Cutaneous mastocytosis: A dermatological perspective.

Mastocytosis is a rare disease characterised by expansion and collection of clonal mast cells in various organs including the skin, bone marrow, spleen, lymph nodes and gastrointestinal tract. The prevalence of mastocytosis has been estimated to be one in 10 000, while the estimated incidence is one per 100 000 people per year. Cutaneous mastocytosis is classified into (i) maculopapular cutaneous mastocytosis, also known as urticaria pigmentosa; (ii) diffuse cutaneous mastocytosis; and (iii) mastocytoma of the skin. In adults, cutaneous lesions are usually associated with indolent systemic mastocytosis and have a chronic evolution. Paediatric patients, on the contrary, have often cutaneous manifestations without systemic involvement and usually experience a spontaneous regression. Diagnosis of cutaneous mastocytosis may be challenging due to the rarity of the disease and the overlap of cutaneous manifestations. This short review describes pathogenesis and clinical aspects of cutaneous mastocytosis with a focus on diagnosis and currently available therapies.

Efficacy of Vismodegib in pigmented basal cell carcinoma: Appearances are deceiving.

Basal cell carcinoma (BCC) is the most common skin cancer in humans. Pigmented basal cell carcinoma (pBCC) is a rare variant of BCC. Vismodegib, was the first drug to be approved for the treatment of locally advanced (laBCCs) or metastatic basal cell carcinoma. The aim of this study was to evaluate the efficacy of Vismodegib in patients with pBCCs. We retrospectively analyzed patients receiving Vismodegib as treatment for laBCCs presenting also various pBCCs. After 6 months of treatment, we performed excisional biopsies of pBCCs, that apparently at clinical and dermoscopic assessment did not respond to therapy. A total of nine patients were assessed. After 6 months of treatment, locally advanced target BCCs showed complete remission in four out of nine patients (44.4%), four patients (44.4%) were considered in partial remission and one patient (11%) showed no response to treatment. On the contrary, all the pBCCs showed both clinically and dermoscopically resistance to treatment. Therefore, clinically persistent pBCCs were surgically removed in three patients. Histology showed a complete elimination of the neoplastic cells together with features of previous regression. Our findings indicate that the efficacy of Vismodegib is higher than that documented by clinical or even dermatoscopic observation alone.

Baseline neutrophil/lymphocyte ratio (NLR) and red blood cell distribution width (RDW) correlate with advanced stages in cutaneous squamous cell carcinoma.

BACKGROUND: The neutrophil/lymphocyte ratio (NLR) and red blood cell distribution width (RDW) at diagnosis have been shown to correlate with advanced disease and to be prognostic factors in many tumors. However, their role as a prognostic factor for cutaneous squamous cell carcinoma (cSCC) has not yet been studied. OBJECTIVE: Therefore, the aim of our study was to evaluate the correlation of NLR and RDW with stages of disease in patients with cSCC in order to define whether or not higher values of these two markers correlate with a more aggressive disease. METHODS: We retrospectively analyzed the NLR and RDW in a total of 51 newly diagnosed cSCC patients. NLR and RDW were calculated using data obtained from the complete blood count (CBC). RESULTS: Median NLR among patients with the non-advanced disease (in situ and stage I) was 2.2, whereas median NLR for patients with advanced disease was 4.87. Median RDW among patients with early stage disease was 13.7%, while median RDW in patients with advanced disease was 15.81%. Statistical analysis showed positive associations of advanced cSCC stages with NLR or RDW higher than 3.07 or 14.5%, respectively. CONCLUSIONS: Therefore, our analysis demonstrated how both NLR and RDW represent cheap and easily available factors that could be used as markers for advanced cSCC. They could help to identify patients with advanced stages disease that requires a strict follow-up.

Quality of Life and Psychological Impact in Patients with Atopic Dermatitis.

Atopic dermatitis (AD) is a dermatological disorder that affects patients' mental health and psychological state in complex ways. The importance of understanding the entire scope of this burden is well recognized, but there is limited comprehensive information about the resulting stress on adult patients with AD. This study aimed to determine the degree of psychological stress in patients with AD compared to healthy participants. A total of 352 adult patients participated in this cross-sectional study-174 with AD and 178 healthy participants. Demographic and clinical data were collected. Itch and sleep disturbance were assessed using a numeric rating scale and a visual analogue scale. The 20-item Toronto Alexithymia Scale (TAS-20) and Beck Depression Inventory (BDI) questionnaires were administered to assess the symptoms of alexithymia and depression. Quality of life (QOL) was assessed in AD patients using the Dermatology Quality Index. In our study, we found high TAS-20 and BDI scores among patients with AD. The prevalence of alexithymic personality features was 56.3% in patients with AD versus 21.3% in healthy controls (p < 0.001). Based on BDI scoring (BDI-21 > 13), depression was suspected in a significantly higher number of patients with AD than in the control group (56.9% (99/174) vs. 15.7% (28/178); p < 0.0001). Eczema Area and Severity Index (EASI) score did not show any significant correlations with psychological parameters. Among clinical parameters, only sleep disturbance was positively correlated with depression (R = 0.307, p < 0.005). Our data show that the severity index score as a representative factor of skin involvement has a limited role in predicting the effect of skin diseases on mental status. Screening and assessment for psychiatric disorders, QOL, and sleep disturbance in patients with atopic dermatitis cannot be neglected by physicians and they should be treated in clinical practice with the consideration of psychosomatic approaches.

Impact of secukinumab on patient-reported outcomes in moderate to severe plaque psoriasis: a review of clinical studies.

Introduction: Perception of illness varies among individuals and psoriasis of the same severity can be perceived in different ways by patients, making it essential to evaluate quality of life (QoL) since it can provide information on the impact of the disease on the patient's overall well-being. The use of patient-reported outcomes in clinical trials provides the ability to integrate objective clinical assessment with the patient's perception of their own state of health. Areas covered: The introduction of anti-IL17 agents in clinical practice has given patients the possibility to achieve a PASI90 response (almost clear skin) or even higher (complete clear skin) in the majority of patients. There is accumulating evidence in support of PASI90 response as the new standard goal for therapy based on its greater correlation with health-related QoL. The present review summarizes current knowledge of the effects of secukinumab on the QoL of patients with psoriasis using patient-reported outcome measures. Expert Opinion: Secukinumab, the first approved drug of this new class, has fully reached a new therapeutic paradigm not only in terms of clinical efficacy, but also in terms of patient satisfaction and self-rated health.

The Effects of Association of Topical Polydatin Improves the Preemptive Systemic Treatment on EGFR Inhibitors Cutaneous Adverse Reactions.

Epidermal Growth Factor Receptor inhibitors (EGFRi) are approved as therapeutic options in several solid tumors. Cutaneous papulopustular eruption is the most frequent cutaneous adverse-event (AE), usually treated with emollient or corticosteroids according to toxicity grade. Our study evaluated the efficacy and safety of a topical product containing polydatin, a glycosylated polyphenol, natural precursor of resveratrol showing anti-inflammatory and anti-oxidative activities, for the prevention and treatment of skin papulopustular rash in EGFRi-treated patients. Forty oncologic patients treated with EGFRi were enrolled in two groups: group-A, 20 patients with papulopustular AE, and group-B, 20 patients without cutaneous manifestations. The study consisted of twice-daily application of polydatin cream 1.5% (group-A) and 0.8% (group-B) for 6 months. In group-A patients, we observed at week 4 a remarkable improvement of skin manifestation and quality of life evaluated with National-Cancer-Institute-Common-Terminology-Criteria for Adverse-Events (NCI-CTCAE), Dermatology-Life-Quality-Index (DLQI) score and Visual-Analogue-Scale (VAS) pruritus, with a statistical significance of p < 0.05. None of the patients of group-B developed skin AEs to EGFRi. No cutaneous AEs related to the polydatin product were reported in both groups. Polydatin can be a good topical aid for the prevention and management of papulopustular rash in cancer patients receiving EGFRi, also capable of improving cancer patients' quality of life.

In which patients the best efficacy of secukinumab? Update of a real-life analysis after 136 weeks of treatment with secukinumab in moderate-to-severe plaque psoriasis.

Background: There is limited long-term, real-world evidence on the efficacy and safety in patients with plaque psoriasis treated with secukinumab. We present results at 136 weeks in a real-world setting with focus on special populations.Research design and methods: Retrospective analysis of 151 patients with chronic plaque psoriasis who initiated treatment with secukinumab between September 2015 and May 2019. Secukinumab 300 mg was administered once weekly for 5 weeks followed by once monthly.Main outcome measures: Clinical and laboratory assessments were performed up to 136 weeks.Results: At 16 weeks, 90%, 79%, and 63% of patients achieved Psoriasis Area and Severity Index (PASI) 75, PASI 90, and PASI 100, respectively, compared with 79%, 72%, and 55% of patients after 136 weeks of therapy with secukinumab. Fifteen of the 151 patients experienced an adverse event, the most common of which was candida infection (4%). Biological treatment naïve was significantly associated with response to therapy at 1 and 2 years (P < 0.0001). There were no safety issues in patients with infection with HBV, HCV or mycobacterium tuberculosis.Conclusions: Our results confirm the rapidity of action of secukinumab as well as its long-lasting efficacy and good safety in real-world clinical practice.

Use of Guselkumab for the Treatment of Moderate-to-Severe Plaque Psoriasis: A 1 Year Real-Life Study.

Little information is available from real-life studies evaluating the efficacy of guselkumab in moderate-to-severe psoriasis. In this real-life study, we retrospectively examined a database of 52 patients with moderate-to-severe psoriasis treated with guselkumab (100 mg, s.c.) and followed for 1 year. Disease severity and treatment response was assessed by the Psoriasis Area and Severity Index (PASI) at baseline and after 4, 12, 20, 28, 36, 44, and 52 weeks. Predictors of a PASI response were evaluated by univariate and multivariate regression. After 12 months, 84.2% of patients (mean age 51.3 ± 14.1 years) treated with guselkumab achieved a PASI score of <3. Furthermore, PASI score decreased from 20 ± 13.3 at baseline to 4.4 ± 4.7 and 2.7 ± 3.9 at 12 and 20 weeks, and PASI 75, 90, and 100 response was achieved in 84.2%, 78.9%, and 63.2% of patients respectively at 12 months. Stepwise multivariate regression analysis revealed that previous biological treatment and the presence of comorbidities were associated with poorer response between 28-44 weeks, however the presence of obesity per se was not associated with poorer response. Difficult-to-treat areas were also improved as early as 12 weeks following guselkumab. Guselkumab was observed to be effective and safe in patients with moderate-severe chronic psoriasis in a real world-setting.