onycholysis. Detection of these signs is simple and can, in selected cases, help to avoid mycology.