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BACKGROUND: Immunofluorescence testing is an important tool for diagnosing blistering diseases. OBJECTIVE: To characterize the immunofluorescence findings in patients diagnosed with autoimmune blistering skin diseases. METHODS: We retrospectively analyzed immunofluorescence results encompassing a 10-year period. RESULTS: 421 patients were included and divided into 2 groups: group 1- intraepidermal blistering diseases (n=277) and 2- subepidermal blistering diseases (n=144). For group 1, positive DIF findings demonstrated: predominance of IgG intercellular staining (ICS) and C3 for pemphigus foliaceus-PF (94% and 73% respectively), pemphigus vulgaris-PV (91.5%-79.5%) and paraneoplastic pemphigus-PNP (66%-33%); ICS IgA in 100% of IgA pemphigus cases, and IgG deposits in the basement membrane zone (BMZ) along with ICS in one Hailey-Hailey patient. The IIF findings revealed mean titers of 1:2.560 for PV and 1:1.280 for PF. For paraneoplastic pemphigus, IIF was positive in 2 out of 3 cases with rat bladder substrate. In group 2, positive DIF findings included multiple deposits at basement membrane zone for epidermolysis bullosa acquisita-EBA (C3-89%,IgG-79%,IgA-47%,IgM-21%) mucous membrane pemphigoid-MMP (C3,IgG,IgA,IgM-80%) and bullous pemphigoid-BP (C3-91%,IgG-39%,IgA-11%,IgM-6%), and IgA at basement membrane zone for IgA linear disease (99%) and dermatitis herpetiformis-DH (dermal papillae in 84.6%). For lichen planus pemphigoides, there was C3 (100%) and IgG (50%) deposition at basement membrane zone. indirect immunofluorescence positive findings revealed basement membrane zone IgG deposits in 46% of BP patients, 50% for EBA, 15% for IgA linear dermatosis and 50% for LPP. Indirect immunofluorescence positive results were higher for BP and EBA with Salt-Split skin substrate. CONCLUSION: Our results confirmed the importance of immunofluorescence assays in diagnosing autoimmune blistering diseases, and higher sensitivity for indirect immunofluorescence when Salt-split skin technique is performed.

O penfigoide de membranas mucosas é entidade nosológica encarada como um fenótipo, que engloba várias dermatoses autoimunes com lesões bolhosas subepidérmicas, ocorrendo predominantemente nas membranas mucosas, com êxito cicatricial. O acometimento esofágico no penfigoide de membranas mucosas é raro e observado em pacientes com lesão disseminada. As alterações mais comuns são múltiplas membranas ou constrições esofagianas. No presente relato, os autores apresentam paciente com PMM sem lesões cutâneas e estenose esofágica grave, que entrou em remissão após uso de imunoglobulina venosaMucous membrane pemphigoid (MMP) is a rare nosological entity. MMP consists of a clinical phenotype in which several autoimmune subepidermal bullous diseases are classified. It occurs predominantly in the mucous membranes and usually results in scarring. Esophageal involvement in MMP is rare and is generally seen in patients in whom lesions are widespread. The most common alterations are multiple esophageal membranes or strictures. In the present case, the authors report on a patient with MMP without any skin lesions and with severe esophageal strictures who went into remission following use of intravenous immunoglobulin

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Background: Mucous membrane pemphigoid (MMP) is a heterogeneous group of autoimmune blistering disorders characterized by subepithelial separation and the deposition of immunoglobulins and complement along the basement membrane zone (BMZ). This disease is diagnosed with direct immunofluorescence testing showing a linear deposition of immunoglobulins and/or complement along the BMZ and indirect immunofluorescence testing showing circulating IgG (and sometimes IgA) autoantibodies along the BMZ. In this case report we describe desquamative gingivitis secondary to IgG/IgA pemphigoid and the management of this challenging variant of MMP. Methods: Routine histology, direct immunofluorescence testing, and indirect immunofluorescence testing were utilized and correlated to the clinical findings to diagnose this unusual immunobullous disease. Results: Direct and indirect immunofluorescence testing confirmed the clinical diagnosis of IgG/IgA pemphigoid as the cause of desquamative gingivitis and the other mucosal findings in this patient. A treatment program including dapsone and other drugs completely resolved the oral lesions alter 14 months of therapy. Conclusions: Desquamative gingivitis associated with IgG/IgA pemphigoid can be challenging to diagnose and treat. After 14 months of treatment, a combination therapy consisting of dapsone with cimetidine and vitamin E to enhance drug efficacy and frequent intramuscular administrations of triamcinolone achieved control of both the oral and genital elements of IgG/IgA pemphigoid in this patient. [ABSTRACT FROM AUTHOR]

Objectives: To describe a method to measure the progression of ocular cicatricial pemphigoid and to compare its facility with traditional methods used to measure the progression of the disease. Methods: The proposed method consists of measuring (in millimeters) the total relative inferior conjunctival surface available in 3 gaze positions. This method was used to monitor 7 eyes of 4 patients with ocular cicatricial pemphigoid over 2 years. The changes in the conjunctival measurements from baseline were compared with the changes documented by traditional methods. Results: During the study, 2 eyes remained stable (changes, <3 mm), 2 had a decrease of 10 mm or more, and 3 had a change in measurements between 4 and 9 mm. With the proposed method, we demonstrated the detection of more subtle changes in the conjunctiva of all patients. Patients who had changes between 4 and 9 mm easily underwent staging by the traditional systems when the new technique was used as a reference. Conclusion: The proposed method offers an objective variable that can be used in consecutive visits to detect subtle progression or disease control in patients with ocular cicatricial pemphigoid. [ABSTRACT FROM AUTHOR]

Objectives: The purpose of the meeting of the Consensus Development Group was to critically evaluate the current published data on the use of intravenous immunoglobulin (IVIg) therapy in the treatment of autoimmune mucocutaneous blistering diseases (AMBDs) and to discuss the industrial preparation and safety features of this biologic agent. Participants: The participants were physicians who frequently treat patients with these diseases and included dermatologists, oral medicine specialists, ophthalmologists, and immunologists. The members of the group provided input and discussion in their areas of expertise. The participants were invited attendees. Evidence: Data samples included only published information in the English-language literature. The expert opinions and experience of the members of the Consensus Development Group were vital to the discussion. Consensus Process: A consensus was achieved by an open discussion and cumulative agreement on all issues relevant to the use of IVIg therapy in the treatment of AMBDs. Special emphasis was placed on indications for its use, determination of outcome parameters, and development of a protocol for its therapeutic use. We also focused on its safety and on prevention of adverse effects. Conclusion: This consensus statement outlines the scope of IVIg treatment; provides guidelines for its use, including indications, prescreening, premedications, dose, frequency, and monitoring; and defines the end point of therapy. [ABSTRACT FROM AUTHOR]

Background: Cicatricial pemphigoid (benign mucous membrane pemphigoid) is an autoimmune vesiculobullous disease that affects mucosal tissues of adults and rarely presents in children. Only 9 cases in the English literature have reported cicatricial pemphigoid in children, primarily as oral mucosal lesions. This paper presents a case of childhood cicatricial pemphigoid that clinically manifested as necrotizing ulcerative gingivitis (NUG). Methods: A 9-year-old girl presented with gingival bleeding and discomfort for 2 weeks. NUG was suspected and the patient was treated with antibiotics and an oral hygiene regimen. When the condition did not improve after repeated treatment trials, routine hematoxylin and eosin (H&E) and direct immunofluorescence examinations were performed. Results: Microscopic examination of H&E stained sections showed a non-specific ulceration with chronic inflammation. Direct immunofluorescence studies of peri-lesional tissue showed linear deposition of C3 at the basement membrane zone that was consistent with a diagnosis of cicatricial pemphigoid. Conclusion: Cicatricial pemphigoid is an autoimmune ulcerative condition that is rarely seen in children. Immunofluorescence studies are essential to differentiate this condition from other ulcerative oral lesions. [ABSTRACT FROM AUTHOR]

Cicatricial pemphigoid (CP) is a chronic subepidermal bullous dermatosis which primarily involves the mucous membranes. The oral cavity and the eye are most frequently involved. Since extension of the lesion into the pharynx and esophagus causes sore throat and dysphagia and progressive ocular lesions may cause blindness, early and valid diagnosis is very important. Here we present a case of cicatricial pemphigoid with onset at age 45 in a patient who manifested severe periodontal disease and showed the lesion on the mucous membranes of the mouth (desquamative gingivitis), skin, and eyes. Since definite diagnosis is very important, we describe how we made a differential diagnosis from other diseases which also accompany desquamative gingivitis. We examined the clinical manifestations, blood test results, HLA-genotype, histopathologic findings of the affected tissue, and immunological findings in relation to autoimmunity. Since many of the CP cases are first referred to periodontists or dentists, we believe that the diagnostic strategy described in the present study will be quite informative for making rapid and definite diagnoses of similar cases. [ABSTRACT FROM AUTHOR]

Cicatricial pemphigoid is one of a number of mucocutaneous disorders that can present in the oral cavity with desquamation, pain, and bleeding of the gingival and oral mucosa. This case report describes the management of cicatricial pemphigoid in a patient with multiple sites of gingival recession using connective tissue grafting to alleviate root surface sensitivity and improve esthetics. The treatment rationale is presented and discussed in terms of timing of therapy and implications for wound healing in patients who present with desquamative gingivitis. [ABSTRACT FROM AUTHOR]

Se presenta un caso de retracción esencial de la conjuntiva en una mujer de 83 años de edad, caso clínico poco frecuente y de gran interés. Se caracteriza por ser una conjuntivitis cicatrizal crónica que no debe ser confundida con el pénfigo vulgar. Afecta más a la mujer que al hombre (2:1), usualmente en pacientes de más de 60 años y rara en menores de 30. Existe la forma idiopática y la inducida por medicamentos. En los individuos afectados de penfigoide cicatrizal se han encontrado autoanticuerpos en la membrana basal del epitelio. Aquí se discuten su diagnóstico diferencial, el tratamiento impuesto, así como las tendencias terapéuticas actuales de la enfermedad. Para ello hicimos una revisión detallada del caso: sus antecedentes personales y familiares, historia de la enfermedad actual, exámenes de laboratorio y tratamiento impuesto.The case of an 83-year-old woman with essential retraction of the conjunctiva, a rare clinical case of great interest, is presented. It is characterized by a cicatrizal chronic conjunctivitis that should not be mistaken for pemphigus vulgaris. Women are more affected than men (2:1). It is usually observed in patients over 60 and rarely in individuals under 30. It may be idiopathic or induced by drugs. Autoantibodies have been found in the basal membrane of the epithelium among subjects with cicatrizal pemphigus. Its differential diagnosis, treatment and the current therapeutic trends of the disease are discussed here. We made a detailed review of the case that included her personal and family history, history of the present disease, laboratory tests, and treatment.