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Bullous Pemphigoid (BP) is a common autoimmune blistering disorder with unknown aetiology. During the last decades, its association with various common comorbidities, including cardiovascular, metabolic, neuropsychiatric, and neoplastic disorders, has been established. However, in recent years an increasing number of BP cases have also been reported in association with rarer diseases, including Acquired Reactive Perforating Collagenosis (ARPC). Patients with coexisting BP and ARPC have been reported to share common clinical features including metabolic comorbidities e.g., Diabetes Mellitus (DM). As the evolution from ARPC cutaneous involvement to classic BP lesions has been more frequently described, it has been suggested that it may represent a new clinical variant of BP with a specific pathogenetic background. Here is reported a challenging case in which a typical onset of BP was later followed by the eruption of atypical ARPC lesions in a patient with multiple non-compensated metabolic and cardiovascular comorbidities.

Psoriasis is a chronic immune-mediated inflammatory cutaneous disease characterized by elevated levels of inflammatory cytokines and adipokine Lipocalin-2 (LCN-2). Recently, natural plant-based products have been studied as new antipsoriatic compounds. We investigate the ability of a leaf extract of the marine plant Posidonia oceanica (POE) to inhibit psoriatic dermatitis in C57BL/6 mice treated with Imiquimod (IMQ). One group of mice was topically treated with IMQ (IMQ mice) for 5 days, and a second group received POE orally before each topical IMQ treatment (IMQ-POE mice). Psoriasis Area Severity Index (PASI) score, thickness, and temperature of the skin area treated with IMQ were measured in both groups. Upon sacrifice, the organs were weighed, and skin biopsies and blood samples were collected. Plasma and lesional skin protein expression of IL-17, IL-23, IFN-γ, IL-2, and TNF-α and plasma LCN-2 concentration were evaluated by ELISA. PASI score, thickness, and temperature of lesional skin were reduced in IMQ-POE mice, as were histological features of psoriatic dermatitis and expression of inflammatory cytokines and LCN-2 levels. This preliminary study aims to propose P. oceanica as a promising naturopathic anti-inflammatory treatment that could be introduced in Complementary Medicine for psoriasis.

Lupus erythematosus tumidus (LET) is a subset of cutaneous lupus erythematosus that generally presents with urticaria-like papules and plaques located on sun-exposed areas. Systemic treatment with antimalarials, especially hydroxychloroquine (HCQ), is the first-line systemic therapy for LET. Even if these drugs have a safe profile, side effects such as retinal toxicity, maculopapular rash, gastrointestinal upset, hemolytic anemia and blue-gray discoloration of the skin or the mucous membranes, have been rarely reported in the literature. Herein, we describe a rare case of a 46-year-old smoking woman with LET who developed a generalized myopathy after HCQ treatment.

It is well known that viral infections play a relevant role in inducing or protecting from autoimmune diseases, thus representing a major environmental factor in the disruption of the immune system in genetically susceptible individuals. Since the beginning of the Covid-19 pandemic a great number of clinical and epidemiological studies have demonstrated that SARS-CoV-2 infection is no exception to the rule by interfering on many different levels in the normal functioning of our immune system. Even though a growing number of case series and case reports has been cited in the literature linking the infection to the new onset of autoimmune diseases, to date very little has been reported concerning a possible correlation between the virus and the clinical resolution of any kind of autoimmune pathology. Here we describe an interesting case of abrupt and unexpected resolution of Lichen planus pemphigoides mucocutaneous lesions in a fully vaccinated patient after a mildly symptomatic SARS-CoV-2 respiratory infection and we speculate on the possible underlying mechanisms correlating the two events.

The term gluten-related disorders (GRD) refer to a spectrum of different clinical manifestations triggered by the ingestion of gluten in genetically susceptible individuals, including coeliac disease (CD), wheat allergy and non-celiac gluten sensitivity (NCGS). GRD are characterized by a large variety of clinical presentations with both intestinal and extra-intestinal manifestations. The latter may affect almost every organ of the body, including the skin. Besides the well-known association between CD and dermatitis herpetiformis, considered as the cutaneous specific manifestation of CD, many other muco-cutaneous disorders have been associated to GRD. In this review, we analyzed the main features of dermatological diseases with a proven association with GRD and those that improve after a gluten-free diet, focusing on the newly described cutaneous manifestations associated with NCGS. Our main hypothesis is that a “cutaneous-gluten sensitivity,” as specific cutaneous manifestation of NCGS, may exist and could represent a diagnostic marker of NCGS.

Cutaneous vasculitis (CV) is an inflammatory skin-limited vascular disease affecting the dermal and/or hypodermal vessel wall. From the pathogenetic point of view, idiopathic forms are described as well as the induction from various triggers, such as drugs, infections, and vaccines. Following SARS-CoV-2 pandemic outbreak, cases of CV induced by both COVID-19 and COVID-19 vaccinations have been reported in literature. The aim of our work was to collect multiple cases available in the literature and analyze the frequency of the different forms of induced vasculitis, as well as their histological and immunopathological features. Although rare, CV induced by Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) and vaccines may provide interesting insights into the pathogenesis of these inflammatory processes that may in the future be useful to understand the mechanisms underlying cutaneous and systemic vasculitis.

Management of cutaneous lupus erythematosus (CLE) involves a combination of preventive measures, topical and systemic drugs, fairly similar for the different subtypes. Although guidelines exist, to date, no specific drugs have been specifically licensed for CLE. Antimalarials remain the first-line systemic treatment, but many patients do not respond, making refractory lupus a challenge for clinicians. The choice of alternative medication should be based on effectiveness, safety and cost. Most of the available drugs for CLE have been adapted from systemic lupus erythematosus (SLE) treatment but the existing literature is limited to small studies and evidence often lacks. As knowledge of pathogenesis of both CLE and SLE is improving, promising new therapies are emerging. In this review, we discuss the available medications, focusing on the novelties under development for CLE.

IntroductionPemphigus encompasses a group of muco-cutaneous autoimmune bullous diseases characterized by the loss of adhesion between keratinocytes. The disease is associated with increased morbidity and mortality.Materials and MethodsWe characterized clinical patterns, survival, comorbidities, and drug prescriptions in patients with pemphigus referred to the Section of Dermatology of the University of Florence from January 2010 to December 2021.ResultsA total of 149 patients were identified (female/male sex ratio = 2.0). Median age at diagnosis was 57.7 ± 17.2 years; 108 patients were diagnosed with pemphigus vulgaris (PV) (72.5%) and 35 (23.5%) with pemphigus foliaceus (PF). Paraneoplastic pemphigus (PNP) and IgA-pemphigus accounted for three patients each. The overall survival rate was 86.9%. Accordingly, 14 (9%) patients died during the study period. The average age at death was 77.8 ± 9.3. Age at diagnosis was a risk factor for death in patients with pemphigus. Average concentration of Dsg3-IgG and Dsg1-IgG was 85.6 ± 68.8 and 75.9 ± 68.4, respectively. The most serious comorbid diseases included cerebro- and cardiovascular accidents and malignancies. Regarding the treatment regimen, we found a substantially stable use of systemic steroids in the 2010–2018 period; the prevalence of use of mycophenolic acid increased, whereas that of azathioprine decreased. The use of rituximab showed the highest increase in the 2013–2018 period. Proton-pump inhibitors and antibiotics were the most frequently prescribed non-immunomodulating drugs.ConclusionsIn this large series of the patients, patients with pemphigus showed a high incidence of serious comorbid diseases, highlighting the importance of a multidisciplinary approach for a proper management of the patients. Rituximab was the immunomodulating drug showing the highest increase in use over time, reflecting the growing evidence of its efficacy as a first-line treatment in pemphigus.

Background: A current popular aesthetic problem, especially among younger women, is striae distensae (SD), also referred to as “stretch marks.”. Aim: The potential use of the 675 nm laser has been investigated in the treatment of SD. Methods: Patients underwent three sessions of the 675 nm laser with a 1-month interval between sessions. A total of three sessions were performed. The Manchester Scar Scale was used to assess stretch mark changes, and the mean scores related to each parameter at baseline and 6M FU after the last treatment session were measured. A clinical photographic evaluation was performed to show the aesthetic improvement of SD. Results: The patients’ treated areas were the abdomen, thighs, buttocks, and breasts. Mean scores related to each Manchester Scar Scale parameter, with their relative percentage change, at baseline and 6M FU after the last treatment session were significantly improved. The total mean Manchester Scar Scale score significantly diminished from 14.16 (±1.30) to 10.06 (±1.32) at 6M FU (p < 0.01). The clinical photographs showed promising aesthetic SD improvement. Conclusions: 675 nm laser therapy demonstrated a good tolerance for the treatment of stretch marks applied to various body areas preventing any discomfort for the patient and with a significant improvement in skin texture.

BackgroundA few studies on vaccination in patients with rheumatic diseases, including arthritis, connective tissue diseases, vasculitis, and psoriatic arthropathy (PsA), demonstrated reduced production of neutralizing antibodies to SARS-CoV-2 Spike RBD (receptor-binding domain contained in the N-terminal of the S1 globular head region) when compared to the general population.ObjectiveThe aim of our study was to observe whether different therapies for PsA [methotrexate, anti-TNF antibodies, soluble TNF receptor (etanercept) or IL-17 inhibitors] have a different impact on SARS-CoV-2 vaccination in a homogeneous population of patients.MethodsWe enrolled 110 PsA patients in remission, assessed with Disease Activity in PSoriatic Arthritis (DAPSA). Of these: 63 were in treatment with anti-TNF-α therapy (26 etanercept, 15 certolizumab, 5 golimumab, 17 adalimumab); 37 with anti-IL17 secukinumab; 10 with methotrexate. All patients underwent vaccination for SARS-CoV-2 with mRNA BNT162b2 vaccine. Assessment of absolute and percentage lymphocyte subsets and anti-SARS-CoV-2 Spike RBD IgG antibody value 3 weeks after the second vaccine dose were performed. In addition, the serum antibody levels of 96 healthy healthcare workers (HCW) were analyzed.ResultsThe mean disease activity assessed with DAPSA score was 2.96 (SD = 0.60) with no significant differences between patients under different medications (p = 0.779). Median levels of neutralizing antibodies to SARS-CoV-2 Spike RBD were 928.00 binding antibody unit (BAU)/mL [IQR 329.25, 1632.0]; 1068.00 BAU/ml [IQR 475.00, 1632.00] in patients taking MTX, 846.00 BAU/ml [IQR 125.00, 1632.00] in patients taking etanercept, 908.00 BAU/mL [IQR 396.00, 1632.00] in patients taking anti-IL17 and 1148.00 BAU/ml [IQR 327.00, 1632.00] in patients taking TNF-α inhibitors, without statistically significant differences between these groups. Mean serum antibody level of HCW group was 1562.00 BAU/ml [IQR 975.00, 1632.00], being significantly higher than in the patient group (p = 0.000816). Absolute and percentage count of lymphocyte subsets were not statistically different between the subgroups under different treatments and when compared with HCW.ConclusionsAs for other rheumatic diseases on immunomodulatory treatment, our data showed a reduced humoral response in PsA patients compared to the control group. However, antibody response did not significantly differ between groups treated with different medications.

Background: Studies have demonstrated a higher risk of nonmelanoma skin cancers (NMSC) and a modestly increased melanoma risk in patients with psoriasis. To date, no biomarkers predictive of evolution have been identified yet. Methods: The aim of this prospective case-control study was to investigate the potential role of neutrophil gelatinase-associated lipocalin (NGAL) as a predictive biomarker of skin cancers in psoriatic patients. Patients with a diagnosis of psoriasis were enrolled, as well as healthy subjects and patients with skin cancers as controls. Plasma protein expression of NGAL, metalloproteinases (MMP)-2, and MMP-9 was performed by an enzyme-linked immunosorbent assay (ELISA). In all the patients who developed skin cancer at follow-up, NGAL, MMP-2, and MMP-9 serum levels were dosed again. Results: Plasma NGAL levels were significantly higher in psoriatic patients with NMSC than without (182.3 ± 36.6 ng/mL vs. 139.9 ± 39.3 ng/mL) (p < 0.001). Plasma NGAL levels were significantly higher (p < 0.00001) in patients with psoriasis and NMSC than in patients with skin tumors without psoriasis (182.3 vs. 122.9). Patients with psoriasis who developed NMSC at follow-up showed increased plasma MMP-9 levels. Conclusion: NGAL seems to play a role in the pathogenesis of NMSC but not melanoma in patients with psoriasis.

Pemphigus vulgaris is an intraepidermal autoimmune mucocutaneous blistering disease whose etiopathogenesis includes various trigger factors, i.e., drugs and malignancies. We present a case of malignancy-exacerbated pemphigus vulgaris which required a careful diagnostic process in order to rule out paraneoplastic pemphigus, along with the challenges posed by the need of treating both cutaneous and oncologic diseases. Possible post-operative complications post-poned the start of first-line immunosuppressive treatment of pemphigus. Moreover, the infective risks had to be minimized during the peak of the COVID-19 pandemic in Italy. Intravenous immunoglobulins were chosen as “bridge” therapy before the tumor surgical excision, followed by rituximab in post-operative phase.

Koebner phenomenon, also known as isomorphic response, is the appearance of active skin lesions in sites of epidermal injury. This manifestation is characteristic of different dermatological diseases, such as lichen planus, vitiligo and psoriasis. We present the case of a psoriatic patient who experienced Koebner phenomenon after Mantoux test, followed by eruption of generalized plaque psoriasis.

The pandemic outbreak of Coronavirus Disease 2019 (COVID-19) led to the development of mRNA vaccines. With the extensive vaccination campaign performed worldwide, many adverse reactions to these drugs have been reported in the literature. Although most of them are mild and self-limiting, they may sometimes cause psychological stress and require efforts to make a differential diagnosis with other conditions. This is the case of lymphadenopathies and lymphedema in patients with a history of cancer. Herein we present a case of lymphedema of the arm developed ten days after a VAXZEVRIA COVID-19 vaccine shot in a patient who had concomitant signs and symptoms compatible with a diagnosis of dermatomyositis. It was later classified as paraneoplastic as instrumental investigation revealed a breast carcinoma contralateral to the site of vaccine injection. With this report we ponder an adverse reaction to COVID-19 vaccination with the aim of bringing new data for clinicians who face similar clinical presentations, particularly controversial for radiologists and oncologists.

Background: A healthy lifestyle is not always able to improve the abdomen’s appearance, especially in those patients who have undergone sudden weight changes. Objective: We aimed at evaluating the efficacy of combined microwaves and flat magnetic stimulation (FMS) to treat abdominal localized adiposity and laxity. Methods: Twenty-five patients were subjected to two treatment sessions per month on the abdominal area with microwaves. FMS was also performed twice per week, with a minimum of two days between each session for two months. The technology uses three types of different protocols: massage, muscle definition (shaping), and muscular strengthening. Measurements, including body mass index (BMI) and waist, and abdominal ultrasound were performed at baseline and three months after the last treatment session. Blood examinations were performed, and a 5-Likert scale questionnaire was used to assess patient satisfaction. Results: At follow-up, three months after the last treatment, the mean waist circumference (WC) was significantly reduced, and skin laxity improved in all patients (p < 0.001). A significant improvement in abdominal muscle tissue thickness was also shown in all abdominal areas, and the thickness of the adipose tissue evaluated by ultrasound was reduced. Conclusions: This study proves that the combination of microwaves and FMS treatment is secure and efficient for treating abdominal subcutaneous fat and skin laxity.

We read with great interest the recent article by Machona MS et al. [1] about a 30-year-old female with a long history of itch and skin eruption associated to abdominal pain, nausea and vomiting. At clinical examination, she had generalised, symmetrical polymorphic skin lesions located on the trunk, buttocks, extensor surface of the lower limbs and upper limbs. According to skin morphology and histopathology a diagnosis of dermatitis herpetiformis (DH) with suspected celiac disease (CD) was made and the patient underwent a gluten-free diet (GFD) associated to dapsone treatment, with the improvement of the signs and symptoms in the follow-up period. In our opinion, the diagnosis in this case could be consistent with linear IgA bullous dermatosis (LABD) more than DH, and the clinical and histopathological examinations could not be considered criteria sufficient enough to establish adiagnosis of DH. Clinically, the patient presented tense blisters in a pearl necklace-like arrangement on the extremities. These lesions are morphologically consistent with LABD more than DH [2,3]. Moreover, the post-inflammatory hypo and hyper-pigmentated maculae on the abdomen are not typical of DH, whose lesions usually clear without post-inflammatory dyschromia, while they could be seen in LABD (Figure 1).

A 26-year-old male with a medical history of risk sexual behavior, is brought to the Emergency Room (ER) with complaints of dyspnea associated with an oral cavity mass. At the interrogation, he referred that the symptomatology started 2 months ago with a lesion of approximately 1cm, originated from the upper gum. Patient also reported dysphonia, and dysphagia due to the expansion of the mass, and a weight loss of 10 kilograms.

Dermatitis herpetiformis (DH) is an inflammatory disease of the skin, considered the specific cutaneous manifestation of celiac disease (CD). Both DH and CD occur in gluten-sensitive individuals, share the same Human Leukocyte Antigen (HLA) haplotypes (DQ2 and DQ8), and improve following the administration of a gluten-free diet. Moreover, almost all DH patients show typical CD alterations at the small bowel biopsy, ranging from villous atrophy to augmented presence of intraepithelial lymphocytes, as well as the generation of circulating autoantibodies against tissue transglutaminase (tTG). Clinically, DH presents with polymorphic lesions, including papules, vesicles, and small blisters, symmetrically distributed in typical anatomical sites including the extensor aspects of the limbs, the elbows, the sacral regions, and the buttocks. Intense pruritus is almost the rule. However, many atypical presentations of DH have also been reported. Moreover, recent evidence suggested that DH is changing. Firstly, some studies reported a reduced incidence of DH, probably due to early recognition of CD, so that there is not enough time for DH to develop. Moreover, data from Japanese literature highlighted the absence of intestinal involvement as well as of the typical serological markers of CD (i.e., anti-tTG antibodies) in Japanese patients with DH. Similar cases may also occur in Caucasian patients, complicating DH diagnosis. The latter relies on the combination of clinical, histopathologic, and immunopathologic findings. Detecting granular IgA deposits at the dermal-epidermal junction by direct immunofluorescence (DIF) from perilesional skin represents the most specific diagnostic tool. Further, assessing serum titers of autoantibodies against epidermal transglutaminase (eTG), the supposed autoantigen of DH, may also serve as a clue for the diagnosis. However, a study from our group has recently demonstrated that granular IgA deposits may also occur in celiac patients with non-DH inflammatory skin diseases, raising questions about the effective role of eTG IgA autoantibodies in DH and suggesting the need of revising diagnostic criteria, conceivably emphasizing clinical aspects of the disease along with DIF. DH usually responds to the gluten-free diet. Topical clobetasol ointment or dapsone may be also applied to favor rapid disease control. Our review will focus on novel pathogenic insights, controversies, and management aspects of DH.

Background: The dermatological manifestations associated with intestinal diseases are becoming more frequent, especially now when new clinical entities, such as Non-Celiac Gluten Sensitivity (NCGS), are identified. The existence of this new entity is still debated. However, many patients with diagnosed NCGS that present intestinal manifestations have skin lesions that need appropriate characterization. Methods: We involved 17 patients affected by NCGS with non-specific cutaneous manifestations who got much better after a gluten free diet. For a histopathological and immunopathological evaluation, two skin samples from each patient and their clinical data were collected. Results: The median age of the 17 enrolled patients affected by NCGS was 36 years and 76% of them were females. On the extensor surfaces of upper and lower limbs in particular, they all presented very itchy dermatological manifestations morphologically similar to eczema, psoriasis or dermatitis herpetiformis. This similarity was also confirmed histologically, but the immunopathological analysis showed the prevalence of deposits of C3 along the dermo-epidermal junction with a microgranular/granular pattern (82%). Conclusions: The exact characterization of new clinical entities such as Cutaneous Gluten Sensitivity and NCGS is an important objective both for diagnostic and therapeutic purposes, since these are patients who actually benefit from a GFD (Gluten Free Diet) and who do not adopt it only for fashion.

Dermatitis herpetiformis (DH) is a rare autoimmune disease linked to gluten sensitivity with a chronic-relapsing course. It is currently considered to be the specific cutaneous manifestation of celiac disease (CD). Both conditions are mediated by the IgA class of autoantibodies, and the diagnosis of DH is dependent on the detection of granular deposits of IgA in the skin. There is an underlying genetic predisposition to the development of DH, but environmental factors are also important. This paper describes these different factors and discusses the known mechanism that lead to the development of skin lesions.

Dermatitis herpetiformis (DH) is an inflammatory cutaneous disease with typical histopathological and immunopathological findings clinically characterized by intensely pruritic polymorphic lesions with a chronic-relapsing course. In addition to classic clinical manifestations of DH, atypical variants are more and more frequently reported and histological and immunological are added to them, whereas the impact on quality of life of patients with DH is increasingly important to a certain diagnosis. The aim of this paper is to describe all the possible clinical, histological, and immunological variants of DH in order to facilitate the diagnosis of a rare disease and, therefore, little known.