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Linear IgA bullous dermatosis (LABD) is an acquired autoimmune subepidermal blistering disorder. Diagnosis always relies on skin pathology and direct immunofluorescence (DIF), with typical linear deposits of IgA along the basement membrane zone (BMZ). The typical clinical manifestation is tense bullae arranged like the "string of pearls" companied with severe pruritus. Dapsone is often considered first-line therapy for LABD, and it is necessary to test the HLA-B*1301 gene to prevent the occurrence of dapsone-induced hypersensitivity syndrome (DHS). Here we report a case of LABD resistant to corticosteroid and sulfasalazine, while waiting for HLA-B*1301 gene test results, dupilumab was used to control severe pruritus.

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Linear immunoglobulin A (IgA) bullous dermatosis (LABD) is an autoimmune condition with various triggers. Because of the lack of randomized controlled trials on LABD treatment, management options are mostly anecdotal. This paper provides a comprehensive review of treatment options from a literature review of reported treatments to arm clinicians with a guideline for the management of LABD in both pediatric and adult patients as well as those recalcitrant to first-line therapy (dapsone and steroids). We additionally illustrate an algorithm to use for the management of LABD to aid clinicians when faced with unique patient circumstances.

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The oral mucosa is a mechanical barrier against the penetration and colonization of microorganisms. Oral homeostasis is maintained by congenital and adaptive systems in conjunction with normal oral flora and an intact oral mucosa. Components contributing to the defense of the oral cavity include the salivary glands, innate antimicrobial proteins of saliva, plasma proteins, circulating white blood cells, keratinocyte products of the oral mucosa, and gingival crevicular fluid. General disturbances in the level of immunoglobulins in the human body may be manifested as pathological lesions in the oral mucosa. Symptoms of immunoglobulin-related general diseases such as mucous membrane pemphigoid (MMP), pemphigus vulgaris (PV), linear IgA bullous dermatosis (LABD), Epidermolysis Bullosa Aquisita (EBA), and Hyper-IgE syndrome (HIES) may appear in the oral cavity. In this review, authors present selected diseases associated with immunoglobulins in which the lesions appear in the oral cavity. Early detection and treatment of autoimmune diseases, sometimes showing a severe evolution (e.g., PV), allow the control of their dissemination and involvement of skin or other body organs. Immunoglobulin disorders with oral manifestations are not common, but knowledge, differentiation and diagnosis are essential for proper treatment.

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Linear Immunoglobulin A Bullous Disease (LABD) is a rare dermatosis whose pathomechanisms are not yet completely understood. LABD has different features characterizing adults and children in terms of potential triggers, clinical manifestations, and prognosis. The aim of the present study is to review all neonatal and pediatric cases of LABD and summarize the major characteristics. Childhood LABD is mainly idiopathic with a benign prognosis. Neonatal cases are difficult to differentiate from infectious diseases and usually have a poor prognosis. Drugs are one of the possible triggers that can activate autoimmune responses through antigen mimicry and epitope spreading as well as different stimuli (e.g., infections, inflammatory diseases, trauma). The gold standard for the diagnosis is based on direct immunofluorescence. Prognosis is generally favorable but often depends on the prompt dermatological diagnosis, treatment and follow-up guaranteed by a multidisciplinary team, including pediatricians for this group of age.

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Immunoglobulin A (IgA) is generally considered as a non-inflammatory regulator of mucosal immunity, and its importance in diversifying the gut microbiota is increasingly appreciated. IgA autoantibodies have been found in several autoimmune or chronic inflammatory diseases, but their role in pathophysiology is ill-understood. IgA can interact with the Fc receptor FcαRI on immune cells. We now established a novel IgA autoimmune blistering model, which closely resembles the human disease linear IgA bullous disease (LABD) by using genetically modified mice that produce human IgA and express human FcαRI. Intravital microscopy demonstrated that presence of IgA anti-collagen XVII, - the auto-antigen in LABD-, resulted in neutrophil activation and extravasation from blood vessels into skin tissue. Continued exposure to anti-collagen XVII IgA led to massive neutrophil accumulation, severe tissue damage and blister formation. Importantly, treatment with anti-FcαRI monoclonal antibodies not only prevented disease, but was also able to resolve existing inflammation and tissue damage. Collectively, our data reveal a novel role of neutrophil FcαRI in IgA autoantibody-mediated disease and identify FcαRI as promising new therapeutic target to resolve chronic inflammation and tissue damage.

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Objective: To study the chemical constituents of stems and leaves from Aphanamixis sinensis and evaluate their antibacterial activities. Methods: The compounds were isolated and purified by various column chromatography techniques, such as silica gel, ODS, Sephadex LH-20, and MCI, and their structures were identified by physiochemical properties and spectroscopic data. Moreover, antimicrobial activities against Staphylococcus aureus ATCC 25923, Escherichia coli CICC 10003, and Salmonella enterica UK-1 8956 of compounds 1-13 were evaluated by filter paper method. Results: Fifteen compounds were isolated from A. polystachya which were elucidated as (23E)-25-methoxycycloart-23-en-3β-ol (1), 25-hydroxy-cycloart-23-en-3-one (2), 23 (Z)-9,19-cycloart-23-ene-3β,25-diol (3), 23 (E)-cycloart-23-en-3β,25-diol (4), ent-labd-8 (17), 13E-dien-15-ol (5), vulgaro (6), ambroxdiok (7), α-cadinol (8), 1 (10)-en-oxo-7α-isopropanoleremophilane (9), (5R,7R,10S)-isopterocarpolone (10), 1S,4S,5S,10R- 4,10-guaianediol (11), (4R,5R,7S,9S,10S)-(-)-eudesma-11 (13)-en-4,9-diol (12), phytol (13), ergosterol endoperoxide (14) and 7α-hydroxysitosterol (15). Conclusion: All isolates were obtained from this plant for the first time, and compounds 2, 5, 7-10, and 12-15 were isolated from the genus Aphanamixis for the first time. Compound 5 showed obvious activity against S. aureus ATCC 25923 with the MIC value of 5 μg/mL.

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@#INTRODUCTION: Linear IgA bullous disease (LABD) is a rare autoimmune blistering disease characterized by subepithelial bullae and linear IgA deposition along the basement membrane zone of the epidermis. Lesions present as pruritic papulovesicles and tense bullae which may coalesce forming annular or polycyclic urticarial plaques with blistering on the edge of the lesions forming the classic “string of pearls” sign. Lesions may affect the face, trunk, and extensor extremities. Incidence rates range from 0.5 to 2.3 cases per million individuals per year. Due to its rare occurrence, there are only a fewdocumented reports on cases of LABD, particularly in the Filipino population. CASE REPORT: A 33 year-old Filipino female consulted because of a 3-week history of severely pruritic vesicles and crusts on the face, trunk, and arms. Patient noted no gastrointestinal symptoms on consultation. Skin punch biopsy revealed subepidermal blisters with collection of neutrophils at the dermal papillae. Direct immunofluorescence showed strong (+2) deposits of linear IgA at the dermo- epidermal junction in perilesional skin thus validating the diagnosis. The patient’s serum was negative for IgA anti-tissue transglutaminase and IgA antiendomysial antibodies. Patient was treated with topical corticosteroids and Dapsone 50 mgs OD with remarkable improvement. CONCLUSION: Linear IgA bullous disease has very few reported cases especially in the Philippine setting. Dapsone is considered the first-line treatment. The disease may persist for a decade or longer, and relapses may occur. Careful history-taking accompanied by the histological, immunofluorescence, and serological findings must be done to ensure proper treatment and good prognosis.

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Adverse medication side effects are not uncommon in the inpatient setting, where polypharmacy is the norm. Linear IgA bullous dermatosis (LABD) can be a cutaneous side effect of commonly used inpatient medications, such as vancomycin. Symptoms of LABD can be severe, and proper recognition of this drug-induced disease is important to ensuring proper treatment, including the removal of the inciting agent. This report describes a case of vancomycin-associated LABD in a 66-year-old man and the proper management of drug-induced LABD.