Does Clearance of Vulvar Lichen Sclerosus after a Corticosteroid Treatment Correspond to a Decrease in Disease-Related Burden? Results from a Cohort Study Using Pictorial Representation of Illness and Self-Measure and the Dermatology Life Quality Index.

BACKGROUND AND OBJECTIVES: Complete clearance of vulvar lichen sclerosus (VLS) occurs in a minority of treated patients. Disease persistence may impact patient well-being. The main objective of this study was to assess if achieving a complete clearance with a corticosteroid treatment leads to a benefit in terms of patient suffering and quality-of-life (QoL) impairment. METHODS: We performed an observational study on a cohort of VLS women, who applied mometasone furoate 0.1% ointment for 12 weeks. At treatment completion (T1), we compared the patients who achieved clearance in symptoms (Global Subjective Score [GSS] = 0) or in objective features (Global Objective Score [GOS] = 0) or in both with those who achieved a lower degree of improvement, on the basis of Pictorial Representation of Illness and Self-Measure (PRISM) and Dermatology Life Quality Index (DLQI) scores. RESULTS: In the whole sample (n = 101), GSS, GOS, PRISM, and DLQI scores significantly improved after treatment from baseline; 34 patients (35.8%) achieved GSS = 0, 26 (25.7%) achieved GOS = 0, and 11 (11.5%) clearance of GSS and GOS. PRISM scores at T1 were significantly higher in patients who achieved clearance of symptoms when compared with those who did not, including patients achieving 50-99% GSS improvement from baseline. DLQI scores were lower in patients who achieved clearance of symptoms, signs, or both when compared with the others. CONCLUSIONS: VLS clearance corresponded to a significant improvement in the QoL of VLS patients, also in comparison with those who achieved a substantial but incomplete decrease of symptom and sign scores, and should become an ideal therapeutic goal.

DECISA Project (DErmatology Clinics in Italy: Survey on Alitretinoin): A real-life retrospective cohort multicenter study on 438 subjects with chronic hand eczema.

Alitretinoin is the only systemic agent approved to treat moderate-severe chronic hand eczema (CHE) unresponsive to potent topical corticosteroids. No nationwide Italian data regarding real-life efficacy, safety, and tolerability of treatment are available. The DECISA project (DErmatology Clinics in Italy: Survey on Alitretinoin) retrospectively examined data from a registry including 15 Dermatology Clinics authorized to prescription of alitretinoin for CHE patients. Disease severity was assessed at baseline, and after 3 and 6 months of treatment, using the 5-point Physician Global Assessment (PGA) and the modified Total Lesion-Symptoms-Severity (mTLSS) scores. Between November 2010 and July 2018, data of 248 male and 190 female patients (mean age 49.71 ± 13.20 years) treated with alitretinoin were collected. Of them, 43.2% had irritant contact dermatitis, 22.2% allergic contact dermatitis, 18.0% atopic dermatitis, 16.7% mixed (irritant/allergic) type of eczema. At 3 months, the 420 re-evaluated patients showed significantly reduced mTLSS and PGA (P < .0000001 vs baseline for both); PGA was clear/almost clear in 35.6% of cases. At 6 months, the 341 re-evaluated patients showed significant (P < .0000001) improvement of mTLSS and PGA vs baseline and 3 months (PGA clear/almost clear: 41.4%). Relapses occurred in 125 patients; 58 underwent an additional course of alitretinoin, with similarly good results. No relevant safety issues were reported; 86 patients experienced adverse effects, which forced 40 to prematurely stop treatment. The DECISA project results confirm the real-life efficacy, safety and tolerability of alitretinoin in the treatment of moderate to severe CHE refractory to standard topical therapies.

The link between hidradenitis suppurativa and phylloid hypomelanosis in partial trisomy-13 mosaicism: New evidences and further genetic/pathogenetic insights.

Partial trisomy-13 mosaicism (PT13M) is a rare condition. Among its possible associated cutaneous features, phylloid hypomelanosis (PH), characterized by leaf-like macules reminiscent of floral ornaments in the form of round or oval spots and patches and oblong lesions, is typical. Two cases of PH associated with hidradenitis suppurativa (HS) have been already reported in the literature. We report a third child with PH due to PT13M associated with HS-like lesions limited to hypomelanotic regions. We hypothesize that follicular occlusion genes may be located in the duplicated part of chromosome 13.

A New Hybrid Therapeutic Approach to Solitary Keratoacanthoma: Complete Recovery in Six Patients.

Introduction: Solitary keratoacanthoma (SKA) is generally considered as a well-differentiated form of squamous cell carcinoma, but it usually runs a benign course and a not aggressive behavior. Diagnostic criteria, prognosis, and treatment of SKA are not fully defined yet. Surgical treatment with fusiform excision represents the gold standard; nonoperative intralesional therapy of KA is uncommon but may provide a valid option in some categories of patients. Case Series Presentation: We report our experience regarding the treatment of SKA with a hybrid treatment consisting of a minimally invasive technique such as curettage followed by intralesional corticosteroid administration in the same session. Six patients affected with KA were treated ending in a complete resolution, with good esthetic outcome, no relapse after 1 year, and satisfaction of the patients. Discussion and Conclusion: The combined treatment allows us on the one hand to avoid radical surgery in selected patients and particular anatomic areas and on the other the side effects that the use of intralesional chemotherapy/immunosuppressive drugs can entail.

Seven years-experience of adalimumab therapy for Hidradenitis Suppurativa in a real-life dermatologic setting.

INTRODUCTION: Hidradenitis Suppurativa (HS) often causes severe impairment of the quality of life of patients affected, as it is characterized by recurrent relapses of inflammation and predisposes to retractive scars, with severe alteration of anatomy of the affected regions. Adalimumab is currently the only approved long-term biological therapy for this disease. MATERIAL AND METHOD: We retrospectively review the data of HS patients treated with Adalimumab at the 'Hidradenitis Suppurativa Clinic', University of Ferrara, Italy since the drug was first available to October 2020. The aim is to describe our real-life experience in a clinical outpatient service. We assessed the main demographic features, therapy duration, reasons of suspension and efficacy (evaluated by HiSCR - Hidradenitis Score) in relation to surgical procedures, hospitalization, number of areas involved by the disease and BMI > 30. We also assessed the aspects related to the use of adalimumab's biosimilar. RESULTS: Data on 76 patients, with a mean age of 38.26 ± 14.74 years and mean BMI 28.10 ± 5.92 were collected. Most of the treated patients had Hurley stage III (58/76); mean Sartorius score was 115.5 ± 55.86, mean IHS4 was 76.1 ± 44.3. A statistically significant correlation between hospitalization and cessation of adalimumab, the loss of the achievement of the HiSCR, and surgery was found. No need to do surgery was a protective factor against the failure of adalimumab treatment, meaning that the most severe cases are more likely to fail the biological therapy. CONCLUSION: New scenarios are opening up in clinical practice: the arrival of biosimilars allow greater sustainability of expenditure, while the anti-IL17 allow the patient who has failed therapy with adalimumab a valid and safe therapeutic option to be undertaken. A comprehensive care including hospitalization, a specific antibiotic therapy and surgical treatment is often mandatory to achieve a satisfactory control of the disease.

Vulvar Lichen Sclerosus from Pathophysiology to Therapeutic Approaches: Evidence and Prospects.

Vulvar lichen sclerosus (VLS) is a chronic, distressing, inflammatory disease with an enormous impact on quality of life. Treatment goals are relieving symptoms, reversing signs and preventing anatomical changes. Despite the availability of numerous therapeutic options, treatment outcome may not be entirely satisfactory and a definitive cure does not exist. This may be due to the fact that the exact VLS etiopathogenesis remains unknown. The objectives of this paper were to review the most up-to-date knowledge on VLS etiopathogenesis and to consider the available therapies through the lens of a plausible pathogenetic model. An electronic search on both VLS etiopathogenesis and its treatment was performed using the National Library of Medicine PubMed database. Based on current knowledge, it is conceivable that various, heterogeneous environmental factors acting on a genetic background trigger an autoimmune, Th-1 response, which leads to a chronic inflammatory state. This, in turn, can determine both tissue and micro-vascular injury and activation of signaling pathways involved in fibroblast and collagen metabolism. This pathogenetic sequence may explain the effectiveness of anti-inflammatory treatments, mostly topical corticosteroids, in improving VLS clinical-pathological changes. Further deepening of the disease pathways will presumably allow key mediators to become new therapeutic targets and optimize the available treatments.

The Association among Pyoderma Gangrenosum, Ulcerative Colitis, and Hidradenitis Suppurativa and the Syndromic Hidradenitis Suppurativa Network: A Case Report.

Hidradenitis suppurativa (HS), together with other inflammatory diseases, is involved in a syndromic network where different combinations of signs and symptoms characterize the definition. The observation of the concurrent occurrence of HS, pyoderma gangrenosum (PG), and inflammatory bowel disease (IBD), in detail ulcerative colitis (UC), led the authors to describe a new association. The patient, a 36-year-old woman, who saw IBD as the first appearing condition, shortly followed by HS and PG, was referred because of a clinical situation quickly worsening. A severe aggravation of both GI symptoms and general systemic situation total led to total colectomy. Surprisingly, shortly after the radical surgical treatment of UC, the cutaneous manifestations of HS and PG with no specific treatment almost completely disappeared suggesting the existence of a common etiopathogenetic mechanism and possibly an inductor role of UC on the other disorders. The presentation of this case offers the opportunity to deal with the fact that the resolution of one of the associated conditions may lead to the clearance of one or more of the others. It confirms a pathogenetic link between them and the pivotal role of one of them, in this case colitis.