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.

"Your Skin Tells You" Campaign for Keratinocyte Cancers: When Individuals' Selection Makes the Difference.

BACKGROUND: Prevention campaigns for skin cancers have focused primarily on melanoma, and over time there has been increasing awareness of the need to select the population to be screened to maximize program effectiveness. OBJECTIVES: The objective of the study was to report the results of a free dermatological initiative, as part of an awareness campaign dedicated to keratinocyte cancers, targeting individuals pre-selected through a short questionnaire. METHODS: One day of dermatological consultations was held at 15 dermato-oncology referral centers during May 22-June 30, 2021. For selection, individuals answered a telephone interview consisting of 7 yes/no questions on risk factors. Demographics, clinical characteristics of suspicious tumors, and histopathologic diagnosis of excised lesions were collected. Suspicion rate, detection rate, and positive predictive values (PPVs) for any skin cancer, basal cell carcinoma (BCC), cutaneous squamous cell carcinoma (cSCC), and melanoma were calculated. RESULTS: A total of 320 individuals (56.9% males; 43.1% females) with a median age of 69.6 (range 21-91) years qualified for the screening initiative. Overall, skin cancers and precancerous lesions were diagnosed in 65.9% of the patients. Suspicion rate was 28.7% for any skin cancer (92/320), 22.8% for BCC (73/320), 4.7% for cSCC (15/320), and 1.2% for melanoma (4/320). Detection rate was 23.4% for any skin cancer (PPV 93.7%), 18.1% for BCC (PPV 95.1%), 4.4% for cSCC (PPV 93.3%), and 0.9% for melanoma (PPV 75%). CONCLUSIONS: Selection of individuals at high risk is a cost-effective approach for early detection campaigns for keratinocyte cancers.

Can Ultrasound Examinations Generate Pain in Hidradenitis Suppurativa Patients? Results from a Multicentric Cross-Sectional Study.

BACKGROUND: Pain is not a trivial issue for hidradenitis suppurativa (HS) patients and has been considered a domain in the Core Outcome Set. To date, there is no evidence about pain caused by the ultrasound examinations. OBJECTIVE: The aim of the study was to assess the presence of pain generated by the ultrasound examinations of HS patients. METHODS: A multicentric cross-sectional study for detecting pain during the ultrasound examinations of HS patients using a validated verbal questionnaire immediately after the imaging studies. Statistical analysis included demographic data and possible associations with sex, age, location, clinical (Hurley), and ultrasonographic scoring (SOS-HS). The statistical tests were two proportions Z test, χ2 test, Student's t test, and ANOVA. A p < 0.05 was considered significant. RESULTS: 317 patients met the criteria. 77.3% of them did not present pain. Of cases with pain, 59.8% were mild, 16.7% moderate, and 23.6% severe. No significant association was found with sex, age, staging, location, or the number of affected regions. Although nonsignificant, severe pain cases were more frequent in the clinical Hurley III and ultrasonographic SOS-HS III stages. CONCLUSION: Pain generated by the ultrasound examination of HS patients is infrequent.

Therapeutic strategies for oral lichen planus: State of the art and new insights.

Oral lichen planus (OLP) is a chronic inflammatory disease of the oral mucosa. Several clinical subtypes of OLP have been reported, including the reticular and erosive one. On the one hand, reticular OLP is usually asymptomatic and is characterized by white streaks surrounded by well-defined erythematous borders. On the other hand, erosive OLP shows ulcerations and erosions surrounded by erythematous mucosa. While reticular OLP is relatively easy to control, erosive OLP is extremely painful and refractory to therapies, limiting the quality of life of the patients. In addition, treating erosive OLP is extremely tricky, and a gold standard treatment has not yet been established. However, several therapeutic approaches have been reported as effective, including systemic corticosteroids, systemic retinoids, and anti-interleukin (IL)-17/anti-IL-23 drugs. Indeed, our group and other several authors reported the effectiveness of anti-IL17, anti-IL12/23, and anti-IL23 agents in refractory OLP, highlighting the urgency of clinical studies on the use of anti-IL agents in OLP patients. In this paper, we reviewed the English- and German-language literature about therapeutic strategies for treating OLP, focusing on new systemic therapies for erosive OLP.

Hidradenitis Suppurativa in a Large Cohort of Italian Patients: Evaluation of the Burden of Disease.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic, inflammatory, recurrent, debilitating skin disease of the hair follicle that usually occurs after puberty with painful, deep-seated, inflamed nodules and sinus tracts in the apocrine gland-bearing areas of the body, most commonly the axillae and inguinal and anogenital regions, with a relevant impact on patients' quality of life (QoL). OBJECTIVE: To evaluate how the burden of HS disease impacts on patient well-being and working activities in a large Italian population over a period of 9 months. METHODS: A multicenter, prospective, epidemiologic cohort study was conducted in adult Italian patients with HS. HS severity was assessed through Hurley stage and HS Physician's Global Assessment (HS-PGA), clinical improvement by HS Clinical Response (HiSCR) and partial response, and disease burden through QoL questionnaires (HIDRAdisk, Skindex-16, Dermatology Life Quality Index [DLQI]), and Work Productivity and Activity Impairment - General Health (WPAI:GH). RESULTS: A total of 308 patients (56.2% women; mean age 35.2 ± 12.9 years) were enrolled in 27 dermatologic clinics. Men were older (37.4 years vs. 33.5), more smoking addicted (74.1% vs. 60.1%), and alcohol consumer (34.1% vs. 13.9%), while more women were obese (34.10% vs. 22.22%). At baseline, most patients had a Hurley severity stage of 2 (43.9%), a moderate HS-PGA score (57.1%), and poor QoL (HIDRAdisk: 65.7 ± 23.3, Skindex-16: 60.3 ± 26.9, and DLQI: 10.8 ± 8.1). Patients with more severe disease showed worse QoL. Mean values for the variables related to HS severity decreased during the study period. The achievement of HiSCR and partial response increased during the study. CONCLUSION: This study offers insight into the disease burden of HS in an Italian population. Our results underline the impact of QoL evaluation, also with the use of the HIDRAdisk, in clinical routine as a support to validated severity clinical and instrumental indexes for a "360-degree" assessment of HS patient's burden of disease.

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.

Digital Myxoid Cysts: Correlation of Initial and Long-Term Response to Steroid Injections.

BACKGROUND: Digital mucous cysts (DMCs) are benign myxoid pseudocysts that develop on the distal interphalangeal joint's lateral or dorsal aspects. Management consists either of a surgical approach, conservative therapy, or simple follow-up. OBJECTIVE: To correlate the initial and long-term response with clinical and ultrasound parameters in DMCs treated with intralesional steroids as first-line therapy. METHODS: A single-center prospective open-label study recruited 15 patients affected by DMCs, who had been treated with a cycle of up to 3 steroid injections at a 6 to 9 week time interval. RESULTS: At the first follow-up visit, 53.3% of patients were cleared of DMCs, achieving a complete response, whereas 46.7% experienced a >30% decrease in their DMC volume, and were considered partial responders. After 1 year of follow-up, the cure rate decreased to 40%, and the recrudescence rate was 27.3%. Clinical and sonographic characteristics that positively correlated with a maintained complete response at follow-up were as follows: young age, absence of osteophytes, low volume, complete clearance at T1, and short disease duration (p < .05). CONCLUSION: Intralesional steroid therapy is an easy approach for DMC, with minimal side effects; identifying predictive hallmarks is useful to offer a straightforward surgical treatment to patients who have nonresponder characteristics.

Clinical and Power-Doppler ultrasound features related with persistence of fistulous tracts under treatment with adalimumab in hidradenitis suppurativa: 4 years of follow-up.

Fistulous tracts are the hallmark lesions of hidradenitis suppurativa (HS) and respond poorly to the currently available medical treatments. To evaluate the clinical and ultrasound features related with both healing and persistence of fistulous tracts in patients under treatment with adalimumab, a retrospective analysis of power-Doppler ultrasound (PD-US) images with fistulas in HS patients was performed. The clinical and sonographic staging of HS, body areas involved, and anatomic characteristics of the fistulous tracts were registered and graded. Chi-square test, univariate/multivariate Cox-regression analysis with clustered error, and Kaplan-Meier analysis were computed to analyze data. In total, 151 fistulous tracts from 33 HS patients were included. Age, BMI, length, thickness, subcutaneous pattern, high intensity of PD-US signal, and a high grade of fibrosis/edema were all related to a lower possibility of healing and a high risk of longer persistence at binomial Cox-regressions. Whereas, multivariate regressions showed that high fibrosis, was the variable with the highest risk of poor response and longer survival. Survival-analysis showed that fistulas with high fibrosis or PD-US signal have longer survival time than those with absent/low fibrosis or signal. Limitations include the impossibility to detect with ultrasound lesions less than 0.1 mm and smaller sample size. In conclusion, an accurate assessment of fibrosis may be crucial to define better when a surgical approach-besides the medical treatment-could be required. PD-US may assess the decrease of vascularization in HS lesions and consequently the reduction of inflammation due to immunomodulatory therapies.

Scalp hair whorl patterns in patients affected by Neurofibromatosis Type 1: A case-control study.

BACKGROUND: The hair whorl denotes the spiral disposition of hairs around an axis, which is determined by the follicle growing direction. Atypical variants of scalp hair patterns, identified by abnormally placed or multiple whorls, have been associated with early brain developmental disorders and several dysmorphic syndromes. MATERIALS AND METHODS: A 6-month case-control, prospective monocentric study included an overall number of 557 children. A logistic regression analysis was performed to evaluate the relationship between localization, the number of scalp hair whorls, and their association with neurofibromatosis type 1 (NF1). RESULTS: NF1 positively correlates with a frontal localization, whereas a negative association was found with a parietal whorl pattern (P < 0.001). CONCLUSION: Evaluation of scalp whorls gains importance in the neonatal settings and may contribute to suspect the early diagnosis of NF1, as the related National Institutes of Health diagnostic criteria cannot be usually observed at an early age.

Ultrasound features of the subungual glomus tumor and squamous cell carcinomas.

BACKGROUND: Nail tumors often pose a significant challenge for the clinician, as they are burdened by a high delay in diagnosis and paucity of clinical signs in the early stage. In most cases, the onset of pain or nail plate dystrophy leads to imaging investigations such as plain radiography, magnetic resonance imaging, and ultrasonography (US) when surgery is pending. MATERIAL AND METHODS: A 2-year monocentric study evaluated the histologically confirmed cases of subungual glomus tumor (GT) and squamous cell carcinoma (SCC) with a pre-surgical US assessment, and reviewed their sonography features. A total of 7 digital GTs and 6 SCCs of the nail were collected. RESULTS: GT and SCC are easily distinguishable on US. Compared to SCC, GT was positively associated with an ovoid shape, well-defined borders, bone cup-scalloping without cortical erosion, and a rich intralesional vascular pattern (P < 0.05). Vice versa, SCC was significantly correlated with irregular shape, ill-circumscribed margins, an infiltrative growth pattern with peripheral hypoechoic foci, posterior acoustic shadow, cortical erosion, and a highly vascular multipolar peripheral pattern (P < 0.05). CONCLUSION: The rapid assessment of GT and SCC sonographic features may contribute to reduce diagnostic delay, expedite management, and decrease the risk of surgical relapse.

Melanocytic lesion in children and adolescents: an Italian observational study.

Malignant melanoma is a rare neoplasm in the pediatric age group. One of the main risks factors is represented by the presence of a high number of melanocytic nevi. Sun exposure in pediatric age represents a predictor of melanocytic nevi number in the adult age and there is a direct correlation between the presence of melanocytic moles in early childhood and the development of many nevi in adults, suggesting that a high number of nevi in childhood should be considered as a predictor of melanoma development during adult life. The predominance of dermoscopic types of melanocytic nevi varies according to the individual's age and depends on endogenous or exogenous signaling, suggesting different pathways of nevogenesis. We evaluated the total amount of melanocytic nevi of pediatric patients and their prevalent dermoscopic pattern. We investigated the reasons for dermatological examination, pointing out the role of older parents' populations in the decision to refer to a dermatological consultant. We performed a prospective observational study on 295 pediatric outpatients consecutively enrolled from July 2018 to July 2019. Descriptive and inferential statistical analyses were performed using logistic and linear regression. 49% of children were characterized by less than 10 nevi, 45% of children by a number of nevi between 10 and 30, whilst 17 patients (5%) had a number of nevi between 30 and 50. The most prevalent dermoscopic pattern was the globular one. An older parenting age was correlated with an autonomous reason for referral and a later first visit. Our data agreed with previous suggestions demonstrating a strong influence of latitude, sun exposure and ethnic background in the development of the number of nevi. To our knowledge, this is the first study, which evaluated the reasons for dermatological examination and the role of older parents' populations in the decision to refer to a dermatological consultant.

Adalimumab Dose Intensification in Recalcitrant Hidradenitis Suppurativa/Acne Inversa.

BACKGROUND: Adalimumab is the only approved compound for the treatment of adult patients with moderate-to-severe hidradenitis suppurativa (HS) who did not respond to a systemic classical treatment. Despite its significant short- and long-term efficacy, a percentage of patients do not respond sufficiently. Moreover, some primary responders experience a response loss with time. OBJECTIVE: To analyse the effectiveness of adalimumab dose intensification in HS patients. METHODS: A case series of adalimumab 80 mg/week subcutaneously (s.c.) compassionate use in patients with HS, who did not respond sufficiently or in primary responders with progressive response loss to the registered adalimumab dose of 40 mg/week s.c. Patients were collected and evaluated retrospectively. Patients' data were extracted from medical records. RESULTS: The 14 patients collected were Caucasian with HS of Hurley stage II-III and moderate or severe International HS Severity Score System (IHS4) stage. Adalimumab dose intensification significantly improved IHS4 score, Pain Index, HS-Physician Global Assessment, pain, and Cardiff Dermatology Life Quality Index. Two young female patients with HS and Crohn's disease developed psoriatic lesions during the treatment with adalimumab 80 mg/week s.c. CONCLUSION: An enhanced level of effectiveness was assessed in the majority of the HS patients treated with adalimumab dose intensification (80 mg/week s.c.). Larger studies are required to evaluate this observation.

Clindamycin versus clindamycin plus rifampicin in hidradenitis suppurativa treatment: Clinical and ultrasound observations.

BACKGROUND: Antibiotics are recognized as first-line treatments for hidradenitis suppurativa (HS), but the data on their efficacy are limited. OBJECTIVE: Evaluate the efficacy of oral clindamycin versus that of clindamycin plus rifampicin in patients with HS. METHODS: A total of 60 patients with mild-to-moderate-severe HS who were classified according to their International Hidradenitis Suppurativa Severity Score System (IHS4) and Hurley scores, were subdivided into 2 groups of 30 patients each (group A, the members of which received clindamycin plus rifampicin, and group B, the members of which were treated with clindamycin alone) and retrospectively studied. The main objective was to evaluate and compare the clinical and ultrasound responses between the groups after 8 weeks of treatment according to the Hidradenitis Suppurativa Clinical Response measure. RESULTS: After the treatment, 17 of 30 patients in group A and 19 of 30 in group B met the primary outcome. Both groups showed a similar improvement of IHS4 score, whereas the Dermatology Life Quality Index and pain Visual Analogue Scale scores improved more in group B. In particular, the reductions in nodule and abscess counts were similar between the 2 groups, whereas the number of draining tunnels decreased more in group B. The factors significantly associated with Hidradenitis Suppurativa Clinical Response score were age, body mass index, IHS4 score, and absence of axillary involvement. Disease-free survival was similar between the 2 groups. LIMITATIONS: The study was not randomized or placebo-controlled. CONCLUSION: Clindamycin may be a useful treatment alternative to antibiotic combination regardless of HS clinical stage.