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The place of pemphigus vulgaris (PV) among autoimmune bullous diseases (AIBD) is well established. It is an acquired chronic, autoimmune, vesiculobullous disease in which IgG antibodies target desmosomal proteins to produce intraepithelial mucocutaneous blistering. The diagnosis is often challenging for the clinicians. It requires a combination of three major features: clinical, histopathological, and immunological. Clinically, oral lesions are the first manifestations of the disease in 50-90% of the patients with widespread blisters affecting the oral mucosa. On the skin, lesions are characterized by flaccid blisters that rapidly progress into erosions and crust formation. Umbilical lesions as a clinical manifestation of PV are peculiar and have rarely been reported, and they are not yet completely elucidated. Umbilical region involvement in patients with pemphigus was assessed in a limited study totalling just 10 patients. This localisation may be a valuable hint easing the diagnosis at the clinical level for patients with oral mucosal blisters. Dentists must be familiar with the clinical manifestations of PV to make an early diagnosis and start an early treatment which determines the prognosis of the disease. To the best of the authors’ knowledge, the coexistence of these lesions with the oral lesions as a first sign of PV in the absence of skin involvement was reported in only one case of pemphigus vegetans (PVe). In this paper, we describe an observation of a female patient that was diagnosed with PV that begun with simultaneous oral and umbilical locations which coexisted for a period of 4 months before the appearance of other cutaneous lesions. We highlight the role of dentists, by being familiar with the clinical manifestations of PV, to make an early diagnosis to start an early treatment which determines the prognosis of the disease and to follow closely the evolution of lesions to change treatment if required. We also discuss the clinical, histological, and immunological features of the disease that enabled the differential diagnosis as well as the appropriate therapeutic management.

Background and Aims: Pemphigus vulgaris is a rare autoimmune intraepidermal vesiculobullous disease involving the skin and mucosa. It impacts the quality of life of both patients and their families. Methods: A total of 70 patients with pemphigus vulgaris (either outpatient or hospitalized) were enrolled using the simple sampling method between 2016 and 2017 from the dermatology clinic at Faghihi Hospital, Shiraz, Iran. A validated Persian version of the Family Dermatology Life Quality Index (FLDQI) questionnaire was filled by a family caregiver. The questionnaire contained 10 items assessing the quality of life of the family. Demographic variables were recorded in a separate form. Results: The mean age of the patients was 51 ± 11.3 years and that of the family caregivers was 32 ± 8.8 years. The FLDQI score was higher (poorer quality of life) if the patient was male, older, had shorter disease duration or had fewer disease recurrences (P = 0.046, 0.01, 0.001 and >0.001, respectively). Higher scores were also obtained in the less-educated caregivers (P = 0.026) but there was no association with either gender or age (P = 0.399, 0.1). Conclusion: Pemphigus vulgaris significantly affects the Family Dermatology Life Quality Index. Education and counseling of family caregivers by various support groups such as Pemphigus Family Associations could be effective in improving the quality of life of the caregivers. Limitations: This study did not assess the effect of comprising domain analysis, severity of disease, patients’ Dermatology Life Quality Index (DLQI), mucosal involvement, response to treatment, outpatient or admitted status, socioeconomic status, or the quality of life among the various family members.

Drug-induced pemphigoid and drug-induced linear IgA disease can be difficult to differentiate from bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), and linear IgA disease (LAD) because of only minor differences in clinical, histopathologic, and immunopathologic features. However, differentiation can be of major importance because of a different approach, treatment, and outcome. Diagnosis is mainly based on the time relation between start of the suspected drug(s) and onset of the lesions. Drug-induced BP and drug-induced LAD tend to be self-limiting after withdrawal of the culprit drug.

Drug-induced pemphigus can be induced or triggered by drugs, most often penicillamine and other thiol-associated drugs. The time from first intake of the drug to onset of the reaction is often relatively long compared to other cutaneous adverse drug reactions. Clinically, it can present as pemphigus foliaceus (most often), pemphigus erythematosus, pemphigus herpetiformis, or pemphigus vulgaris. Opposing to idiopathic pemphigus, pruritus, a prodromal stage, and absence of mucosal involvement are frequent, while laboratory findings are similar. The pathogenesis is not completely known, but probably includes endogenous and exogenous factors. Withdrawal of the culprit drug is mandatory, although the disease may not subside if caused by nonthiol drugs.

Vesiculobullous lesions in systemic lupus erythematosus (SLE) are a rare cutaneous manifestation of cutaneous and/or systemic LE with variable presentation. The diagnosis of SLE‐associated vesiculobullous diseases remains challenging, due to the poorly defined similarities and nosology in immunohistopathological and clinical and features.

Linear IgA bullous dermatosis is an acquired subepidermal immunoglobulin-mediated vesiculobullous disease. In this retrospective, observational, descriptive study, we describe the clinical characteristics, treatments, and outcomes of 17 patients with linear IgA bullous dermatosis. Two children had been vaccinated 2 weeks before the onset of symptoms, 2 had had bronco-obstructive respiratory symptoms, and 1 had received intravenous antibiotic therapy. We also observed an association with autoimmune hepatitis in one patient and alopecia areata in another. One boy had VACTERL association. Diagnosis was confirmed by histopathology and direct immunofluorescence. Sixteen patients were treated with dapsone, which was combined with oral corticosteroids in 8 cases and topical corticosteroids in two. Of note in this series was the occurrence of relapses in the perioral area coinciding with infections and vaccination, and the association between linear IgA bullous dermatosis and autoimmune hepatitis and VACTERL association.

Vesiculobullous lesions in lupus erythematosus (LE) are a rare cutaneous manifestation of cutaneous and/or systemic LE with variable presentation. While the minor forms of LE-associated vesiculobullous disease may cause disfigurement and discomfort, the severe forms can present with hyperacute reaction and life-threatening consequences. Specific LE and aspecific cutaneous LE are defined by the presence or absence of interface change on histopathology that can be applied to vesiculobullous diseases in relation to LE. However, the diagnosis of LE-associated vesiculobullous diseases remains difficult, due to the poorly defined nosology and the similarities in clinical and immunohistopathological features among them. Herein, we thoroughly review the topic of vesiculobullous skin disorders that can be encountered in LE patients and organize them into four groups: LE-specific and aspecific vesiculobullous diseases, LE-related autoimmune bullous diseases, and LE in association to non-autoimmune conditions. We sought to provide an updated overview highlighting the pathogenesis, clinical, histological, and immunopathological features, laboratory findings, and treatments and prognosis among vesiculobullous conditions in LE.

Visphota kushta (blistering skin disease) is characterized by transparent blisters with thin skin covering. Management of Visphota Kushta in Ayurveda is rarely reported. The case reported here showed significant regression in the condition in short span of time and could completely stop the use of anti histamines and corticosteroids. A 32 year old female, presented with complaints of blisters over both upper and lower extremities associated with edema, burning sensation, pain, severe itching and oozing since three months. The treatments were given after ascertaining the involved dosha and the samprapti (pathogenesis). The involved dosha were and Pitta (metabolic factor) and Kapha (binding factor) dosha. Pitta - kapha dosha hara line of treatment was adopted in terms of mitigating and purificatory therapy. It helped in arresting the progression of the condition and a complete healing of blisters. Photographs were taken during and after the treatment for records. The blister completely resolved and the skin was normal as before. The patient was back to her normal routine with no signs of relapse. The outcome was a combined effect of both shamana and shodhana chikitsa along with pathya sevana. Keywords: Vesiculobullous disease, Ayurveda, Panchakarma, Kushta, Shodhana

Background Linear IgA bullous dermatosis (LABD) is a rare autoimmune subepithelial vesiculobullous disease due to IgA autoantibodies directed against different antigens of the basement membrane zone (BMZ) of the skin and/or mucosae. It affects mainly preschool-aged children and adults, with only few studies on large series. The aim of this study was to assess possible differences between adults and children regarding clinical presentation, immunopathologic features, management and course of the disease. Methods A retrospective review of 38 LABD patients, followed-up from November 2006 to September 2018, was performed. Results Of 38 patients, 27 were adults and 11 children. Mean age at diagnosis was 5.4 years and 60.6 years in the pediatric and adult group, respectively. Considering both groups, limbs were the most commonly involved site (73.7%), followed by trunk (55.3%), head (36.8%) and buttocks (13.2%). Interestingly, head (p = 0.008), particularly perioral (p = 0.001), involvement, as well as “string of pearls” arrangement (p = 0.03), were more prevalent in children. Mucosal involvement was seen in 9 (23.7%) patients and was more frequent in children than adults (45.5% vs 14.8%, respectively, p = 0.09). Linear IgA deposits along the BMZ were observed in 30 patients (78.9%), while linear/granular IgA deposits in 8 patients (21.1%). Dapsone was the most commonly used drug (78.9%) and complete remission was achieved in most cases (81.6%). Conclusions Our epidemiological and clinicopathological findings relative to a large cohort of LABD patients are mostly consistent with the literature data. Interestingly, head, notably perioral, involvement and “string of pearls” arrangement occurred more frequently in the paediatric than adult group. The above clinical parameters may be regarded as diagnostic tools for LABD in children. Electronic supplementary material The online version of this article (10.1186/s13023-019-1089-2) contains supplementary material, which is available to authorized users.

Linear immunoglobulin A disease (LAD) is a rare, autoimmune, vesicular-bullous disease that is either idiopathic or drug-induced, most commonly by vancomycin and in rare instances by amlodipine. In drug-induced LAD, certain uncommon and atypical clinical features can occur. In our patient, a 49-year-old woman with amlodipine-induced LAD, atypical features such as koebnerization and palmo-plantar involvement occurred. She presented with tense, clear fluid-filled vesicles, bullae, and erosions all over her body, especially on the palms and soles, with some lesions showing a string-of-pearls appearance. The lesions were preceded by pruritus, and the patient had changed her anti-hypertensive medication from telmisartan to telmisartan-amlodipine for previous 10 days. Skin biopsy and direct immunofluorescence testing confirmed LAD. During the hospital stay, along with new crops of lesions, a few vesicles were present along the lines where she had scratched and the band of tight elastic sleeves of the sterile gown she wore, which is suggestive of koebnerization. Knowing the atypical manifestations of drug-induced LAD may aid clinicians in determining an early diagnosis, and LAD should be an important consideration in the differential diagnosis of vesiculobullous disease with palmar-plantar involvement. Amlodipine is a commonly used anti-hypertensive drug, so knowledge of its potential to cause this disease is important. Furthermore, knowing the potential for koebnerization, avoidance of trauma and the gentle handling of these patients can lead to early recovery from this self-limiting disease. Keywords: Amlodipide, linear IgA disease, drug-induced eruption, vesiculo-bullous disorders

Pemphigus is a chronic autoimmune vesiculobullous disease caused by autoantibodies directed against desmogleins (Dsgs). Pemphigus vegetans is a rare variant of pemphigus vulgaris, characterized by vegetative plaques, primarily occurring in flexures. It is a less common disease and involves the skin and mucosa due to disintegration of cellular adherence (acantholysis), resulting in intradermal split. We report this case of pemphigus vegetans of Neumann type for its rare presentation. We report a case of pemphigus vegetans, involving lips in a middle-aged female and sparing flexural areas. This patient was diagnosed on the basis of histopathology and treated successfully with intralesional steroids, resulting in the resolution of her plaques.

Mucous membrane pemphigoid (MMP) is a chronic, autoimmune, subepithelial vesiculobullous disease that very frequently affects the mucous membranes and less often the skin. Oral cavity is the most commonly affected site and desquamative gingivitis (DG) is the most common manifestation. This is the main reason why dentists play a vital role in the diagnosis and managing the oral health of patients. Treatment is usually challenging, however, it only can achieve temporary symptomatic effect. We report a case of desquamative gingivi-tis, manifestation of MMP that was treated successfully with topical corticosteroid. The latter was applied by using of individual made custom trays to improve clinical efficacy.&nbsp

Objective: Aims of this study were to test the efficacy of anti-BP180-NC160 ELISA in the diagnosis of oral pemphigoid compared to the gold standard, represented by direct immunofluorescence and pathological examination, to correlate the antibody titers with the severity of the disease and the demographical data. Materials and Methods: Patients with a suspect of oral pemphigoid were enrolled and underwent biopsy and sera collection both, in order to perform histopathological examination, direct immunofluorescence and ELISA. The test outcomes were compared, and ELISA sensitivity, specificity, accuracy, and negative and positive predictive values were calculated. Results: ELISA showed good specificity (83.3%), while sensitivity was only 50%. A moderate correlation between antibody titers and disease severity was recorded. Conclusions: Mucomembranous Pemphigoid is an autoimmune autoantibody-mediated blistering disease, often affecting exclusively the oral mucosa. Currently, the biopsy is required to diagnose this disease, but serological tests are also commonly employed during clinical practice as adjunctive tools. BP180-NC160 ELISA should be considered an ancillary diagnostic test in course of oral pemphigoid; direct immunofluorescence+histologic examination remains the diagnostic gold standard.

Pemphigus is an autoimmune disease affecting the skin and mucosae. Oral lesions are common and sometimes are the only manifestations of the disease. The clinical presentations of pemphigus might mimic other vesiculobullous lesions of the oral cavity. We report a case of a 60-year-old male patient who complained of erosive lesions in the oral cavity. The lesions were diagnosed as pemphigus based on histopathological examination and immunofluorescence findings. The patient did not respond to topical steroids and low doses of systemic steroids. Since the patient began developing dermatological manifestations, he was administered pulse steroid therapy. He is currently under observation and his clinical signs and symptoms show improvement, although he has developed hyperglycemia as a complication.

Key clinical message We report an uncommon presentation of bullous dermatosis by linear IgA. There are few cases reported in the literature with this form of presentation starting with mucosal lesions and then evolving into a similar bullous pemphigoid pattern. In addition, we emphasize the importance of direct immunofluorescence for the definitive diagnosis.

Objectives Lichenoid granulomatous reaction can be subcategorized into lichenoid granulomatous dermatitis or lichenoid granulomatous stomatitis. It is described in the literature as demonstrating a band-like lichenoid infiltration with clusters of histiocytic or granuloma formation. In this study, we presented a large case series of lichenoid granulomatous stomatitis including demographics, histological features, and subcategories and compared findings with current literature. Methods and materials We retrieved all cases diagnosed with lichenoid reaction with granulomatous inflammation between January 1, 2000, and August 1, 2016, from the University of Florida Oral Pathology Biopsy Service Archives. Results A total of 47 patients with tissue from 50 biopsy sites were included. The majority of the cases were noted in females (64%) with a mean age of 59 years (range 30-88). Most patients were Caucasian, and the most common sites in descending order were: gingiva, buccal mucosa, vestibule, tongue, lip, palate, and occasional multifocal involvement. Clinical impressions by the treating clinicians included leukoplakia, dysplasia, carcinoma in situ, squamous cell carcinoma, lichen planus, vesiculobullous disease, trauma, and allergy. The histologic features of these cases ranged from lichenoid mucositis with numerous aggregates of histiocytes to well-formed granulomas, in accordance with previous classifications of similar dermatologic lesions. All cases revealed positive CD 68 in the histiocytic infiltrates and were negative for microorganisms utilizing acid-fast bacilli, Grocott methenamine-silver, and periodic acid-Schiff stains. Conclusion Lichenoid granulomatous disease may be more common than previously reported; however, its etiology remains unknown and patients should be kept under long-term clinical follow-up.

Bullous pemphigoid (BP) is a chronic, autoimmune vesiculobullous disease that frequently occurs in the elderly population. Previous epidemiological studies have suggested an association between BP and neurological diseases; some studies, however, showed conflicting results. This study aimed to investigate if patients with BP have significantly higher risks for neurological disorders, compared to controls. A comprehensive search was performed using MEDLINE, EMBASE and Cochrane library databases. Case-control and cohort studies that assessed the relationship between BP and neurological diseases were included. DerSimonian and Laird random-effects models were utilized to calculate the pooled relative risks (RRs). Publication bias was evaluated qualitatively by constructing a funnel plot and quantitatively by conducting Egger's test. Fourteen studies, with 23 369 BP cases and 128 697 controls were included in this meta-analysis. Patients with BP were significantly more likely to have stroke (RR 2.68, 95% CI: 2.07-3.46), Parkinson's disease (PD; RR 3.42, 95% CI: 3.01-3.87), dementia (RR 4.46, 95% CI: 3.23-6.16), epilepsy (RR 2.98, 95% CI: 1.42-6.28), multiple sclerosis (RR 12.40, 95% CI: 6.64-23.17) and any aforementioned neurological disease (RR 4.93, 95% CI: 3.62-6.70), compared to controls. Moderate to high heterogeneity were observed for analyses of most neurological diseases, except for PD and multiple sclerosis. This study provided support for a significant association between BP and neurological diseases. Clinicians should be aware of this association and manage modifiable risk factors for neurological diseases accordingly.

Oral benign pemphigoid of mucous membranes (OBPMM) is a chronic and autoimmune mucocutaneous inflammatory vesiculobullous disease, which forms subepithelially leaving extensive superficial ulceration. We report the case of a 41-year-old female, feoderma, rural worker, diabetic, which arrived at the stomatology center with chief complaint of a wound in her mouth during 8 years. There were no skin or ophthalmologic alterations. Intraoral examination showed a single reddish erosion with ulcerated surface in the middle on the right side buccal mucosa, with white borders without elevation, definite borders, oval-shaped, non-infiltrative, stimulus painful. The diagnosis of the injuries was obtained through biopsy and histopathology, and the established treatment was topic corticosteroid, patient was also informed about her condition regarding on morbidities related to OBPMM. This case illustrates an exclusive OBPMM and the importance of a correct patient follow-up.

Drug-induced hypersensitivity immune reactions are exaggerated immunoinflammatory responses to allergenic components of the medications that occur in genetically susceptible subjects. The type of hypersensitivity immune response generated, whether antibody mediated or T cell mediated, or an immune complex reaction is determined by multiple factors, including the molecular characteristics of the allergen, the route of administration of the medication, the manner of presentation of the allergen by antigen-presenting cells to naïve T cells, the repertoire of the T cell receptors, and the cytokine profile within the microenvironment. This review deals with the clinical and histopathological aspects of adverse immunologically mediated oral mucosal reactions to systemic medication. We elaborate on diseases showing features of lichenoid tissue reaction/interface dermatitis-stomatitis, autoimmune vesiculobullous oral lesions, and immunoglobulin E- (IgE-) and immune complex-mediated oral reactions to drugs.

Background: Direct immunofluorescence (DIF) test for tissue-bound autoantibodies, has been found to be of value in the diagnosis of several dermatological disorders. The location and pattern of deposition of immunoreactants helps in classifying various immune-mediated diseases. Aims and Objectives: The aim of this study was to analyze the concordance between the clinical, histopathological and DIF diagnosis in bullous and nonbullous lesions of the skin, and thus determine the impact of immunofluorescence on diagnosis. Materials and Methods: A total of 215 skin biopsies performed in suspected immune-mediated vesiculobullous disease, vasculitis or dermatosis, were studied. Histopathological examination was done along with DIF study for deposits of immunoglobulin G(IgG), IgA, IgM, and C3. Results: Direct immunofluorescence was positive in 103/215 cases. There was very good concordance between the clinical, histological and DIF results (observed agreement = 93.4%, κ =0.90, with 95% confidence interval = 0.86–0.94). The overall sensitivity of DIF in immune-mediated skin disorders was 98.0%. DIF was positive in 52/53 cases (98.1%) in the pemphigus group and 24/25 (96.0%) bullous pemphigoid cases. None of the clinically suspected cases of dermatitis herpetiformis showed DIF positivity. A positive lupus band test was seen in 9/9 (100%) cases of lupus erythematosus. DIF was positive in 10/10 (100%) clinically suspected cases of Henoch–Schönlein purpura. In 110 cases, negative DIF results helped to rule out immune-mediated vesiculobullous disorders, lupus erythematosus and vasculitis, and the final diagnosis was made on the basis of the clinical features and/or histopathology. Conclusion: Direct immunofluorescence is a useful supplement for the accurate diagnosis of immune-mediated dermatological disorders, and helps to classify various autoimmune bullous disorders. When the clinical features/histopathology are inconclusive, the diagnosis often can be made on the basis of the DIF findings alone. A combination of the clinical features, histopathology and DIF usually gives the best results.

Bullous systemic lupus erythematosus (BSLE) is an autoantibody-mediated vesiculobullous disease in patients with SLE. Autoimmunity in BSLE is characterized by the presence of circulating anti-type VII collagen antibodies. BSLE patients often present with multiple, tense, clear fluid-filled vesicles and bullae overlying erythematous edematous plaques. Skin biopsy from BSLE patients shows subepidermal bullae with numerous neutrophils and only occasional eosinophils. Furthermore, immunofluorescence examination showed linear deposition of lgG, lgA, C3, and C1q along the basement membrane zone. BSLE patients with corticosteroids treatment constantly do not receive a marked improvement, while dapsone generally dramatically improved the skin condition. Recently, it has been reported that quite a few cases of BSLE were successfully treated with other immune suppressive drugs. Therefore, a comprehensive review of the treatment of BSLE would be beneficial to cure the disease.

Childhood linear immunoglobulin A dermatosis is a rare autoimmune vesiculobullous disease. It results in linear deposition of autoantibodies (immunoglobulin A) against antigens in the basal membrane zone, leading to subepidermal cleavage. Additional depositions of immunoglobulin G and complement-3 might occur. It is still debated whether concomitant findings of immunoglobulins A and G should be considered a subtype of this dermatosis or a new entity. Further studies are needed to recognize this clinical variant.

Linear IgA bullous dermatosis is a rare autoimmune vesiculobullous disease characterized by linear deposition of IgA along the basement membrane zone. It is classically idiopathic, but may also arise secondary to drug exposure. A heterogeneous spectrum of clinical features has been described, including a rare, morbid variant mimicking toxic epidermal necrolysis. Herein, we present a case of vancomycin-induced linear IgA bullous dermatosis that manifested clinically as toxic epidermal necrolysis and resolved with dapsone therapy.

Linear IgA bullous dermatosis (LABD) is an autoimmune vesiculobullous disease, which is typically idiopathic but can also rarely be caused by medications or infections. Vancomycin is the most common drug associated with LABD. Lesions typically appear 24 hours to 15 days after the first dose of vancomycin. It is best characterized pathologically by subepidermal bulla (blister) formation with linear IgA deposition at the dermoepidermal junction. Here we report an 86-year-old male with a history of left knee osteoarthritis who underwent a left knee arthroplasty and subsequently developed a prosthetic joint infection. This infection was treated with intravenous vancomycin as well as placement of a vancomycin impregnated joint spacer. Five days following initiation of antibiotic therapy, he presented with a vesiculobullous eruption on an erythematous base over his trunk, extremities, and oral mucosa. The eruption resolved completely when intravenous vancomycin was discontinued and colchicine treatment was begun. Curiously, complete resolution occurred despite the presence of the vancomycin containing joint spacer. The diagnosis of vancomycin-induced linear IgA bullous dermatosis was made based on characteristic clinical and histopathologic presentations.

Background: Pemphigus vulgaris is an autoimmune vesiculobullous disease characterized by vesicles and or bullae affecting skin and mucosa. Oral lesions are the first manifestation of the disease in majority of cases according to literature. This study is conducted to know the prevalence of oral lesions in pemphigus vulgaris patients at the time of presentation.Methods: This was a retrospective study. Departmental records were evaluated from January 2013 to January 2018. All the demographic data of pemphigus vulgaris patients at the time of first presentation, duration of disease, were assessed and tabulated. The clinical data of these patients during their subsequent visits to clinic were assessed for development of new oral lesions during course of disease.Results: Total number of patients were 55. The mean age was 42.5 years. 12 (21.8%) were males and 4 (78.1%) were females. Out of 55 patients 37 (67.27%) cases had oral involvement and 18 (32.72%) cases did not have oral lesions at the time of presentation. Out of 37 patients who had oral lesions, 34 (91.89%) patients had both oral and skin lesions and 3 (08.10%) patients had only oral lesions at the time of presentation. Out of the18 patients with no oral lesions, 3 (16.66%) patients developed oral lesions within 3 months. Total number of patients with oral lesions out of 55 were 40 (72.7%).Conclusions: Our study shows that oral lesions as initial manifestation of pemphigus vulgaris is 67.2%.

Objectives Chronic ulcerative stomatitis (CUS) is a rare disease of unknown etiology. The histopathologic features are similar to lichen planus, but direct immunofluorescence (DIF) studies show characteristic presence of IgG in basal and parabasal epithelial nuclei.This study will review a case series of CUS and assess if the entity is more similar to lichen planus or vesiculobullous diseases. Methods An IRB-approved retrospective search of CUS was performed within the archives of the UF Oral Pathology Biopsy Service between 2007 and 2017. Findings Seventeen cases, all female, were included. The median age was 64 years (range 47-83 years). Eleven patients were Caucasian, one was Asian, and one was African-American. Race was not specified in four cases. Buccal mucosa (8/17) was the most common location, followed by gingiva (7/17), buccal vestibule (1/17), and gingiva/buccal mucosa (1/17). The most common clinical presentations were pain/burning (13/17), erythema (13/17), whiteness (11/17), ulcerations/erosions (5/17), blisters/positive Nikolsky's sign (5/17), sloughing (2/17), striae (2/17), and recession (1/17). The clinical impression was lichen planus in 12 cases. Of these twelve cases, 4 included vesiculobullous disease as a differential. Four cases did not include a clinical impression and one listed erythema multiforme as the clinical impression. All cases were confirmed with DIF testing that showed a characteristic speckled pattern of IgG in basal and parabasal cells. Eleven of these cases were also positive for fibrinogen and two cases were faintly positive for C3. None of the cases were positive for IgA or IgM. Conclusion Since CUS has overlapping clinical, histological, and immunofluorescence features with lichen planus and vesiculobullous diseases, clinicians and pathologists should consider this unusual, but significant, entity whenever oral ulcerative diseases with mixed features are encountered.

Pemphigus vulgaris (PV) is a rare mucocutaneous vesiculobullous disease characterized by the development of autoantibodies against the desmosomal proteins. Current treatment is largely based on systemic immunosuppression using systemic corticosteroids. Immunosuppressive drugs used in the treatment of the disease may increase the risk of infection and delayed healing, which are of concern in dental treatment procedures in this group of patients. The clinical outcomes of implants in PV have not been investigated. We present a case of PV rehabilitated with an implant-supported prosthesis with a 32-month follow-up and discuss the important points in the surgical and prosthodontic phases.

Background Linear IgA bullous dermatosis (LABD) is an acquired autoimmune sub-epidermal vesiculobullous disease characterized by continuous linear IgA deposit on the basement membrane zone, as visualized on direct immunofluorescence microscopy. LABD can affect both adults and children. The disease is very uncommon, with a still unknown incidence in the South American population. Materials and methods All confirmed cases of LABD by histological and immunofluorescence in our hospital were studied. Results The confirmed cases were three females and two males, aged from 8 to 87 years. Precipitant events associated with LABD were drug consumption (non-steroid inflammatory agents in two cases) and ulcerative colitis (one case). Most of our patients were treated with dapsone, resulting in remission. Discussion Our series confirms the heterogeneous clinical features of this uncommon disease in concordance with a larger series of patients reported in the literature.

Background.Pemphigus vulgaris is an autoimmune vesiculobullous disease, affecting the skin and mucous membranes. It is reported to be associated with other autoimmune diseases including autoimmune thyroid diseases. However we report herein a case of pemphigus vulgaris associated with autonomous toxic nodule.Case Presentation.A 51-year-old woman was evaluated for blisters and erosions that develop on her trunk, face, and extremities, with a five-year history of progressively enlarging neck mass, and a past medical history of pemphigus vulgaris seven years ago. The condition was associated with palpitation, dyspnea, and heat intolerance. Thyroid function tests and thyroid scan were compatible with the diagnosis of thyrotoxicosis due to autonomous toxic nodule. Exacerbation of pemphigus vulgaris was proved by skin biopsy from the patient which revealed histologic picture of pemphigus vulgaris.Conclusion.Autoimmune thyroid diseases are reported to associate pemphigus vulgaris. To our knowledge, this case is the first in the English literature to report association between pemphigus vulgaris and autonomous toxic nodule and highlights the possibility of occurrence of pemphigus vulgaris with a nonautoimmune thyroid disease raising the question: is it just a coincidence or is there an explanation for the occurrence of both conditions together?