Il gene KIT nella mastocitosi familiare

Familial cutaneous mastocytosis is an exceptional condition of unknown etiology. In this study we report the largest series of patients with familial cutaneous mastocytosis without other manifestations (18 affected subjects from seven unrelated families), and we investigate the role of germ-line KIT mutations in the pathogenesis of the disease. The mean age at onset was 5.4 years (range from birth to 22 years), and the clinical behavior was variable over a mean follow up period of 15.1 years (range 2-36): improvement in seven, stability in eight and worsening in the remaining three patients. The pattern of inheritance was compatible with an autosomal dominant trait with incomplete penetrance; a female preponderance (14 females vs 4 males, ratio 3.5:1) was noted; among the six women who have been pregnant at least once, three experienced important clinical changes during pregnancy. No germ-line mutation was found in the exons 10, 11, and 17 of the KIT proto-oncogene, which are the most commonly mutated exons in sporadic mastocytosis. However, in the majority of affected subjects we found the Met541Leu polymorphic variant of the KIT gene, which seems to confer a growth advantage to mast cells in vitro. This observation further suggests that the Met541Leu may be a predisposing factor of cutaneous mastocytosis, although it seems to be neither necessary nor sufficient for the development of the disease

Silver nitrate for Kaposi’s sarcoma nodules: A new look at an old treatment

Introduction: Fungating classic Kaposi’s sarcoma (KS) tumors represent a therapeutic challenge given the increased risk of resistance to traditional local chemotherapy and the frequent concomitant skin infections. Objective: To study the effect and tolerability of silver nitrate cauterization in patients with fungating KS tumors. Methods: A single arm, prospective study in patients with soft, fungating and/or oozing KS tumors cauterized with silver nitrate sticks. The application was repeated once every three weeks until the lesion became fibrous or after two consecutive applications without any clinical improvement. Results: Eleven patients (10 males, one female, mean age of 75.8 years) with at least one fungating KS tumor of the lower limbs were treated. Complete or partial resolution of the KS lesion was achieved in 91% of patients after an average of 2.1 treatments (range, 1–4 treatments). Conclusions: Fungating KS tumors can be treated simply, effectively and safely in the office with silver nitrate cauterization

Non-ablative fractionated laser skin resurfacing for the treatment of aged neck skin

Abstract Background: Aging of the neck skin includes poikiloderma of Civatte, skin laxity and wrinkles. While the vascular alterations of poikiloderma of Civatte can be effectively treated with lasers or intense pulsed light, a successful treatment of dyschromia, skin laxity and wrinkles is still difficult to achieve. Objective: To evaluate the safety and efficacy of non-ablative fractional 1540 erbium glass laser for the treatment of aged neck skin, also by means of in vivo reflectance confocal microscopy (RCM). Methods: A prospective study for neck resurfacing in 18 women with aged neck skin. Six laser treatments were performed in 4-week intervals with a 1540-nm erbium-glass fiber laser. Results: By using a 6-point grading scale, the mean score (±SD; range) at baseline was 3.6 (±1.5; 1-6) for skin dyschromia, 2.9 (±1.4; 1-6) for laxity and 3.3 (±1.3; 1-5) for wrinkles. Three months after the last laser session, we found a significant clinical improvement of dyschromia (p = 0.0002; Wilcoxon test), and wrinkles (p = 0.0004; Wilcoxon test), with a mean (±SD) reduction of 2.5 (±1.0) and 1.9 (±1.1) points in the 6-point grading scale, respectively. No change was observed in laxity. These results were also supported by structural changes documented by RCM. Conclusion: Non-ablative fractional 1540 erbium glass laser was both safe and effective for the treatment of dyschromia and wrinkles, but not effective for the laxity of the neck skin

Prevalence of antibodies against Kaposi's sarcoma associated herpes virus (KSHV) complement inhibitory protein (KCP) in KSHV-related diseases and their correlation with clinical parameters.

Kaposi's sarcoma-associated herpes virus (KSHV) encodes its own inhibitor of the complement system, designated KSHV complement control protein (KCP). Previously, we detected anti-KCP antibodies in a small group of 22 patients suffering from Kaposi's sarcoma (KS) and KSHV-related lymphoproliferative diseases (Vaccine, 25:8102-9). Anti-KCP antibodies were more prevalent in individuals suffering from KSHV-related lymphomas than KS and also in those with high titer of antibodies against lytic KSHV antigens. Herein we analyze anti-KCP antibodies in 175 individuals originating from three different groups from northern Sweden or Italy, which included patients suffering from classical or HIV-associated KS, Multicentric Castleman's Disease, KSHV-associated solid lymphoma, pleural effusion lymphoma and healthy individuals with detectable KSHV immune response. Our current study confirmed previous observations concerning antibody prevalence but we also analyzed correlations between anti-KCP antibodies and classical KS evolution, clinical stage and viral load in body fluids. Furthermore, we show that patient's anti-KCP antibodies are able to decrease the ability of KCP to inhibit complement. This fact combined with results of statistical analysis suggests that KCP inactivation by specific antibodies may influence progression of classical KS

COVID-19 Vaccination in Patients with Classic Kaposi’s Sarcoma

The novel coronavirus disease 2019 (COVID-19) has represented an overwhelming challenge for worldwide health systems. Patients with cancer are considered at higher risk for severe COVID-19 and increased mortality in case of infection. Although data on the novel severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination in patients with cancer are limited, there is enough evidence supporting anti-infective vaccination in general in patients with active cancer, or with history of previous malignancy. Subjects with classic Kaposi’s sarcoma (KS) represent a small subset of cancer patients, which should be considered at heightened risk for infections due to several factors including age, and impaired immune function status. Several cases of human herpesviruses reactivation among critically ill COVID-19 patients have been described. Moreover, in case of severe infection and treatment with immunomodulating agents, patients with CKS are exposed at significant risk of viral reactivation and disease progression. Considering the baseline clinical risk factors of patients with CKS, and the complex interplay of the two viral agents, SARS-CoV-2 vaccination should be strongly recommended among patients with KS. KS represents an interesting field to study the interactions among chronic viral infections, SARS-CoV-2 and the host’s immune system. Prospective observational studies are needed to provide more insights on vaccine activity and safety among patients with cancer, optimal vaccine schedules, potential interactions with antineoplastic therapies, and other comorbidities including chronic viral infections

Combination of fractional erbium-glass laser and topical therapy in melasma resistant to triple-combination cream

Melasma is a common melanosis often difficult to treat

Melasma and low-energy Q-switched laser: Treatment assessment by means of in vivo confocal microscopy

Melasma is an acquired disorder of pigmentation for which several therapeutical options are currently available. Among them, low-energy Q-switched Nd:YAG laser (1,064 nm) has been successfully applied in case series although its long-term efficacy has yet to be proven. In vivo confocal microscopy is a novel imaging technique that permits to explore the skin tissue at a nearly histologic resolution. The aim of our study was to evaluate the laser treatment efficacy by means of confocal microscopy at different time points to elucidate the mechanism of action of the laser and its possible drawbacks. Eight women who presented with melasma on the face were subjected to low-energy Q-switched Nd:YAG laser treatment and then analyzed by means of confocal microscopy. An overall improvement of melasma was noticed clinically and microscopically by means of confocal microscopy. Four cases revealed the presence of dendritic-shaped cells upon confocal microscopy and those patients were the ones presenting with a relapse of the disease. The interpretation of these cells is that they could represent reasonably activated melanocytes. This opens the question whether laser treatment should be modulated to avoid side-effects associated with its use. Confocal microscopy has emerged as an excellent tool for a better understanding of the changes occurring during laser treatment

Nonablative fractional photothermolysis for acne scars: clinical and in vivo microscopic documentation of treatment efficacy

Fractional photothermolysis has been shown to improve various types of scarring, including atrophic acne scars. The aim of the present authors was to assess the efficacy and safety of the nonablative fractional photothermolysis in the treatment of moderate and severe acne scars. Eighty-seven patients with moderate or severe acne scarring were treated with six sessions with a 1540-nm Erbium glass fiber laser at 3-week intervals. Six months after the final session, 7/87 (8%) patients showed a moderate improvement, whereas 80/87 (92%) patients had a marked improvement. In a subset of patients, the present authors also applied in vivo reflectance confocal microscopy to highlight the relevant microscopic changes. Hence, early and late posttreatment findings, most importantly the replacement of a coarser collagen with a new one, similar to the collagen seen in healthy skin, were observed. In accordance to previous studies, the present authors conclude that nonablative fractional photothermolysis is a safe and effective treatment for moderate or severe acne scarring