Autoimmune Vesiculobullous Disease Research Articles

Proposal of a modified technique of Nikolsky's sign in oral autoimmune vesiculobullous diseases.

Nikolsky's sign, originally described for skin lesions, presents challenges when applied to the oral mucosa. To address this, a modified Nikolsky's sign has been proposed specifically for the oral mucosa. In this variant, a gentle breath of air from the air syringe embedded in the dental unit is used to inflate a pre-existing collapsed blister (non-induced technique). Alternatively, in the induced technique, a healthy peri-lesion mucosal site is gently scratched with a blunt dental tool, and after a few minutes, air is blown on the same area to inflate any newly formed blister. The sign is considered positive if a blister is raised from the blown surface. The described modified Nikolsky's sign improves the visualization of oral vesicles and blisters in a cost-effective, easy, and minimally invasive manner. Its elicitation can aid in referring patients to specialized tertiary care units.

Direct Immunofluorescence in Cutaneous Vesiculobullous Lesions: A Cross-sectional Study in a Tertiary Care Center

Introduction: Cutaneous noninfectious autoimmune vesiculobullous diseases are a wide variety of lesion. They can show clinical and histopathological overlap, posing diagnostic challenges. Clinically, vesiculobullous lesions might not present with typical morphology and distribution in all cases. The specific histomorphological changes are observed only in an early and intact bulla. Immunofluorescence (IF) is a histochemical staining technique which detects antibodies in the tissue and body fluids. IF an adjuvant to histopathology is considered as gold standard in the diagnosis of vesiculobullous diseases. Methodology: A two-year retrospective cross-sectional descriptive study was conducted in the department of pathology, in a tertiary care center from January 2018 to December 2019. Paraffin embedded hematoxylin and eosin slides were retrieved from the archives and analyzed. Biopsies received in Michel’s medium were utilized for immunofluorescence study. Results: A total of 67 cases with Vesiculobullous lesions were analysed. The most common lesion diagnosed was Bullous Pemphigoid (BP) (29 cases -46.7%), followed by Pemphigus Vulgaris (PV) (19 cases- 30.6%). The highest number of cases were noted in the 7th decade, with a slight female preponderance. Sixteen out of 19 cases of pemphigus vulgaris and 28 out of 29 cases of bullous pemphigoid showed positive histomorphological correlation with IF. The PPV for pemphigus vulgaris was 100% and sensitivity was 84.2%, and in bullous pemphigoid, both the PPV and sensitivity was 96.5%. Higher concordance was noted between Histopathology and IF diagnosis in cases of BP in comparison to PV. Conclusion: Vesiculobullous lesions are heterogeneous in nature and can have clinical and histopathological overlap, posing diagnostic challenges. IF is considered gold standard and is necessary for the diagnosis in every case. A combined clinical, histopathological and IF examination is the best approach to accurate diagnosis. Keywords Bullous, Pemphigus, Vesiculobullous, Direct Immunofluorescence

Clinical Evaluation and Comparison between Effects of Rituximab and Prednisolone with Imuran to Neutrophils and Platelet Counts in Pemphigus Vulgaris Patients

Abstract Background: Pemphigus vulgaris (PV) is an autoimmune vesiculobullous mucocutaneous disease with life-threatening consequences. Rituximab (RTX) has recently emerged as an effective treatment for PV. Objectives: This study aims to determine changes in neutrophil and platelet counts for PV patients treated with RTX or corticosteroids combined with Imuran (azathioprine). Materials and Methods: The present cross-sectional study was conducted in the Department of Dermatology at Baghdad Teaching Hospital, Baghdad, Iraq. Thirty PV patients received two types of treatment: 15 patients were administered RTX and 15 patients took corticosteroids with Imuran (azathioprine). Neutrophil and platelet counts were detected at the hospital laboratory. Results: The age range of PV patients was 27–67 years, and the mean ±SD was 44.866 ± 10.979 years for the patient group treated with RTX and 44.133 ± 11.519 for the patient group treated with corticosteroids and Imuran. In patients treated with RTX, 26.6% were males and 73.33% were females, with a female-to-male ratio of 2.75:1. In patients treated with corticosteroids with Imuran, 40% were males and 60% were females, with a female-to-male ratio of 1.5:1. The results revealed that among patients treated with RTX, 30% of them had mucocutaneous lesions, 6.66% had oral lesions, and 13.33% had cutaneous lesions. Among the other groups treated with corticosteroids and Imuran, 23.33% had mucocutaneous lesions, 13.33% had oral lesions, and 13.33% had cutaneous lesions. Among the patients treated with RTX, 53.33% had normal range of neutrophil count, while 46.6% had neutrophil count above the NR, while in patients treated with corticosteroids and Imuran, 40% had a normal range and 60% had above the normal range. Conclusion: RTX was a more effective treatment compared to corticosteroids with Imuran because no patient remained in an active state during this study. PV was prevalent in females aged 40 years with mucocutaneous lesions.

Diagnostic Utility of Direct Immunofluorescence on Paraffin-Embedded Skin Biopsy Samples for the Diagnosis of Autoimmune Vesiculobullous Lesions.

Background Autoimmune vesiculobullous diseases (AIBDs) are a group of diseases characterized by blisters of the skin/mucosa due to the presence of circulating autoantibodies against antigens in the epidermis or the dermo-epidermal junction. Direct immunofluorescence (DIF) for immunoglobulin (Ig)G, IgC3, and IgA on fresh-frozen tissue is the gold standard diagnostic test for AIBDs. However, DIF in the absence of frozen tissue is challenging for the diagnosis of AIBDs. This study aimed to analyze the practical utility of DIF using paraffin-embedded skin biopsy rather than fresh frozen tissue for the diagnosis of AIBDs. Methodology This cross-sectional comparative study included 30 cases of AIBDs. DIF for IgG and IgA was performed on paraffin-embedded tissue (PE-DIF) after proteinase digestion on histopathologically confirmed 15 pemphigus vulgaris (PV), three pemphigus foliaceous (PF), four bullous pemphigoid (BP), three dermatitis herpetiformis (DH), three subcorneal pustular dermatosis (SCPD), and one case each of linear IgA disease and pemphigoid gestationis (PG). PE-DIF staining pattern was compared with the DIF on fresh frozen tissue (FF-DIF). Results All cases of PV and PF showed an intercellular IgG chicken wire staining pattern similar to FF-DIF. However, background staining was more intense in PV cases while less intense in PF cases. Three BP cases showed linear IgG staining in PE-DIF. DH, SCPD, linear IgA disease, and PG cases did not show IgG positivity. Out of three DH cases, two cases showed granular IgA positivity while linear IgA positivity along the basement membrane was seen in a single case of linear IgA disease. Negative IgG staining was observed in SCPD. Immunofluorescence in PE-DIF was rapidly deteriorating than in FF-DIF. Conclusions DIF done on paraffin-embedded tissue can be used as a supplement and salvage technique with histopathology for the diagnosis of AIBDs,particularly when a cryostat facility for frozen tissue is not available and the patient is unable to undergo a second biopsy procedure.

Use of IgG4 Immunohistochemistry as a Diagnostic Tool for Pemphigus

Pemphigus belongs to the group of antibody mediated autoimmune vesiculobullous disease (AIBD) of skin and mucous membranes. Antibodies are directed against the desmosomal adhesion proteins and IgG4 is the predominant subclass. It is a rare disease associated with significant mortality and morbidity. DIF is the gold standard test for AIBD. It requires fresh frozen section which is a major technical issue in most centres. The aim of this research was to study the diagnostic accuracy of IgG4 immunohistochemistry in pemphigus. The study was conducted as a diagnostic test evaluation in a tertiary care hospital. Paraffin blocks of histopathologically diagnosed AIBD were used for doing immunohistochemistry. The findings were compared with DIF findings. Statistical analysis was performed using SPSS, version 20.0. 48 paraffin blocks of AIBD were selected, DIF of all cases were already done from outside centre. There were 21 cases of pemphigus, 25 cases of bullous pemphigoid and one case each of epidermolysis bullosa acquisita and dermatitis herpetiformis. The sensitivity of IgG4 IHC was found to be 85.7%, specificity 96.3%, positive predictive value of 94.7% and negative predictive value 89.7%. So, we conclude that IgG4 IHC done on paraffin sections can be used as a diagnostic tool for pemphigus.

Diagnostic delay in autoimmune oral diseases.

Autoimmune diseases (ADs) affect about 5% of the general population, causing various systemic and/or topical clinical manifestations. The oral mucosa is often affected, sometimes as the only involved site. The misdiagnosis of oral ADs is an underreported issue. This narrative review focuses on diagnostic delay (DD) in oral ADs (oral lichen planus [OLP], oral Pemphigus Vulgaris, mucous membrane pemphigoid, oral lupus erythematosus, orofacial granulomatosis, oral erythema multiforme [EM], and Sjogren syndrome). Extensive literature research was conducted via MEDLINE, Embase and Google Scholar databases for articles reporting the time spent to achieve the correct diagnosis of oral ADs. Only 16 studies reported DD in oral ADs. Oral autoimmune vesiculobullous diseases are usually diagnosed after 8 months from the initial signs/symptoms, the Sjogren Syndrome diagnosis usually requires about 73months. No data exist about the DD in OLP, oral lupus erythematosus, orofacial granulomatosis, and oral EM. The diagnosis of oral ADs can be difficult due to the non-specificity of their manifestations and the unawareness of dentists, physicians, and dental and medical specialists about these diseases. This can lead to a professional DD and a consequential treatment delay. The delay can be attributed to the physicians or/and the healthcare system (Professional Delay) or the patient (Patient's Delay).

Mucous Membrane Pemphigoid: A Case Report with Oral and Ocular Presentation

To describe the diagnosis and management of mucous membrane pemphigoid (MMP) with oral and ocular presentation. Mucous membrane pemphigoid constitutes a heterogeneous group of chronic, autoimmune vesiculobullous diseases characterized by blister formation that has a propensity to affect different mucous membranes of the body. The most commonly affected areas include the oral cavity, mucous membranes of the eyes, throat, genitalia, and nose. This disease usually affects elderly women with a peak incidence at around 50-70 years of age; however, rare cases have been diagnosed in children. The symptoms of MMP include recurrent blistering lesions which eventually rupture and occasionally heal with scarring that may lead to certain complications involving the eyes and throat regions. In this report, we describe a 66-year-old female patient who complained of oral and ocular lesions for a period of 2 years. Pain, burning mouth, and gingival inflammation were present. Ocular examination showed mild conjunctivitis with scar formation at the lateral canthus of the left eye. The patient also noticed periods of water-filled balloon-like formation in the gingiva that rupture spontaneously leaving sore spots. A biopsy was obtained from perilesional tissue and sent for histopathological examination, correlation of clinical and histological features directed us toward the diagnosis of MMP. The patient was treated for both oral and ocular lesions using topical corticosteroid therapy in conjunction with antifungal and antibacterial drugs. The response to local treatment was augmented via effective periodontal therapy to control the concurrent plaque-induced gingival inflammation and via using a customized application tray to sustain the drug efficacy. A multidisciplinary approach is often necessary in order to treat MMP lesions efficaciously. Early diagnosis and effective treatment protocol using systemic or topical corticosteroid therapy along with other therapeutic means including periodontal therapy, good oral hygiene practice, and timely follow-up are very useful in preventing long-term complications due to this disease.

Leukocytoclastic vasculitis in association with linear epidermal basement membrane zone immunoglobulin deposition: Linear vasculitis.

Cutaneous leukocytoclastic vasculitis (LCV) has a distinctive clinical and light microscopic presentation; however, the etiologic basis of LCV is varied. Most cases are attributable to immune complex deposition within a vessel wall and represent an Arthus type III immune complex reaction. The prototypic immunoreactant profile is characterized by granular deposits of components of complement activation in concert with immunoglobulin within the cutaneous vasculature. We encountered nine patients with vasculitic and/or vesiculobullous clinical presentations exhibiting an LCV in association with an immunoreactant profile characterized by homogeneous linear deposits of immunoglobulin along the dermal epidermal junction in a fashion resembling an autoimmune vesiculobullous disease. Among the clinical presentations were palpable purpura, urticarial vasculitis, and vesiculobullous eruptions with supervening purpura. Two patients with Crohn disease presented with classic palpable purpura with biopsy-proven LCV, and direct immunofluorescence (DIF) studies demonstrated linear immunoglobulin G (IgG) with floor localization on the salt-split skin assay. Four patients with systemic lupus erythematosus (SLE) showed purpuric vesiculobullous lesions, with evidence of a neutrophilic interface dermatitis and LCV in three of the four. The remaining patient had urticarial nonbullous lesions showing small-vessel vasculitiswith a neutrophilic interface dermatitis. In all of the patients with SLE, DIF studies showed linear immunoglobulin deposits within the basement membrane zone (BMZ). These constellation of findings clinically, light microscopically, and by immunofluorescence were those of a vasculitic presentation of bullous systemic lupus erythematosus. Two patients had linear IgA disease, which was drug induced in one and paraneoplastic in the other, and the dominant morphology on biopsy in both cases was an LCV. One patient microscopically demonstrated drug-associated and eosinophilic enriched LCV with DIF studies showing striking linear deposits of IgG suggestive of bullous pemphigoid, which was consistent with a vasculitic presentation of drug-induced bullous pemphigoid. In all cases, typical granular vascular immunoglobulin and complement deposition compatible with immune complex mediated vasculitis was observed. It is likely that local immune complexes derived from BMZ antigen bound to antibody are pathogenically relevant. We propose the designation of linear vasculitis for this unique scenario of LCV and linear immunoglobulin epidermal BMZ staining, which in some cases represents a vasculitic presentation of conventional autoimmune vesiculobullous disease.

Bullous systemic lupus erythematosus with nephrotic syndrome in an adolescent girl: a case report

Bullous systemic lupus erythematosus is an uncommon autoimmune subepidermal vesiculobullous disease occurring in less than 5% of patients with systemic lupus erythematosus. The diagnosis of bullous systemic lupus erythematosus is based on clinical picture, histopathological findings, direct immunofluorescence and other immunologic tests. Importantly, establishing the diagnosis of bullous systemic lupus erythematosus requires the occurrence of systemic lupus erythematosus according to the American College of Rheumatology criteria. We report a case of bullous systemic lupus erythematosus in a 16-year-old Vietnamese girl, who demonstrated an acquired vesiculobullous eruption associated with nephrotic syndrome and fulfilled the European League Against Rheumatism/ American College of Rheumatology 2019 classification criteria of systemic lupus erythematosus. Clinical examination showed numerous tense, clear fluid-filled vesicles and bullae affecting the whole body with the predilection for the face, neck, chest, abdomen and bilateral inner thigh areas. Erosions were observed on the tongue and the buccal mucosa. Histopathologic examination showed subepidermal vesicle containing abundant neutrophils and perivascular mixed inflammatory cell infiltration. Microabscesses at tips of dermal papillae and the features of a leukocytoclastic vasculitis were not seen. Direct immunofluorescence of perilesional skin biopsies demonstrated linear deposition of IgG, IgA, IgM and C3 in the basement membrane zone. Linear immunoglobulin deposition displayed a u-serrated pattern with the predominant staining intensity of IgG. Serologic tests showed positive antinuclear antibody. Complement components assay revealed low levels of C3 (66.5 mg/dL) and C4 (3,9mg/dL). Proteinuria level was increased to 3.5g/24 hours. The final diagnosis of bullous systemic lupus erythematosus with nephrotic syndrome was made. The patient was treated with a high-dose oral glucocorticoid scheme and showed significant clinical improvement.

Antituberculosis Drug-induced Linear IgA Dermatosis

Introduction: Linear IgA dermatosis is a rare autoimmune vesiculobullous disease characterized by homogeneous linear IgA deposits in basement membrane of epidermis, and it can be idiopathic or drug-induced. The pathogenesis of drug-induced linear IgA dermatosis is not fully known yet, but it is associated with specific T cells. The clinical manifestations of the disease include vesiculobullous eruption, erythematous plaques, or string of pearls. Most cases still need additional therapy to avoid the expansion of the disease. Case Presentation: In this study, we present a 17-year-old male patient with erythema plaques, vesicles, and bullae with erosion in facial, oral, neck, trunk, genital, and extremities, pruritus, and burning sensation. The patient was undergoing pulmonary tuberculosis (TB) treatment for one week. Physical examination was done, and total BSA 10% and negative Asboe-Hansen sign were seen. The treatment consisted of delaying administration of TB drugs, desoximetasone cream 0.25%, cetirizine 10 mg, and aspiration of bullae. Conclusions: Drug-induced linear IgA dermatosis can occur at any age due to the administration of rifampicin and other antibiotics, angiotensin-I converting enzyme (ACE) inhibitors, or nonsteroidal anti-inflammatory drugs (NSAIDs). The drug can stimulate specific T cells that release Th2 cytokines to produce IgA antibodies against the basement membrane of epidermis. Drugs may cause an autoimmune response by cross-reaction with the target epitope, altering the conformation of epitopes, or exposing previously sequestered antigens to the immune system. The causative drug was stopped, and methyl prednisolone 0.5 - 1 mg/kg/day was given as initial therapy. In this study, we reported a rare case of a 17-year-old male with anti-TB drug-induced linear IgA dermatosis. Diagnosis was done based on clinical manifestation, histopathology, and immunofluorescence. The causative drug was stopped, the patient was given topical and systemic steroid therapy and drug desensitization. Remission was noted after six weeks of therapy, and oral steroid was slowly tapered and stopped on day 42. After stopping oral steroids, no lesions were reported. A 6-month follow-up revealed no signs of recurrence.

Pemphigus and its variants with direct immunofluorescence: Case series of 32 cases

Pemphigus is a group of autoimmune vesiculobullous diseases of skin and mucous membrane. Histologically characterised by intraepidermal bulla and immunologically by circulating IgG autoantibodies targeted against keratinocytes. We are presenting 32 cases of pemphigus and its variants. The histopathologic and direct immunofluorescence (DIF) findings are described.

Pemphigus Vegetans of Neumann Type on Lip: A Case Report

: 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.

Custom-made trays for targeted treatment of oral vesiculobullous diseases: A proposed protocol.

Topical and intralesional corticosteroids (CS) are effective treatments for oral manifestations of autoimmune vesiculobullous diseases (VBD); however, may cause unnecessary absorption in the adjacent healthy mucosal surfaces, local infections, atrophy, and injection pain. To propose a protocol using a unique modality for targeted administration of CS for the treatment of local manifestations of oral VBD. The medical records of nine patients with systemic CS-resistant VBD oral lesions, were reviewed following 3 weeks of treatment and 3 months of tapering using a custom-made tray designed for precise application of topical CS targeted to the involved mucosa. Following treatment, lesions have healed in 8/9 patients. Involvement of surrounding mucosa was minimal, and no systemic adverse effects were recorded. The proposed protocol using custom-made trays for targeted CS treatment could be an effective alternative treatment for oral VBD.

Oral Paraneoplastic Pemphigus and Pharyngeal Squamous Cell Carcinoma: A Rare Case Report and Review of Literature

Paraneoplastic pemphigus (PNP) is an autoimmune vesiculobullous disease of the skin and mucous membrane associated with benign and malignant neoplasm. The most common malignancies associated with PNP are hematologic and lymphomatoid. Association of oral PNP with squamous cell carcinoma of the pharynx is very scarce, very few cases are reported, and also the cases of PNP are usually refractory to the treatment mainly those associated with hematologic. Here, we reported a case of oral paraneoplastic pemphigus with squamous cell carcinoma of the pharynx that resolved on its own after completion of treatment of pharyngeal squamous cell carcinoma.

Evaluation of methicillin-resistant Staphylococcus aureus infections in pemphigus vulgaris patients: cross-sectional study

Background Pemphigus vulgaris (PV) is a potentially fatal autoimmune vesiculobullous disease. Staphylococcus aureus is the most common cause of cutaneous bacterial infection in pemphigus. Methicillin-resistant S. aureus (MRSA) is a common multidrug-resistant bacterium. MRSA infection is high among dermatology inpatients and vesiculobullous diseases patients are the commonest infected.Objective This study aimed at determining the causative organisms of the secondary bacterial skin infections in PV patients, identifying MRSA, doing antibiotic sensitivity pattern for MRSA, and determining the risk factors for acquiring MRSA infection among PV patients in the locality.Patients and methods This cross-sectional study included 54 PV patients. All patients were subjected to history taking, general, and dermatological examinations. Pemphigus disease area index score was calculated. Three swabs for bacterial culture and antibiotic sensitivity testing were obtained (one from vesicle or bulla, one from pustule, and the third from the nose).Results In all, 51 (94.4%) patients showed positive cultures for S. aureus either as nasal carrier, skin colonization, or skin infection. There was overlap as some patients showed more than one type of positive culture at the same time. Of the patients, 19 out of 51 patients (37.25%) had positive cultures for MRSA. All MRSA strains were sensitive to vancomycin. Also, they had good sensitivity to trimethoprim–sulfamethoxazole, amikacin, ciprofloxacin, and erythromycin, while all MRSA strains were resistant to cefoxitin and ampicillin. Hospitalization, previous antibiotics use in the last 4 weeks prior to this study, length of hospital stay, history of hospital-acquired infections, and community-acquired infections were significantly higher among MRSA-positive patients.Conclusion There is high MRSA infection/colonization among PV patients. MRSA responds to cheap antibiotics as trimethoprim–sulfamethoxazole, amikacin, and ciprofloxacin.

Frequency of Type 8 Human Herpes Virus in Patients with Pemphigus Vulgaris in Tabriz Sina Hospital since 2016-2017

Background: Pemphigus vulgaris is the most common form of autoimmune vesiculobullous skin disease. Human herpesvirus-8 (HHV-8) has a well-known association with Kaposi's sarcoma. Considering the reports about the incidence of Kaposi's sarcoma in patients with pemphigus and bolus pemphigoid without evidence of HIV infection, this study was designed to investigate the presence of HHV-8 in patients with pemphigus. Methods: In this cross-sectional and descriptive-analytic study, 27 patients with newly diagnosed pemphigus vulgaris were selected. The control group included 27 patients with benign non- vesiculobullous skin lesions. IHC (Immunohistochemistry) staining was performed to detect HHV-8 in tissue samples taken from skin lesions or mucosal lesions in both groups. Results: IHC showed HHV-8 in 12 patients in case group with pemphigus vulgaris (44.4%) and 9 patients in control group (33.3%) (P=0.402). Conclusion: Although HHV-8 prevalence was higher in Pemphigus vulgaris patients, this difference was not statistically significant, and there is unlikely to be a causal relationship between HHV-8 and Pemphigus.

Prevalence of oral lesions in pemphigus vulgaris: a retrospective study

<p class="abstract"><strong>Background:</strong> 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.</p><p class="abstract"><strong>Methods:</strong> 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.<strong></strong></p><p class="abstract"><strong>Results:</strong> 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%).</p><p class="abstract"><strong>Conclusions:</strong> Our study shows that oral lesions as initial manifestation of pemphigus vulgaris is 67.2%.</p>

Clinicopathological and immunofluorescence study of vesiculobullous disorders

<p class="abstract"><strong>Background:</strong> Vesiculobullous diseases are mostly immune mediated and diagnosed based on the clinical features, histology and Immunofluorescence. The aim of the study was to identify the immunofluorescence pattern in auto immune vesiculobullous diseases and correlate it with the clinical profile and histology.</p><p class="abstract"><strong>Methods:</strong> Patients attending the dermatology outpatient department in a tertiary hospital with vesiculobullous diseases, suggestive of auto immune aetiology were evaluated clinically. Histopathology and direct immuno-fluorescence were done in all patients.<strong></strong></p><p class="abstract"><strong>Results:</strong> During the one year period from June 2008 to July 2009, 40 patients with vesiculobullous disorders clinically suggestive of auto immune aetiology attended the outpatient department. Out of the 40 patients, 22 (55%) patients were diagnosed to have intraepidermal with female preponderance and 18 patients (45%) sub epidermal blistering diseases. Bullous pemphigoid was the commonest sub epidermal disease, seen in 8 patients.</p><p class="abstract"><strong>Conclusions:</strong> In all cases diagnosed clinically as pemphigus a histological diagnosis of pemphigus was made (100%). The clinical variants of pemphigus could also be diagnosed in all cases histologically (100%). The positive and negative predictive value was 100% in pemphigus group cases. Histology of all patients showed subepidermal bulla (100%). A specific diagnosis could be made in 18 patients with sub epidermal disease (100%). DIF was found to be an invaluable tool in diagnosing different diseases belonging to the sub epidermal group, but it was not of much help in sub classifying variants of pemphigus.</p><p class="abstract"> </p>

Pemphigus in the eastern region of Turkey.

IntroductionPemphigus refers to a group of rare autoimmune vesiculobullous diseases with high morbidity and mortality, mainly affecting the skin and the mucosae.AimTo evaluate the incidence of pemphigus in the Eastern region of Turkey by analysing the clinical characteristics of the patients including the drugs used in the treatment and their side-effect profiles and dosing schedules, duration of treatment, length of hospital stay, and the characteristics of the lesions.Material and methodsThe retrospective study included 130 pemphigus patients who were admitted to the in- and out-patient dermatology clinics at the University Medical School between January 2005 and October 2017. Age, gender, length of hospital stay, localization of the lesions, initial and maintenance dosages, treatment-related complications, comorbidities, family history, and smoking status were recorded for each patient.ResultsThe 130 patients included 62 (47.69%) men and 68 (52.31%) women, of which 73 (56.15%) patients had a smoking history. The annual incidence rate of pemphigus was 1.036/100,000 population and pemphigus vulgaris (PV) was the most common clinical subtype of pemphigus in our patients (n = 122; 93.85%). The most common side effect of the treatment was oral candidiasis, followed by Cushingoid appearance.ConclusionsThe results indicated that the incidence of pemphigus is remarkably high in the Eastern region of Turkey and the associated factors including lifestyle changes should be taken into mind when considering these patients.

Pemphigus Vulgaris Presenting as a Refractory Desquamative Gingivitis: Six Years of Follow-Up

Pemphigus vulgaris (PV) is a severe, chronic, autoimmune vesiculobullous disease, characterized by the formation of autoantibodies against epithelial glycoproteins, especially desmoglein 1 and 3, which triggers epithelial acantholysis. The disease may affect the skin and pharyngeal, laryngeal, esophageal, nasal conjunctival, genital, and oral mucosal areas. Oral lesions usually present as superficial erosions and ulcerations, distributed randomly at any site. However, the palate, labial mucosa, ventral tongue, and gingiva are the most affected sites. The incidence is rare under 50 years of age. This study reported the case of a young woman on immunotherapy for PV, referred by the dermatologist for refractory gingivitis. Clinical examination showed desquamative gingivitis associated with periodontal disease. The gingival biopsy followed by histopathological analysis established the diagnosis of gingival pemphigus. The patient received appropriate gingival and periodontal treatment; after 6 years of follow-up, it was observed that the desquamative gingivitis and periodontal loss were controlled.