Therapeutic approaches and targets for treatment of autoimmune bullous diseases.

Autoimmune bullous diseases are a heterogeneous group of diseases characterized by the development of cutaneous and mucosal vesicles, blisters, and finally erosions. The common pathogenetic mechanism is the presence of autoantibodies targeting structural proteins of the skin and mucous membranes (demosomes and hemidesmosomes): in the case of pemphigus, the antigens are intraepidermal, whereas in the case of pemphigoid, dermatitis herpetiformis, and epidermolysis bullosa acquisita they are subepidermal. Mucosal involvement typically affects the oral and ocular mucosa, but in some cases, the upper airways or the upper digestive tract are affected. The burden on patients' lives could be severe due to the impairment of normal feeding or breathing. In other cases, they may represent paraneoplastic syndromes. Since autoimmune bullous diseases may result in significant morbidity and mortality, depending on the grade of cutaneous and mucosal involvement, a prompt therapeutic approach is mandatory and, in recalcitrant cases, may be challenging. The first line therapy consists of corticosteroids, both topical and systemic. Once remission or control of the acute phase is obtained, adjuvant therapies need to be introduced in order to spare the corticosteroid load and minimize side effects such as iatrogenic diabetes or osteoporosis. Herein, we describe all current therapeutic approaches to autoimmune bullous diseases, also including emerging therapies.

Lichen sclerosus with enlarged vessels: A variant of lichen sclerosus in young girls.

Vascular findings have rarely been described in the setting of lichen sclerosus. Enlarged vessels within the atrophic plaques have been observed on the free margins of the labia minora and clitoral hood. The enlarged vessels completely remit upon treatment of lichen sclerosus with an ultra-potent corticosteroid ointment in the acute phase. During maintenance therapy with calcineurin inhibitors, there was no recurrence of the enlarged vessels.

Congenital fibrous hamartoma of the tip of the tongue: A novel peculiar entity.

Congenital fibrous hamartoma of the tip of the tongue, a peculiar and novel entity, consists of one or two asymptomatic pearly or yellowish nodules, not exceeding 0.5 cm in maximum diameter, at the tip of the tongue, ventrally or dorsally. Unlike other localizations, congenital fibrous hamartoma of the tip of the tongue is not associated with cleft lip or palate, or with feeding problems. Surgical excision should be avoided, reserved only for dubious cases, since the lesions are benign and stable over time.

Contact dermatitis due to masks and respirators during COVID-19 pandemic: What we should know and what we should do.

Given the current lack of a therapeutic vaccine for coronavirus disease 2019 (COVID-19), preventive measures including mask wearing are crucial in slowing the transmission of cases. However, prolonged wearing of protective respirators, medical and fabric masks can easily generate excessive sweating, moisture and friction. Closed and warm environments heighten the skin's permeability and sensitivity to physical or chemical irritants, leading to chronic cumulative irritant contact dermatitis or, rarely, even allergic contact dermatitis. Although not representing a life-threatening condition, contact dermatitis can have a significant impact on emergency management, as it is potentially able to reduce work performance and create emotional discomfort due to the involvement of evident body areas. To minimize the skin breakdown, adherence to standards on wearing protective and safe equipments and avoidance of overprotection should be performed. At the same time, some measures of skin care are recommended. Here, we offer some tips on how to prevent and manage contact dermatitis due to masks not only in health care workers, but also in the general population during this COVID-19 outbreak.

Severe de-novo palmoplantar and nail psoriasis complicating Nivolumab treatment for metastatic melanoma.

Nivolumab, a fully human IgG4 immune checkpoint modulator, binds to the programmed cell death 1 (PD-1) receptor on T cells and blocks their inhibition. Thus, it increases the anticancer host immune response by allowing T cells to attack tumor cells. Although anti-PD-1 immunotherapy is typically well accepted, deregulation of immune tolerance caused by nivolumab may determine immune-related adverse events, among which skin toxicities represent the most common. We report a case of severe new-onset palmoplantar and nail psoriasis after receiving nivolumab treatment for metastatic melanoma.

Sonographic features of vaccination granulomas in children with delayed-type hypersensitivity to aluminum.

In this article, we report the sonographic features of vaccination granulomas in three children sensitized to aluminum. Although the recognition of the vaccination granuloma relies on the clinical examination, misdiagnosis is frequent, leading to distressful procedures or prolonged antibiotic administration. In all our cases, sonography revealed a teardrop-shaped echogenic central structure, suggesting the deposition of aluminum crystals along the route of administration with consequent subcutaneous degenerative changes, and a surrounding hypoechoic cap, which reflects the changeable inflammatory reaction and the granuloma formation.

Efficacy and safety of biosimilar rituximab in the treatment of pemphigus vulgaris: a single center experience of 12 cases.

OBJECTIVE: Rituximab, a chimeric monoclonal antibody that targets the CD-20 molecule on B-cells' surface, has led to significant advances in the treatment of autoimmune pemphigus in recent decades. The aim of the study was to assess the clinical efficacy as well as safety data for biosimilar rituximab with emphasis on the treatment of pemphigus vulgaris. METHODS: A total of 12 individuals with pemphigus vulgaris treated with biosimilar rituximab were followed for at least 1 year. We assessed patient characteristics, disease history, pemphigus disease area index (PDAI) score, anti-desmoglein (Dsg)1 and anti-Dsg3 antibody level, clinical response, and any adverse events during each clinical follow-up visit. We also recorded time to achieve complete remission, duration of complete remission and time to relapse. RESULTS: A consistent decrease in the PDAI score was observed (p<.0001), as well as a significant decrease of anti-Dsg3 values. In addition, the clinical response of this population confirmed that the equivalent is not inferior to Rituximab (p=.521). CONCLUSIONS: The introduction or the switch to Rixathon, a rituximab biosimilar, appears to be safe and may provide an opportunity to reduce health system costs because of its similarity to the reference drug in terms of safety and efficacy.