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Background: Data on outcomes of Coronavirus disease 2019 (COVID-19) infection in autoimmune bullous diseases (AIBDs) patients is scarce. Materials and methods: This single-centre survey-based-observational study included patients registered in the AIBD clinic of the Postgraduate Institute of Medical Education and Research, Chandigarh, India. All registered patients were contacted over telephone between June and October 2021. A survey was conducted after obtaining informed consent. Results: Among 1389 registered patients, 409 completed the survey. Two hundred and twenty-two (55.3%) patients were females and 187 (45.7%) were males. The mean age was 48.52 ± 14.98 years. Active disease was reported by 34% patients. The frequency of COVID-19 infection in responders was 12.2% (50/409), with a case-fatality ratio of 18% (9/50). Rituximab infusion after the onset of pandemic significantly increased the risk of COVID-19 infection. Active AIBD and concomitant comorbidities were significantly associated with COVID-19 related death. Limitations: Relative risk of COVID-19 infection and complications among AIBD patients could not be estimated due to lack of control group. The incidence of COVID-19 in AIBD could not be determined due to lack of denominator (source population) data. Other limitations include telephonic nature of the survey and lack of COVID-19 strain identification. Conclusion: Use of rituximab is associated with higher probability of COVID-19 infection, while advanced age, active disease and presence of comorbidities may increase the risk of COVID-19 mortality in AIBD patients. [ABSTRACT FROM AUTHOR]

Background Autoimmune bullous diseases (AIBDs) are a group of chronic blistering diseases that affect mucocutaneous membranes. These diseases are common worldwide and affect males and females of all races. Treatment options include corticosteroids, rituximab, and topical agents. We evaluated the epidemiology of AIBD in Kerman, Iran, over a five-year period. Methods This retrospective study was conducted on 100 patients diagnosed with an AIBD between 2015-2020. All patients were included in this study by census method. Factors like age, sex, disease type, and age at diagnosis were evaluated. Data analyses were performed using SPSS version 26. Results One hundred patients participated in this study, and the male-to-female ratio was 1:1.08. The mean age of participants was 51.99±19.52 years, and the mean age at diagnosis was 49.19±8.31. Out of 100 patients, 82 suffered from pemphigus disease (70 pemphigus vulgaris; 12 pemphigus foliaceus), 17 had bullous pemphigoid, and one patient had pemphigus gestationis. Conclusion In many countries, pemphigus is the most common AIBD. Although AIBDs are uncommon, the diagnosed cases increase with age. [ABSTRACT FROM AUTHOR]

Bullous pemphigoid (BP) is an autoimmune blistering skin disease. Current treatment strategies are limited by their efficacy and/or side effect profile and the need for safer and effective alternatives is undeniable. We aimed to conduct a systematic review focusing on the efficacy and safety of omalizumab in BP patients. Embase, PubMed, Cochrane, and clinicaltrials.gov were searched for English and French articles published from inception to July 1, 2021, using search terms "omalizumab" OR "Xolair" OR "IGE025" OR "olizumab" AND "bullous pemphigoid." Screening and data extraction was performed by two raters independently. The primary outcome was complete response (CR), and secondary outcomes were partial response (PR), flare-ups, adverse events/vital status. In total, 22 articles were included, with a total of 56 patients. All patients had a refractory BP with mean disease duration of 13.5 ± 20.2 months (Standard Deviation (SD)) and failed 3.1 ± 1.6 therapies and many remained corticosteroids dependent. Overall, 87.5% of patients responded to treatment (55.4% CR and 32.1% PR), 7.1% discontinued the protocol and only 5.4% were non responders. A third of patients were able to discontinue all other therapies and most others were able to discontinue or taper systemic corticosteroids to <10 mg daily. Flare-ups occurred in 57.7% of patients upon discontinuation of omalizumab and/or steroid tapering, most patients recaptured response thereafter. Omalizumab was well tolerated by most patients. Omalizumab appears to be a promising treatment for BP with a good response rate and safety profile. However, several limitations were identified in current literature, and highlight the need for randomized controlled trials of omalizumab in BP. [ABSTRACT FROM AUTHOR]

Although relatively uncommon, autoimmune bullous diseases carry the risk of increased mortality and can significantly impact quality of life. This group of diseases is broad and encompasses subepidermal conditions such as bullous pemphigoid, cicatricial pemphigoid, epidermolysis bullosa acquisita, dermatitis herpetiformis, and linear IgA bullous dermatosis, as well as intraepidermal conditions such as pemphigus and its variants. The pathophysiology of each condition is incompletely understood but broadly involves the formation of autoantibodies targeting skin adhesion proteins, a process that relies on a complex interplay between a dysregulated immune system, genetic predisposition, and environmental factors. We review the impact of nutrition on pathogenesis, clinical course, and treatment of various autoimmune bullous diseases. [ABSTRACT FROM AUTHOR]

A 60-year-old man with extensive erosive lesions within the scalp and face, limbs, trunk, and nasal and oral mucous membrane involvement was admitted to the ward with worsening of symptoms in the course of pemphigus vulgaris. The exacerbation took place during an attempt to reduce doses of immunosuppressive drugs and glucocorticosteroids. Additional examinations, otorhinolaryngological and dental consultation did not show another cause of this condition. Biological therapy with a monoclonal antibody directed against the CD20 antigen, rituximab, was introduced. The study presents the treatment efficacy in the patient 2 weeks after the last drug dose and compares them with the latest available knowledge on rituximab therapy in pemphigus vulgaris.

Type 2 immunity, illustrated by T helper 2 lymphocytes (Th2) and downstream cytokines (IL-4, IL-13, IL-31) as well as group 2 innate lymphoid cells (ILC2), is important in host defense and wound healing. 1 The hallmark of type 2 inflammation is eosinophilia and/or high IgE counts and is best recognized in atopic diathesis. Persistent eosinophilia, such as seen in hypereosinophilic syndromes, leads to fibrosis and hence therapeutic Type 2 inhibition in fibrotic diseases is of high interest. Furthermore, as demonstrated in cutaneous T cell lymphoma, advanced disease is characterized by Th1 to Th2 switch allowing cancer progression and immunosuppression. Development of targeted monoclonal antibodies against IL-4Rα (eg, dupilumab) led to a paradigm shift for the treatment of atopic dermatitis (AD) and stimulated research to better understand the role of Type 2 inflammation in other skin conditions. In this review, we summarize up to date knowledge on the role of Type 2 inflammation in skin diseases other than AD and highlight whether the use of Type 2 targeted therapies has been documented or is being investigated in clinical trials. This manuscript reviews the role of Type 2 inflammation in dermatitis, neurodermatitis, IgE-mediated dermatoses (eg, bullous pemphigoid, chronic spontaneous urticaria), sclerodermoid conditions and skin neoplasms. [ABSTRACT FROM AUTHOR]

Epidermolysis bullosa simplex (EBS) is a rare autosomal dominant, genetic condition where bullous lesions, larger than 0.5 cm, affect an area of the skin that is exposed to mechanical friction or minor trauma. Prevention of the bullous lesions starts with family and patient education, with infants requiring greater care and control of their environment. Every individual with EBS will have a treatment plan specifically tailored to the severity and extent of skin involvement. This article provides a comprehensive overview of EBS, including diagnostic approach, preventative considerations, and current treatments. [ABSTRACT FROM AUTHOR]

Background: Psoriasis and autoimmune bullous diseases (BD) are chronic, recurrent, immune-mediated pathologies, characterized by skin eruptions and associated symptoms: itching, pain, burning and stinging. Their influence on patients' quality of life (QoL) can be significant, affecting both physical and emotional well-being, and leaving an impact on daily activities and social relationships. Objective: This study aims to evaluate the QoL in individuals with psoriasis and BD (pemphigus, bullous pemphigoid and dermatitis herpetiformis). Material and methods: A cross-sectional, descriptive study was conducted on 50 patients admitted to the Dermatovenerology Clinic of the Mureş Clinical County Hospital, between July 2023 and February 2024, and diagnosed with psoriasis (34 patients) and BD (16 patients, with the predominance of pemphigus). Data were collected using two questionnaires: one included information about the patient, skin lesions, symptoms and treatment; the other was the Dermatology Life Quality Index (DLQI) questionnaire, where a higher score indicates a greater impact on the individual's QoL. The statistical analysis included Pearson and Spearman's correlation, t-tests and the Mann-Whitney test, with a significance level of p<0.05. Results : Psoriasis and BD had a moderate effect on patients' QoL, with an average DLQI value of 7.61 ± 6.04 for psoriasis and 8.81 ± 4.38 for BD. The most impacted aspects of QoL were clinical symptoms and feelings, while work and school were the least influenced. Statistically significant differences were observed in the DLQI values between patients receiving local and systemic treatment (3.9 ± 3.3 for psoriasis and 5.62 ± 1.92 for BD) and those without any treatment (8.91 ± 5.35, p=0.01 for psoriasis, 12.83 ± 3.43, p=0.0003 for BD). Regarding psoriasis, combined local and systemic treatment showed a lower DLQI value (3.9 ± 3.3) compared to local treatment alone (11.33 ± 7.39, p=0.01); in addition, a positive correlation was found between DLQI and disease extent (r=0.56, p=0.0005). No other significant correlations were identified for either psoriasis or BD. Conclusions: Psoriasis and BD moderately impair individuals' QoL. Patients receiving both local and systemic therapies experienced a better QoL compared to those without any treatment. Specifically, for psoriasis, combined local and systemic therapies demonstrated superiority in promoting a better QoL compared to local treatment alone. These findings emphasize the importance of therapeutic medical intervention for both conditions, together with adequate psychological and social support. [ABSTRACT FROM AUTHOR]

Bullous diseases are a group of dermatoses primarily characterized by the presence of vesicles (0.1-0.9 cm) or bullae (>1 cm). There are various categories of bullous disease: allergic, autoimmune, infectious, mechanical, and metabolic. These diseases affect individuals in all decades of life, but older adults, age 65 and older, are particularly susceptible to bullous diseases of all etiologies. The incidence of these disorders is expected to increase given the advancing age of the general population. In this comprehensive review, we will outline the common bullous diseases affecting older individuals and provide an approach to evaluation and management., (Copyright © 2023 Elsevier Inc. All rights reserved.)

The American Society of Dermatopathology has established an Appropriate Use Criteria (AUC) Committee with the intention of establishing evidence‐based recommendations regarding the appropriateness of various ancillary tests commonly utilized by dermatopathologists. Periodic acid Schiff (PAS) and Grocott (or Gomori) methenamine silver (GMS) stains represent some of the most commonly employed ancillary tests in dermatopathology. The utility of these tests was targeted for evaluation by the AUC. This literature review represents a comprehensive evaluation of available evidence for the utility of PAS and/or GMS staining of skin and nail biopsies. In concert with expert opinion, these data will be incorporated into future recommendations by the AUC for PAS and GMS staining in routine dermatopathology practice. [ABSTRACT FROM AUTHOR]

A 60-year-old man with extensive erosive lesions within the scalp and face, limbs, trunk, and nasal and oral mucous membrane involvement was admitted to the ward with worsening of symptoms in the course of pemphigus vulgaris. The exacerbation took place during an attempt to reduce doses of immunosuppressive drugs and glucocorticosteroids. Additional examinations, otorhinolaryngological and dental consultation did not show another cause of this condition. Biological therapy with a monoclonal antibody directed against the CD20 antigen, rituximab, was introduced. The study presents the treatment efficacy in the patient 2 weeks after the last drug dose and compares them with the latest available knowledge on rituximab therapy in pemphigus vulgaris. [ABSTRACT FROM AUTHOR]

Introduction: 'Chronic inflammatory immune-related skin disease' (ISDs) is an umbrella term grouping together heterogeneous entities characterized by chronic inflammation potentially involving the whole skin. We are not covering all ISDs in this review, but take a few as the most representative, including nonbullous and bullous diseases. The question we are aiming to address can be summarized as follows: 'despite the differences, is it possible to define some unifying epidemiologic characteristics and shared progression pathways which can guide the organization of healthcare?' Areas covered: This review covers incidence, prevalence, risk factors and prognosis of psoriasis, atopic dermatitis (AD), pemphigus and pemphigoid. Medline, Embase and the Cochrane Library were searched for papers published between January 2005 and December 2019. Expert opinion: ISDs epidemiology varies according to the ISD type, age, sex, climate, and sociodemographic variables. AD and psoriasis pose a considerable public health burden owing to their high prevalence worldwide and morbidity. Their secular trend of increasing incidence points to a role for environmental factors and gene-environment interactions. Bullous diseases are much rarer, with limited data available. Worldwide, the leading cause of skin disease disability-adjusted life-years (DALYs) is attributable to AD. Future research should focus on risk factors and prevention at the global level. [ABSTRACT FROM AUTHOR]

Background: Bullous pemphigoid (BP) autoantibody levels are generally elevated in patients with BP but can be present nonspecifically in patients without BP.Objective: To analyze the clinical findings of patients with elevated BP180 or BP230 autoantibody levels and negative direct immunofluorescence (DIF) study findings.Methods: We retrospectively reviewed records of patients seen at our institution during January 1, 2005-December 31, 2015, who were positive for BP180 or BP230 autoantibodies and had a negative DIF study finding. These patients' demographic characteristics and BP180 and BP230 levels were compared with those of a BP control group who were positive for BP180 or BP230 autoantibodies and had positive DIF study findings.Results: We identified 208 patients with BP autoantibodies but without positive DIF study findings. These patients' mean age and enzyme-linked immunosorbent assay values were significantly lower than those of the control group. Dermatitis was the most common final clinical diagnosis. Of the 208 patients, 41 (19.7%) had at least 2 years' follow-up. Four patients had positive DIF results upon repeating the test and ultimately received pemphigoid diagnoses.Limitations: Retrospective design with limited follow-up.Conclusion: Patients might harbor serum BP autoantibodies in the context of a wide range of dermatoses. Low positive BP180 and BP230 autoantibody levels should not be overinterpreted as evidence for BP in the setting of a negative DIF. [ABSTRACT FROM AUTHOR]

Subepidermal autoimmune bullous diseases of the skin and mucosae comprise a large group of chronic diseases, including bullous pemphigoid, pemphigoid gestationis, mucous membrane pemphigoid, linear IgA bullous dermatosis, epidermolysis bullosa acquisita, and anti-p200 pemphigoid. These diseases are characterized by an antibody response toward structural components of the basement membrane zone, resulting in subepidermal blistering. The epidemiological features of these diseases vary substantially in different regions of the world. Observational studies investigating comorbidities and associations among patients with these diseases are inconsistent and sometimes inconclusive. This review provides a brief overview regarding each one of the subepidermal autoimmune bullous diseases. In addition, it summarizes the most recent understanding of the epidemiological features and associations of this group of organ-specific autoimmune diseases. [ABSTRACT FROM AUTHOR]

Regulatory T cells (Treg) are a phenotypically heterogeneous group ofcells, playing an important role in regulation of the immune response.To date, several subtypes of regulatory cells have been identified. Themost important of them are: lymphocytes CD4+CD25+, Tr1, Th3 andNK cells. Regulatory T lymphocytes act by secretion of suppressivecytokines (IL-10, TGF-β) or direct cell-to-cell interaction. Functionalabnormalities of regulatory T cells are important in pathogenesis ofmany diseases, especially autoimmune ones. Based on published studies,the review discusses the role of regulatory T cells in pathogenesisof selected bullous skin diseases.

Introduction: Erythema multiforme (EM) is characterised by acute cutaneous and mucous bullous lesions. Infections are the most common cause. The herpes virus is associated with 50% of cases of erythema multiforme. There are two clinical aspects, the minor and the major forms. Erythema multiforme minor represents a localised eruption of the skin. The major form presents with cutaneous and mucous (oral and/or genital) lesions and general symptoms. Observation: Three cases of recurrent EM are described. The first case involves mucous EM and the other two mucocutaneous EM. Discussion: Oral mucosal involvement consists of multiple bullous lesions and post-bullous erosions. The skin is affected by the typical target lesions, but this sign is inconsistent. Histology shows a detachment between the epithelium and basal lamina. Treatment is symptomatic with analgesics and local antiseptic. Topical corticosteroids may be prescribed for recurrent cases. Conclusion: The diagnosis of EM should be considered for recurrent oral ulcers associated with other mucous membranes. Target lesions on the skin are characteristic of EM. [ABSTRACT FROM AUTHOR]

Bullous pemphigoid ( BP) is an autoimmune subepidermal blistering disorder that has been reported to be associated with malignancies. Some authors described several cases of pemphigoid associated with malignancies ( PAM); however, the evidence of this correlation still remains controversial. Several theories have been postulated to explain the relationship between malignant neoplasms and BP; the main theory suggests that antibodies directed against tumor-specific antigens of malignant cells may cross-react with antigens (like BP antigens) in the basement membrane zone leading to the formation of blisters. We performed an extensive review of the English published work focusing on the epidemiology, the pathogenetic theories and the clinical and histological aspects of the disease. We identified 40 cases of PAM: of these, seven cases were associated with hematological malignancies and 33 with solid tumors. Physicians should be aware of the existence of PAM and we suggest an oncological screening in early-onset pemphigoid, in patients with a former oncological history, in those with signs and symptoms that could be related to a neoplasm and in BP refractory to common immunosuppressive therapy. [ABSTRACT FROM AUTHOR]

Introduction: Recent developments in implantology have led to an extension of its indications, especially regarding special care patients. This paper offers a review of the literature on the subject of implantology in cases of oral mucosal lesions. Methods: A review of the literature was performed using MEDLINE, without a date range, searching for articles describing oral implant treatments in patients with oral mucosal alterations. Results: Few cases of oral implant treatments in such patients are described. The results of the literature review showed that oral implant treatments greatly enhance the quality of life of these patients. Discussion: Although these pathologies used to be considered contraindications to dental implant placement, recent data shows the feasibility of such treatments. Conclusion: Nonetheless, both strict oral hygiene and regular maintenance are essential to intercept any potential pathology reactivation. [ABSTRACT FROM AUTHOR]

Misdiagnosis may result from biopsy site selection, technique, or choice of transport media. Important potential sources of error include false-negative direct immunofluorescence results based on poor site selection, uninformative biopsy specimens based on both site selection and technique, and spurious interpretations of pigmented lesions and nonmelanoma skin cancer based on biopsy technique. Part I of this 2-part continuing medical education article addresses common pitfalls involving site selection and biopsy technique in the diagnosis of bullous diseases, vasculitis, panniculitis, connective tissue diseases, drug eruptions, graft-versus-host disease, staphylococcal scalded skin syndrome, hair disorders, and neoplastic disorders. Understanding these potential pitfalls can result in improved diagnostic yield and patient outcomes. [ABSTRACT FROM AUTHOR]

Junctional epidermolysis bullosa (JEB) is a rare genodermatosis characterized by a split in the lamina lucida usually because of mutations in LAMA3, LAMB3 and LAMC2 resulting in absence or reduction of laminin-332. Rare subtypes of JEB have mutations in COL17A1, ITGB4, ITGA6 and ITGA3 leading to reduction or dysfunction of collagen XVII, integrin α6β4 and integrin α3. The classic finding under light microscopy is a paucicellular, subepidermal split. We describe the unusual presence of an eosinophilic infiltrate in the bullae and subjacent dermis in a neonate with JEB, generalized intermediate (formerly known as non-Herlitz-type JEB), discuss the histologic differential diagnosis for a subepidermal blister in a neonate, review the literature regarding cases of epidermolysis bullosa (EB) presenting with inflammatory infiltrates, and discuss mechanisms to explain these findings. This case highlights that eosinophils can rarely be seen in EB and should not mislead the dermatopathologist into diagnosing an autoimmune blistering disorder. [ABSTRACT FROM AUTHOR]

Background Bullous pemphigoid (BP) has been associated with neurologic disorders. Objective We sought to analyze the association between BP and neurologic disorders. Methods We retrospectively identified residents of Olmsted County, Minnesota, with a first lifetime diagnosis of BP between January 1, 1960, and December 31, 2009. Three age- and sex-matched Olmsted County, Minnesota, residents without BP were selected as control subjects for each patient. We compared history of or development of neurologic disorders (dementia, Alzheimer disease, Parkinson disease, multiple sclerosis, cerebrovascular disease, and seizures) between groups using case-control and cohort designs. Results In all, 87 patients with BP were identified and matched to 261 control subjects. The odds of a previous diagnosis of any neurologic disorder or a history of dementia were significantly increased among cases compared with controls (odds ratio 6.85; 95% confidence interval [CI] 3.00-15.64; P < .001; and odds ratio 6.75; 95% CI 2.08-21.92; P = .002, respectively). Both Parkinson disease (hazard ratio 8.56; 95% CI 1.55-47.25; P = .01) and any type of neurologic disorder (hazard ratio 2.02; 95% CI 1.17-3.49; P = .01) were significantly more likely to develop during follow-up in patients with than without BP. Limitations Small geographic area and retrospective study design are limitations. Conclusion Findings confirmed an association of BP with neurologic disorders, especially dementia and Parkinson disease. [ABSTRACT FROM AUTHOR]

Background The twin goals of long-term disease control and minimizing toxicities related to immunosuppression necessitate efforts to find effective steroid-sparing agents in the management of patients with autoimmune bullous diseases. Pemphigus especially requires a long view, because the disease can persist throughout a patient's lifetime, yet few clinical trial reports exist to guide the practitioner. Objectives We review the response of pemphigus patients to tetracycline, doxycycline, or minocycline plus niacinamide (TCN/NAM) as steroid-sparing therapy and to determine the effects of TCN/NAM on autoantibody levels during the long-term treatment of pemphigus. Methods This was a retrospective chart review in a private medical dermatology practice setting of all pemphigus patients treated between 1993 and 2013. Clinical responses to TCN/NAM therapy after initial high-dose steroid induction therapy and pemphigus antibody levels were recorded over the course of disease flares and treatment cycles along with any related side effects. Anti-desmoglein 1 and 3 titers were compared in a subset of patients over time, and a statistical analysis was performed to correlate the clinical response with antibody levels. Results Fifty-one pemphigus patients (43 with pemphigus vulgaris, 7 with pemphigus foliaceous, and 1 with pemphigus erythematosus) received at least 3 months of TCN/NAM, and 16 patients with pemphigus vulgaris had 1 set of pemphigus antibody titers correlating to a baseline/flare and clinical remission. TCN/NAM was associated with disease control in 43 of 51 patients, with a duration of response ranging from 1 to 13 years (mean, 3.14 ± 2.97 years). Thirteen of 51 patients needed no additional treatment for complete disease control, while 33 of 51 needed intermittent topical clobetasol or short courses of oral steroids for long-term management. There were 5 nonresponders. Antidesmoglein titers trended lower in TCN/NAM responders, but only desmoglein 3 approached statistical significance (anti-desmoglein 1, P = .21; anti-desmoglein 3, P = .02). Limitations This is a retrospective analysis from a single practice. A lack of serial autoantibody titers limited statistical analyses. Conclusion TCN/NAM may be useful as a steroid-sparing therapy for pemphigus. [ABSTRACT FROM AUTHOR]

Autoimmune bullous diseases are a group of skin disorders resulting from an autoimmune reaction against intercellular adhesion molecules or components of the basement membrane of skin and mucosa. Autoimmune disorders often occur in patients with a history of another autoimmune disease and most autoimmune diseases have a striking female predominance. In this review, we aim to analyze the different associations of autoimmune bullous diseases with other autoimmune diseases and highlight the distinctiveness of the female gender in these associations., Competing Interests: None., (Copyright © 2022 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of Women’s Dermatologic Society.)

O pênfigo foliáceo endêmico (PFE) é uma afecção onde fenômenos auto-imunes são freqüentemente relacionados com sua patogênese. Abordaram-se neste estudo, alguns testes laboratoriais freqüentemente alterados em outras doenças auto-imunes. Foram estudados em 20 pacientes com PFE a presença ou alterações de fator antinúcleo (FAN), fator reumatóide (FR), proteína C-reativa (PCR), velocidade de hemossedimentação (VHS), eletroforese de proteínas e o número totalde leucócitos. Encontrou-se PCR positiva em mais de 65 % dos casos, leucocitose moderada na maioria dos pacientes, VHS aumentada e alterações discretas da análise de proteínas do sangue. O FAN e o FR apresentaram-se negativos em todos os casos. Embora estes exames sejam considerados inespecíficos, o seu estudo associado ao quadro clínico pode colaborar no acompanhamento do PFE.Endemic pemphigus foliaceus (EPF) has its pathogenesis frequently associated to autoimmune phenomena. In this paper, afew routine laboratory tests, usually disturbed in some autoimmune diseases, were taken in 20 patients with EPF, which were screened for antinuclear antibodies (ANA), rheumatoid factor (RF), C-reactive protein (CRP) and changes of eritrocyts sedimentation rate (ESR), serologic proteins electrophoresis and total leucocyte count. The CRP was found in 60% of cases, leucocitosis in 85%, high ESR in all of them and mild alterations in serologic proteins analysis. No ANA or RF was found. Although widely accepted as inespeciflc tests, we believe that an association of the laboratory routine tests with clinicalfindings, can prove to be helpful in the follow up care of these patients.

Immune-mediated inflammatory skin diseases are characterized by a complex multifactorial etiology, in which genetic and environmental factors interact both in genesis and development of the disease. Nutrition is a complex and fascinating scenario, whose pivotal role in induction, exacerbation, or amelioration of several human diseases has already been well documented. However, owing to the complexity of immune-mediated skin disease clinical course and breadth and variability of human nutrition, their correlation still remains an open debate in literature. It is therefore important for dermatologists to be aware about the scientific basis linking nutrition to inflammatory skin diseases such as psoriasis, atopic dermatitis, hidradenitis suppurativa, bullous diseases, vitiligo, and alopecia areata, and whether changes in diet can influence the clinical course of these diseases. The purpose of this narrative review is to address the role of nutrition in immune-mediated inflammatory skin diseases, in light of the most recent and validate knowledge on this topic. Moreover, whether specific dietary modifications could provide meaningful implementation in planning a therapeutic strategy for patients is evaluated, in accordance with regenerative medicine precepts, a healing-oriented medicine that considers the whole person, including all aspects of the lifestyle. [ABSTRACT FROM AUTHOR]

Background and Aim: Pemphigus is one of the most common types of autoimmune blistering disease that requires systemic immunosuppressive therapy. Immunosuppressive therapy has improved the disease outcome in recent decades, but long-term use of them has side effects. Recently, it has been tried to evaluate immunosuppressive drugs with less side effects. One of them is mycophenolate mofetil. The aim of this study was to evaluate of therapeutic efficacy of this drug in the patients with refractory pemphigus vulgaris. Methods: Three hundred files of patients with pemphigus vulgaris were reviewed and among them 28 patients who had received mycophenolate mofetil due to resistance to treatment were entered to this study. The data were obtained from patients' files and were analyzed using Kendall's tau-c correlation, Mann-Whitney and ANOVA tests. Results: Male to female ratio was 1.8:1 with an average age of 43.3±13.6 year. Twenty-eight cases of pemphigus vulgaris were treated receiving 2gr/day mycophenolate mofetil + 1mg/kg/day prednisolone. Remission occurred after 3 months. Seventeen patients (10 men and 7 women) had complete remission. There was no significant association between gender and treatment response (P=0.58). There was no significant association between mean age of treated patients and untreated patients with treatment response (P=0.77). Also, there was no significant association between severity of mucosal (P=0.80) and severity of skin involvement with treatment response (P=0.80). Ten patients who received mycophenolate mofetil more than 12 months had treatment response to mycophenolate mofetil and they did not have any relapse in the follow up period. Conclusion: Mycophenolate mofetil is effective and safe as an adjuvant therapy in patients with pemphigus vulgaris especially in refractory pemphigus. Initiation of the therapeutic effect is slow. [ABSTRACT FROM AUTHOR]

Abstract: Cytokines are key regulators of physiological inflammatory responses, while aberrant cytokine expression contributes to pathogenesis of autoimmune diseases. We noted increased IL-6 levels in human and murine epidermolysis bullosa acquisita (EBA), a prototypic organ-specific autoimmune bullous dermatoses (AIBD) induced by autoantibodies to type VII collagen (COL7). In contrast to rheumatoid arthritis, blockade of IL-6 led to strikingly enhanced experimental EBA, while treatment with recombinant IL-6 was protective. This was due to classical IL-6 signalling and independent of IL-6 trans-signalling, as treatment of mice with sgp130Fc had no impact on EBA manifestation. Induction of EBA in mice led to increased IL-1ra levels in skin and serum, while blockade of IL-6 completely inhibited IL-1ra expression induced by autoantibodies to COL7. In line, treatment of mice with EBA with recombinant IL-6 induced IL-1ra concentrations exceeding those of untreated animals with EBA, and IL-1ra (anakinra) administration significantly impaired experimental EBA induction. We here identified a novel anti-inflammatory pathway in an organ-specific autoimmune disease. Modulation of this IL-1ra pathway by classical IL-6 signalling demonstrates anti-inflammatory and protective activities of IL-6 in vivo. [Copyright &y& Elsevier]

Epidermolysis bullosa (EB), refers to a group of inherited bullous disorders, characterized by fagility of the skin and mucous membranes, and blister formation in response to minor friction or trauma. Skin fragility and bulla formation of EB result from genetic mutations of any of dozen genes that encode structural proteins which normally reside within the epidermis, the dermo-epidermal juntion, or the upper dermis. There are four major type of inherited epidermolysis bullosa: EB simplex (EBS), junctional EB (JEB), dystrophic EB (DEB) and Kindler syndrome. [ABSTRACT FROM AUTHOR]

Pemphigus is an uncommon autoimmune mucocutaneous blistering disease, mediated by antibodies directed against proteins present on the surface of keratinocytes that provide mechanical structure to the epidermis. Incidences of the various forms of pemphigus vary from country to country. Pemphigus vulgaris consists of the 80% of pemphigus cases and it occurs 10 times more than pemphigus foliaceus. There have been several recent advances in the management of pemphigus. Treatment of with systemic steroids and cytotoxic immunosuppressants can be highly effective. This article will discuss its epidemiology, pathogenesis, clinical variants and approaches to treatment. [ABSTRACT FROM AUTHOR]

Background: Increased risk of spontaneous pneumothorax has been described in patients with Marfan syndrome and has been attributed, in part, to the presence of apical blebs and bullae. Objectives: We assess the risk of pneumothorax and its relationship to the presence of apical blebs and bullae in patients with Marfan syndrome in the era of CT imaging. Methods: A retrospective cohort study was performed of all patients 13 years or older with Marfan syndrome evaluated at the Mayo Clinic, Rochester, Minn., USA, from 1998 through 2008. One hundred and sixty-six patients met the current diagnostic criteria for Marfan syndrome and had chest imaging studies available for review. Results: The median age was 40 years (range 14-71); 37% had a smoking history. Eight of 166 patients (4.8%) had experienced 1 or more episodes of spontaneous pneumothorax, and 2 of these 8 patients had 2 or more episodes. Apical blebs or bullae were identified on radiologic imaging in 16 patients (9.6%). Four of 16 (25%) patients with apical blebs or bullae had a history of spontaneous pneumothorax compared to 4 of 150 patients (2.7%) without blebs or bullae (p = 0.003). Conclusions: The frequency of blebs is relatively low in patients with Marfan syndrome but the risk of pneumothorax is significantly higher in those with radiologically detectable blebs or bullae. Chest CT scanning to identify blebs and bullae may allow risk stratification for pneumothorax in patients with Marfan syndrome. Copyright © 2011 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

The accurate diagnosis of autoimmune bullous diseases of the skin requires evaluation of clinical, histologic, and immunopathologic findings. There is generally a risk of serious side effects or complications in the treatment of autoimmune bullous diseases especially of pemphigus. Therefore definite diagnosis is essential for accurate and effective treatment. Fortunately, there are invaluable laboratory methods such as immunofluorescence tests in confirming a diagnosis that is suspected by clinical examination. In this article, the application techniques of these tests briefly and particularly the immunofluorescence findings that could be obtained in autoimmune bullous diseases were discussed. [ABSTRACT FROM AUTHOR]

Pemphigus vulgaris is a chronic blistering disease with autoimmune origin, related to genetic and environmental factors. It is secondary to the production of IgG auto-antibodies against the desmoglein 1 and 3 amino-terminal region that cause acantholysis and blistering. It can affect skin, mucous membranes and occasionally nail plate. It is characterized by the presence of flaccid blisters which are ruptured easily, crusting and erosions. The diagnosis is based on clinical, histopathological and direct or indirect inmunoflourescence. Recently, the autoantibodies determination by ELISA or immunoblot and immunoprecipitation are been used. The mainstay of treatment is oral corticosteroids, along with adjuvants such as azathioprine, cyclophosphamide, mycophenolate mofetil, rituximab, among others. [ABSTRACT FROM AUTHOR]

We report a patient with herpetiform pemphigus who was negative for autoantibodies to desmoglein (Dsg)1 or 3. He had erythemas with vesicles lining the margins on the trunk and extremities. Histopathology revealed intraepidermal blister with prominent eosinophil infiltration. Direct and indirect immunofluorescence demonstrated the presence of depositing and circulating immunoglobulin (Ig)G autoantibodies, but no IgA antibodies, to keratinocyte cell surface. Nonetheless, neither anti-Dsg1 nor Dsg3 antibodies were detected by enzyme-linked immunosorbent assay. Immunoblotting using human epidermal extracts also showed no reactivity with known intraepidermal or epidermal–dermal junctional substances. Immunoelectronmicroscopy revealed the reactivity on the portion of keratinocyte cell surface but not on the desmosomes. This case suggests that non-desmoglein antigen on keratinocyte cell surface can be targeted in some patients with this unusual variant of pemphigus. [ABSTRACT FROM AUTHOR]

Non-infectious ulcerative oral mucous membrane diseases are difficult to separate at first glance: they can appear as aphthous, bullous, lichenoid, drug-induced or toxic-irritative reactions. The overall considerations of history, localization of lesions, clinical and histological features, as well as direct and indirect immunofluorescence examination are required for the correct diagnosis. Some disorders start preferably at the oral mucosa, like pemphigus vulgaris and Adamantiades-Behçet disease, while others, such as cicatricial pemphigoid and habitual aphthosis generally are confined to the mucous membranes. This overview summarizes clinical and diagnostic features, differential diagnoses and current therapeutic possibilities of non-infectious inflammatory stom-atopathies, which possess a specific position among skin diseases in distinction to infectious or neoplastic oral ulcers. This group of diseases includes aphthous lesions, lichen planus mucosae, lupus erythematosus, disorders with intraepi-dermal or subepidermal formation of blisters including pemphigus, bullous pemphigoid, erythema multiforme and variants as well as allergic or toxic contact stomatitis. [ABSTRACT FROM AUTHOR]

Tacrolimus is the generic name for the macrolide immunosupressant previously known by its experimental name, FK506. This new name is derived from Tsukuba, the location of its discovery; macrolide, its chemical class; and immune suppressant, its primary activity in humans. Recent reports suggest that topical tacrolimus may be used for the treatment of mucosal diseases, including oral psoriasis, Crohn's disease, exfoliative cheilitis, oral lichen planus, graft versus host disease, paraneoplastic pemphigus, bullous pemphigoid and cicatricial pemphigoid. Topical tacrolimus treatment seems to be effective, but this must be proven by well designed randomized controlled studies. [ABSTRACT FROM AUTHOR]