Italian National Registry of Alopecia Areata: an epidemiological study of 699 Italian patients.

BACKGROUND: Alopecia areata (AA) is an organ-specific autoimmune disease that affects the hair follicles of the scalp and the rest of the body causing hair loss. Due to the unpredictable course of AA and the different degrees of severity of hair loss, only a few well-designed clinical studies with a low number of patients are available. Also, there is no specific cure, but topical and systemic anti-inflammatory and immune system suppressant drugs are used for treatment. The need to create a global registry of AA, comparable and reproducible in all countries, has recently emerged. An Italian multicentric electronic registry is proposed as a model to facilitate and guide the recording of epidemiological and clinical data and to monitor the introduction of new therapies in patients with AA. METHODS: The aim of this study was to evaluate the epidemiological data of patients with AA by collecting detailed information on the course of the disease, associated diseases, concomitant and previous events, and the clinical response to traditional treatments. Estimate the impact on the quality of life of patients. RESULTS: The creation of the National Register of AA has proven to be a valid tool for recording, with a standardized approach, epidemiological data, the trend of AA, response to therapies and quality of life. CONCLUSIONS: AA is confirmed as a difficult hair disease to manage due to its unpredictable course and, in most cases, its chronic-relapsing course, capable of having a significant impact on the quality of life of patients.

Efficacy and tolerability of tirbanibulin 1% ointment in the treatment of cancerization field: a real-life Italian multicenter observational study of 250 patients.

BACKGROUND: Tirbanibulin 1% ointment is approved for the field treatment of Olsen grade I actinic keratoses (AKs) of the face and scalp. METHODS: We performed a multicenter retrospective study involving 15 dermatologic units in Italy to investigate the efficacy and tolerability of tirbanibulin in a real-life setting. 250 patients were enrolled. Tirbanibulin, 1% ointment, was applied daily for five consecutive days. The efficacy of treatment was measured with modifications of the Actinic Keratosis Area and Severity Index (AKASI). A satisfactory response was defined by complete (100% reduction in the number of lesions) or partial clearance (75-99%) of treated AKs. RESULTS: Overall, the AKASI score was significantly reduced in the studied population (mean, from 4.1 ± 2.7 to 1.4 ± 1.5; P < 0.001). A satisfactory response was observed in 222 (88.8%) cases. The proportion of satisfactory responses was higher when follow-up was performed after 8 weeks (34/35, 97.1%). The reduction in AKASI was significant in patients with Olsen grade II or III lesions (from 5.3 ± 2.8 to 1.6 ± 1.6; P < 0.001). A satisfactory response was observed in 91/104 (87.5%) cases. AKASI reduction was also significant in patients with trunk or limb AKs (from 7.0 ± 1.3 to 2.0 ± 1.6; P = 0.018) since a satisfactory response was observed in 7/8 (87.5%) cases. Tirbanibulin was well tolerated; all adverse events (AEs) included transient local reactions at the site of treatment. Overall, 231 patients had at least one AE. Only 7 (2.8%) grade 4 AEs were recorded. CONCLUSION: Our retrospective study confirmed that tirbanibulin 1% ointment is effective and well tolerated in a real-life setting and is also promising for Olsen grade II and grade III AKs and AKs localized on difficult-to-treat areas.

Psoriasis and biological drugs at the time of SARS-CoV-2 infection: a mini review outlining risk of infection, seroprevalence, and safety and efficacy of the BNT162b2 vaccine.

Objective: The aim of this study is to review the life of patients with psoriasis on biologic therapy during the SARS-CoV-2 pandemic and the relevance of frailty within this context, reviewing studies that describe the course and severity of infection in patients with psoriasis on biologics, the seroprevalence of SARS-CoV-2, and the safety and efficacy of the BNT162b2 vaccine in these patients. Materials and methods: The keywords "Psoriasis," "Biologics," "SARS-CoV-2," "COVID-19," and "BNT162b2 Vaccine" were used in various combinations on database engines to find relevant articles on this topic. Results: A total of 36 articles were found, with 20 concerning the course, severity, and seroprevalence of SARS-CoV-2 in patients with psoriasis on biologic therapy and 16 concerning safety and efficacy of BNT162b2 in these patients. Discussion: Patients with psoriasis on biologic therapy did not have increased seroprevalence compared with the general population, indicating that they were not at an increased risk of SARS-CoV-2 infection compared with the general population. Furthermore, the immunosuppressive action of biologics may be protective, as patients on biologic therapy had better outcomes and less risk of severe infection. The seroconversion rate against SARS-CoV-2 from the BNT162b2 vaccine was similar in both patients with psoriasis on biologics and the general population, indicating that efficacy is not hindered by the biologic therapy. However, the cellular response in population with psoriasis was significantly less intense, and the humoral immune response was weaker than that in the general population, demonstrating that the possibility of tighter vaccination schedules and additional doses may be advantageous in these patients.

Propranolol for the treatment of infantile hemangiomas: a nine-year monocentric experience from a tertiary hospital.

BACKGROUND: Propranolol is currently considered the first-line therapy for problematic infantile hemangiomas (IH), the most common benign vascular neoplasm of infancy. OBJECTIVES: We present a retrospective observational study aimed at assessing the efficacy of propranolol in 44 IH patients. MATERIALS & METHODS: A nine-year retrospective review considering clinicodemographical and therapy-related variables was performed on medical records of infants treated for IH with oral propranolol. Each lesion was assessed through a numeric severity score based on size and colour both at baseline and after treatment conclusion (p <0.05 was considered statistically significant). RESULTS: Complete remission was achieved in 90.7% cases of IH with a general mean improvement in severity of 94.94%. No severe adverse effects were reported. Preterm patients showed a superior response compared to term infants, even though the difference was not significant (p=0.185). CONCLUSION: Propranolol showed high efficacy in terms of safety profile and cosmetic results. Prematurity and precocious therapy could be linked to a superior response.

A Clinico-Pathological Multidisciplinary Team Increases the Efficacy of Skin Biopsy and Reduces Clinical Risk in Dermatology.

A clinical risk is an inherent risk in healthcare processes, including skin biopsy procedures, and may lead to misdiagnoses, increased healthcare costs and potential harm to patients. Indeed, clinical and histopathological data must be integrated if we are to reduce clinical risks and improve diagnostic accuracy in the diagnosis of dermatologic diseases. Although dermopathology services used to be part of a dermatologist's duty, the recent centralization of these laboratories has caused a loss of expertise and increased both complexity and safety issues. Some countries have implemented clinical-pathological correlation programs aimed at facilitating communication between clinicians and dermatopathologists. However, Italy has regulatory and cultural barriers that make the implementation of these programs difficult. Therefore, an internal analysis was carried out to assess the efficacy and impact that skin biopsy procedures for inflammatory and neoplastic conditions have on the quality of care in our dermatology department. As the analysis evidenced a high number of descriptive pathologic reports and discordant diagnoses, a multidisciplinary group of four dermatologists, four general pathologists and one dermatopathologist was set up. Herein, we present the results of this analysis and project and describe the structure of the multidisciplinary group. We also discuss the pros and cons, possibilities and limitations of our project, including the regulatory barriers of the Italian National Health System.

A monolateral pigmented lesion of the nipple.

Pigmented mammary Paget's disease is a very rare variant of mammary Paget's disease linked to an underlying carcinoma in almost all cases. We present the case of a 62-year-old female patient who came to our attention for the evaluation of a monolateral asymptomatic pigmented lesion of the right nipple, which turned out to be a pigmented mammary Paget's disease unassociated to an underlying malignancy - an extremely rare entity only anecdotally reported in literature. The two main peculiarities of our patient's lesion, the importance of immunohistochemistry in the differential diagnosis and the theories on its pathogenesis are discussed. Further studies are necessary to establish the best treatment options. Click here for the corresponding questions to this CME article.

Omalizumab as a corticosteroid-sparing agent in the treatment of bullous pemphigoid.

Bullous pemphigoid (BP) is the most common autoimmune blistering skin disease, characterized by the development of autoantibodies against hemidesmosomal components BP180 and BP230. The mainstay of therapy is topical and systemic corticosteroids (CS) and immunosuppressors. As this pathology mainly involves the elderly, subjects often have numerous comorbidities that influence the clinical management. Omalizumab is a recombinant humanized monoclonal anti-IgE antibody which has recently emerged as a promising treatment for BP in patients for whom CS are contraindicated or conventional treatments have failed to control the disease. For this study, we selected five patients who presented with corticosteroid-dependent BP with a contraindication to the use of other immunosuppressive treatments. The objectives of our study were to evaluate the effectiveness of omalizumab in controlling BP and allowing to decrease the dosage of systemic CS, assessing the effects of omalizumab on the clinical manifestations and the titers of circulating anti-BP180 and BP230 antibodies, IgE and eosinophils. A reduction in the dose of systemic CS was possible in 100% of the patients and complete resolution of the clinical picture was seen in 100% for skin lesions and in 40% for pruritus. A reduction of circulating IgE was found in 40%, anti-BP180 and BP230 IgGs were decreased in 60% and eosinophils in 80%.

An Ecchymosis with Fulminant Evolution.

We present the case of an 83-year-old woman who presented with an advanced cutaneous angiosarcoma of the head. The tumor had first appeared as a small ecchymosis on her forehead 3 months before admission. There was an extremely rapid and dramatic evolution, as evidenced by photographic documentation by her relatives. Unfortunately, the delay in access to the healthcare system due to the COVID-19 pandemic lockdown and the fulminant growth were the main determinants for our patient outcome.

Treatment beyond progression and locoregional approaches in selected patients with BRAF-mutated metastatic melanoma.

The clinical management of BRAF-mutated metastatic melanoma had an important turning point after the introduction of the targeted therapy. Despite the efficacy and good tolerability of this treatment, the development of resistance mechanisms causes disease progression. The aim of this review is to investigate the role of treatment beyond progression and locoregional approaches in BRAF-mutated metastatic melanoma and provide oncologists dealing with this malignancy a useful road map on when and why to choose this strategy. The article is structured in the form of a narrative review reporting the most significant studies on the subject. Most of the available articles are represented by retrospective studies and case reports, leading to limitations in the final interpretations. Nevertheless, a correct analysis of the selected studies allows the drawing of some conclusions. In well-selected cases, treatment beyond progression could play an important role in the treatment sequence of patients with BRAF-mutated advanced melanoma and would seem to produce good disease control rates and positive survival outcomes. A careful evaluation of the radiological examinations and laboratory tests, based on the clinical conditions, allows the identification of which patients can benefit from this strategy. Such patients are those who, at the time of progression, have favourable features such as a lower performance status according to Eastern Cooperative Oncology Group (ECOG-PS), normal lactate dehydrogenase levels and lower disease burden. The clinical benefit is also consolidated by the addition of locoregional approaches. Locoregional approaches can include electrochemotherapy, radiotherapy or surgery, and their use provides local disease control and a better quality of life for patients.

Trichodynia and telogen effluvium in COVID-19 patients: Results of an international expert opinion survey on diagnosis and management.

BACKGROUND: The cutaneous manifestations of COVID-19 may be useful disease markers and prognostic indicators. Recently, postinfectious telogen effluvium and trichodynia have also been reported. OBJECTIVE: To evaluate the presence of trichodynia and telogen effluvium in patients with COVID-19 and describe their characteristics in relation to the other signs and symptoms of the disease. METHODS: Patients with a history of COVID-19 presenting to the clinics of a group of hair experts because of telogen effluvium and/or scalp symptoms were questioned about their hair signs and symptoms in relation to the severity of COVID-19 and associated symptoms. RESULTS: Data from 128 patients were collected. Telogen effluvium was observed in 66.3% of the patients and trichodynia in 58.4%. Trichodynia was associated with telogen effluvium in 42.4% of the cases and anosmia and ageusia in 66.1% and 44.1% of the cases, respectively. In majority (62.5%) of the patients, the hair signs and symptoms started within the first month after COVID-19 diagnosis, and in 47.8% of the patients, these started after 12 weeks or more. LIMITATIONS: The recruitment of patients in specialized hair clinics, lack of a control group, and lack of recording of patient comorbidities. CONCLUSION: The severity of postviral telogen effluvium observed in patients with a history of COVID-19 infection may be influenced by COVID-19 severity. We identified early-onset (<4 weeks) and late-onset (>12 weeks) telogen effluvium.

Atypical erythema annulare centrifugum in a child with celiac disease.

Herpetiformis dermatitis is the best characterized extraintestinal manifestation of celiac disease (CD). However, other chronic heterogeneous skin lesions have been associated with CD and should be considered in the differential diagnosis.

Real Life Clinical Management and Survival in Advanced Cutaneous Melanoma: The Italian Clinical National Melanoma Registry Experience.

BACKGROUND: Cutaneous melanoma (CM) is one of the most aggressive types of skin cancer. Currently, innovative approaches such as target therapies and immunotherapies have been introduced in clinical practice. Data of clinical trials and real life studies that evaluate the outcomes of these therapeutic associations are necessary to establish their clinical utility. The aim of this study is to investigate the types of oncological treatments employed in the real-life clinical management of patients with advanced CM in several Italian centers, which are part of the Clinical National Melanoma Registry (CNMR). METHODS: Melanoma-specific survival and overall survival were calculated. Multivariate Cox regression models were used to estimate the hazard ratios adjusting for confounders and other prognostic factors. RESULTS: The median follow-up time was 36 months (range 1.2-185.1). 787 CM were included in the analysis with completed information about therapies. All types of immunotherapy showed a significant improved survival compared with all other therapies (p=0.001). 75% was the highest reduction of death reached by anti-PD-1 (HR=0.25), globally immunotherapy was significantly associated with improved survival, either for anti-CTLA4 monotherapy or combined with anti-PD-1 (HR=0.47 and 0.26, respectively) and BRAFI+MEKI (HR=0.62). CONCLUSIONS: The nivolumab/pembrolizumab in combination of ipilimumab and the addition of ant-MEK to the BRAFi can be considered the best therapies to improve survival in a real-world-population. The CNMR can complement clinical registries with the intent of improving cancer management and standardizing cancer treatment.

Sézary Syndrome: a clinico-pathological study of 9 cases.

BACKGROUND: Sézary Syndrome (SS) is a rare and aggressive variant of cutaneous T-cell lymphoma characterized by erythroderma, generalized lymphadenopathy and atypical lymphocytes in peripheral blood. The aim of the study is to describe our experience with SS patients. METHODS: Nine SS patients were retrospectively identified within 288 patients with cutaneous T-cell lymphomas (CTCLs) followed from 1977 to 2017 in the Unit of Dermatology, IRCCS Policlinico San Matteo Foundation, Pavia, Italy. RESULTS: Nine SS patients were described: 5 males and 4 females, mean age at diagnosis 66.1 years (49-87 y), overall survival (OS) after SS diagnosis was 2.6 years (31.5 ms). All the patients showed erythroderma, pruritus and lymphadenopathy. Palmo-plantar hyperkeratosis, nail lesions, alopecia and ectropion were also present. One patient was excluded for significative differences in management. Three lines treatment -extracorporeal photopheresis plus immunomodulator/s plus photo-photochemotherapy- was the most used first-line option for induction of remission, reached in 4 patients out of 8: 3 with Complete Remission (CR), 1 with Partial Remission (PR). Prognostic variables were investigated by univariate analysis: hypereosinophilia, highly elevated ß2µglobulin >3500 µg/L, male sex and highly elevated LDH>450 U/L resulted with statistical power. CONCLUSIONS: The improved comprehension of SS pathogenesis is progressively increasing the still poor survival: 38.5 months (3.2 years) considering only the 6 patients followed in the last five years, versus overall 31.5 months (2.6 years). The correct identification of SS patients remains determinant for the proper overall management. Among unfavorable prognostic markers, levels of ß2µglobulin allow stratification of patients.

Does therapy with biological drugs influence COVID-19 infection? Observational monocentric prevalence study on the clinical and epidemiological data of psoriatic patients treated with biological drugs or with topical drugs alone.

Since the onset of the coronavirus disease 2019 (COVID-19) pandemic, there has been an open debate on the impact of biological drugs used in the treatment of psoriasis. To define whether patients under treatment with biologics suffer from increased morbidity and mortality from COVID-19, compared to psoriatic patients treated only with topical drugs, we designed an observational monocentric prevalence study recording the personal and clinical data of psoriatic patients, with focus on the presentation of signs and symptoms related to COVID-19 in the period of time ranging from 1 January 2020 to 31 May 2020. A total of 180 patients were enrolled into two groups: 100 patients in the topical therapy group and 80 patients in the biological therapy group. No statistically significant difference was found between the groups regarding the prevalence of COVID-19 infection and symptoms at a bivariable analysis with adjustment for confounders. In conclusion, psoriatic patients under treatment with biologics do not seem to be more susceptible to COVID-19 compared to other psoriatic patients and we suggest not interrupting treatment with biological drugs, even in areas suffering from active outbreaks of the disease.