RARE DISEASE
Linear IgA dermatosis
Linear IgA dermatosis
Linear IgA dermatosis
Drug discovery
1
drug
With orphan designation
Overview
Linear IgA dermatosis (LAD) is a rare autoimmune blistering disorder characterized by linear IgA deposition at the dermo-epidermal basement membrane. It manifests with tense blisters, annular plaques, and mucosal involvement (oral, ocular, or genital) in 70–80% of cases [1][6][10]. Diagnosis relies on direct immunofluorescence showing IgA linear deposition. While idiopathic in most cases, triggers include medications (vancomycin, NSAIDs), infections, and malignancies [1][6][11]. First-line treatment is dapsone or sulfonamides, with topical corticosteroids for mild cases [3][16]. Childhood-onset LAD typically resolves within 2–4 years, whereas adult forms are often chronic [10][16].
Burden
Childhood LAD: Self-limiting (median 3-year duration) with minimal scarring [6][10].
Adult LAD: Chronic course (30–40% remission rate) with risks of mucosal scarring (e.g., blindness, esophageal strictures) [1][6][10].
Associated comorbidities: lymphoproliferative disorders, ulcerative colitis, and reduced quality of life due to chronic pruritus and blistering [6][11][15].
Therapies
First-line: Dapsone (50–200 mg/day) or sulfonamides (sulfapyridine/sulfamethoxypyridazine) [1][3][16].
Adjunctive: Topical corticosteroids (clobetasol 0.05%) for localized disease; systemic corticosteroids for severe flares [3][16].
Refractory cases: IV immunoglobulin (IVIg), rituximab, colchicine, or mycophenolate mofetil [1][3][12].
Categories: rare skin diseases
Research Papers
151 drug discovery papers about Linear IgA dermatosis, with 2 first-in-class and 1 next-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
151 drug discovery papers about Linear IgA dermatosis, with 2 first-in-class and 1 next-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
categories:
Small molecules
small molecules
2026-06-16 | Management of Recurrent Linear IgA Dermatosis of Childhood in the Absence of Histopathology and Immunofluorescence: A Case Report from a Low-Resource Setting in South-Western Uganda.
Linear IgA Dermatosis of Childhood (LAD of childhood) is a rare autoimmune sub-epidermal blistering disorder classically confirmed by direct immunofluorescence demonstrating linear IgA deposition along the basement membrane zone. However, in resource-limited settings, access to immunopathological diagnostics is often restricted, posing significant challenges to timely diagnosis and management. We report the case of a 12-year-old girl from rural South-Western Uganda with a history of recurrent vesiculobullous eruptions beginning at 2 years of age, with subsequent episodes at 7 years and a current severe presentation at 12 years. The disease demonstrated a progressive increase in severity with each recurrence. On examination, she had multiple large, tense bullae arranged in annular and polycyclic patterns over the trunk, limbs, thighs, and groin, with a characteristic "string-of-pearls" configuration and no mucosal involvement. Due to unavailability and unaffordability of histopathology and immunofluorescence studies, a clinical diagnosis of LAD of childhood was made based on history, morphology, distribution, recurrence pattern, and exclusion of close differentials. The patient was treated with dapsone, systemic corticosteroids, antibiotic prophylaxis, and local wound care. Within one week of initiating therapy, there was marked clinical improvement, with cessation of new blister formation, resolution of existing bullae, and progressive re-epithelialization. This case underscores the critical role of clinical acumen in diagnosing LAD of childhood in resource-constrained settings where gold-standard investigations are inaccessible. It highlights that a thorough history, careful physical examination, recognition of characteristic lesion patterns, and therapeutic response to dapsone can provide sufficient diagnostic confidence. Importantly, lack of advanced diagnostic tools should not delay initiation of appropriate treatment. This report also emphasizes the need to strengthen diagnostic capacity in low-resource settings while reinforcing the value of clinical judgment in managing rare dermatological conditions.
2026-03-06 | Autoimmune Bullous Diseases Associated With Immune Checkpoint Inhibitors: An Analysis Based on a Systematic Review.
Autoimmune bullous diseases (AIBDs) constitute a rare yet potentially life-threatening subset of immune-related adverse events induced by immune checkpoint inhibitors (ICIs). However, the characteristics of ICI-induced AIBDs (ICI-AIBDs) and the factors influencing patient survival remain incompletely characterized. Therefore, we aimed to synthesize the available information on ICI-AIBD patients and sought to explore factors potentially influencing the survival outcome of this population. A systematic search of 5 databases was conducted. Cox regression analysis was used to identify potential factors affecting patient survival outcomes. Finally, a total of 188 studies with 319 participants were analyzed. The spectrum of AIBDs comprised bullous pemphigoid (n = 254, 79.6%), lichen planus pemphigoides (n = 34, 10.7%), mucous membrane pemphigoid (n = 11, 3.4%), pemphigus group (n = 7, 2.2%), linear IgA bullous dermatosis (n = 5, 1.6%), primarily induced by programmed cell death protein 1 inhibitors. In the exploratory analysis, female (hazard ratio [HR], 2.35; 95% confidence interval [CI], 1.10-5.02; p = 0.020) and pemphigus group (HR, 7.09; 95% CI, 1.87-26.96; p = 0.003) were potentially associated with higher mortality, whereas topical glucocorticoid therapy was potentially protective (HR, 0.44; 95% CI, 0.21-0.93; p = 0.025). In conclusion, we delineate the full clinical spectrum of ICI-AIBDs, tentatively exploring factors potentially affecting patients' survival, which provides insights for individualized therapy and may inform future clinical practice.
2026-01-13 | Linear IgA Bullous Dermatosis in an Elderly Patient Following Meloxicam and Candesartan Use: A Case Report.
Linear immunoglobulin A (IgA) bullous dermatosis (LABD) is a rare autoimmune blistering disorder characterized by subepidermal blisters and linear IgA deposition along the dermo-epidermal junction (DEJ). We report the case of a 61-year-old woman who developed multiple tense vesicles and bullae on an erythematous base affecting both upper and lower extremities after initiating meloxicam for postoperative analgesia following knee arthroplasty. A punch biopsy showed subepidermal blistering, while direct immunofluorescence demonstrated linear IgA deposition along the basement membrane zone, confirming LABD. After discontinuation of the suspected culprit drug and adjustment of her antihypertensive therapy, she was treated with oral dapsone 1 mg/kg (50 mg) daily for two weeks, resulting in clinical improvement. This case underscores the importance of thorough drug history taking and early recognition of drug-induced LABD, particularly in patients on multiple medications, where identifying the true offending agent may be challenging.
2026-01-01 | A Rapidly Evolving Vesiculobullous Eruption in a Toddler: A Case of Linear IgA Bullous Dermatosis
Linear immunoglobulin A (IgA) bullous dermatosis (LABD) is a rare autoimmune neutrophilic dermatosis that can mimic viral or molluscoid eruptions, thereby delaying treatment. We report a 2‐year‐old girl with a 2‐week history of pruritic vesiculobullous rash that began as a single “mosquito bite.” Multiple emergency department visits led to empiric acyclovir and topical steroids. Histopathologic confirmation of LABD, essential for diagnosis, was achieved by a skin biopsy performed on Day 8, which revealed a subepidermal bulla with 3+ linear IgA deposition at the dermoepidermal junction. She improved after dapsone dosed daily at 1 mg/kg −1 , plus a tapering course of prednisolone. Therefore, early consideration of LABD in toddlers with disseminated evolving vesicular lesions can prevent unnecessary treatments and expedite more targeted immunosuppressive therapy.
2025-12-31 | A Rare Type of Bullous Disorder in a Child. Can It Be Chronic Bullous Disease of Childhood?
Chronic bullous disease of childhood (CBDC), also known as childhood linear IgA bullous dermatosis, is a rare autoimmune disorder characterized by sub-epidermal blistering and linear deposition of immunoglobulin A at the demo-epidermal junction. Clinical manifestations in children include tense bulla (arranged annularly in the form of ring of pearl) and vesicles over the erythematous skin. Diagnosis is confirmed by skin biopsy from lesions showing sub-epidermal blisters in histopathological examination and linear deposition of IgA at the dermal-epidermal junction in direct immunofluorescence. Here, we report a case of CBDC in a 5-year-old child who was treated successfully with oral dapsone showing improvement in the skin lesions.
proteins
2023-10-10 | Retrospective analysis of autoimmune bullous diseases in Middle Franconia
Autoimmune bullous diseases (AIBDs) are a group of rare cutaneous disorders affecting cornified skin and mucous membranes. They are characterized by tense or flaccid blistering and erosions due to autoantibodies against desmosomal and hemidesmosomal structural proteins of the skin. This group of disorders can be divided into those of pemphigoid and those of pemphigus diseases. If left untreated, these autoimmune diseases can cause serious or even life-threatening complications such as loss of fluid, superinfections or impaired food intake. Due to modern standardized serological assays, the diagnosis of AIBDs can usually be confirmed in combination with their clinical appearance. Whereas for a long time corticosteroids were the major players in the treatment of these diseases, with the approval of rituximab and other immunosuppressive agents, the therapy has increasingly improved.In this study, we aimed to investigate epidemiologic and clinical features as well as diagnostics and therapy of bullous autoimmune diseases in Middle Franconia, a governorate within the German federal state of Bavaria. Patients diagnosed or treated because of a AIBDs between 01.04.2013 and 31.03.2019 at the dermatological department of the university hospital Erlangen were included in this retrospective study (n = 242). Patients were either diagnosed for the first time (n=176) or the diagnosis has been confirmed (n=66) at the department. The respective incidence was calculated among the 176 subjects who had been diagnosed at the center in this period. Data was taken from patient records and analyzed with Microsoft® Excel. The evaluation included the diagnoses of pemphigus vulgaris (PV), pemphigus foliaceus (PF), bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), linear IgA dermatosis (LAD), epidermolysis bullosa acquisita (EBA), and dermatitis herpetiformis (DH).This study shows that the incidence of each AIBDs in Middle Franconia is low and comparable (PV, PF, LAD, EBA) or lower (BP, MMP, DH) than in other studies and regions. BP is the most common newly diagnosed AIBD in Middle Franconia.Due to the chronic and sometimes severe course of AIBDs, repeated in-house treatments are often necessary. To date, mainly topically and systemically applied corticosteroids in combination with immunomodulators are used as first-line therapy.
2023-09-14 | Linear IgA bullous dermatosis induced by nemvaleukin alfa, an engineered interleukin 2 molecule, in a patient with treatment-refractory metastatic melanoma
Linear IgA bullous dermatosis (LABD) is characterized by linear deposition of IgA at the dermoepidermal junction. It is associated with medications, malignancies, and vaccinations.1 Here, we present a case of a patient with LABD treated with nemvaleukin alfa, a novel, engineered interleukin 2 (IL-2) receptor therapy currently in phase 2 investigation for treatment of advanced melanoma.
2021-08-31 | Updates in the Diagnosis and Management of Linear IgA Disease: A Systematic Review.
Background and Objectives: Linear IgA disease (LAD) is a rare autoimmune blistering disease with linear IgA deposits along the basement membrane zone. Direct immunofluorescence remains the gold standard for diagnosis, but other diagnostic measures reported in recent literature have proven useful in the setting of inconclusive preliminary results. Dapsone is a commonly used treatment, but many therapeutic agents have emerged in recent years. The objective of this study is to provide a comprehensive overview of updates on the diagnosis and management of LAD. Materials and Methods: A literature search was conducted from May to June of 2021 for articles published in the last 5 years that were related to the diagnosis and management of LAD. Results: False-negative results in cases of drug-induced LAD and the presence of IgG and IgM antibodies on immunofluorescence studies were reported. Serration pattern analysis has been reported to be useful in distinguishing LAD from sublamina densa-type LAD. Rituximab, omalizumab, etanercept, IVIg, sulfonamides, topical corticosteroids, and others have been used successfully in adult and pediatric patients with varying disease severity. Topical corticosteroids were preferred for pediatric patients while rituximab and IVIg were used in adults with recalcitrant LAD. Sulfonamides were utilized in places without access to dapsone. Conclusion: In cases where preliminary biopsy results are negative and clinical suspicion is high, repeat biopsy and additional diagnostic studies should be used. Patient factors such as age, medical comorbidities, and disease severity play a role in therapeutic selection.
2021-06-23 | Intravenous Immunoglobulins in a Series of 32 Rare and Recalcitrant Immune Dermatoses.
Intravenous immunoglobulins are an effective and well-tolerated treatment option for immune dermatological diseases. However, they are primarily used to treat diseases with a severe course and are mostly used off-label. Therefore, it is important to document case series on the use of intravenous immunoglobulins in rare immune dermatological diseases. We present here 32 patients who were treated with intravenous immunoglobulins in our clinic between 2015 and 2020. The indications were dermatomyositis, including amyopathic and paraneoplastic forms, as well as overlap-syndromes (n = 18), pemphigus vulgaris (n = 2), mucous membrane pemphigoid (n = 2), linear IgA dermatosis (n = 1), necrotizing vasculitis (n = 1), urticarial vasculitis (n = 1), systemic scleroderma (n = 1), Stevens-Johnson syndrome (n = 1), pyoderma gangrenosum (n = 3), and livedoid vasculopathy (n = 2). The data from this case series confirm the efficacy and tolerability of intravenous immunoglobulins as an adjuvant treatment option for rare therapy-resistant immune dermatoses.
2011-09-19 | Intravenous immunoglobulin in the treatment of autoimmune bullous dermatoses: An update
High-dose intravenous immunoglobulin (IVIg) is being increasingly utilized as an off-label therapy for a variety of autoimmune and inflammatory conditions across various specialties. Numerous reports have shown that it is an effective treatment for autoimmune skin blistering disorders. Unlike most therapies for blistering disorders, IVIg is not immunosuppressive and has a favorable side effect profile. This has allowed its use to expand dramatically over the last decade. However, due to the rarity and severity of autoimmune skin blistering diseases, well-designed prospective trials are generally lacking. This work highlights major research developments and the best evidence to date regarding the treatment of autoimmune pemphigus, bullous pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, pemphigoid gestationis, and linear IgA dermatosis with IVIg, providing an update on its efficacy, proposed mechanisms of action, side effect profile, and indications for use.
antibodies
2026-08-08 | Rituximab for Refractory Adult Linear Iga Bullous Dermatosis: A Case Report
Linear IgA bullous dermatosis (LABD) is a rare autoimmune bullous disease characterized by linear IgA deposition along the basement membrane zone. While dapsone remains the cornerstone of treatment, a subset of adult patients develops severe, treatment-refractory disease requiring prolonged systemic corticosteroid therapy, and the role of rituximab in this setting remains poorly documented. We report the case of a 34-year-old woman with severe mucocutaneous LABD who failed to achieve disease control despite 18 months of treatment with dapsone, high-dose systemic corticosteroids, and doxycycline, resulting in persistent corticosteroid dependence and significant impairment in quality of life. Following confirmation of the diagnosis by repeat histopathology and direct immunofluorescence, rituximab was initiated using the pemphigus protocol (two 1-g infusions administered two weeks apart), followed by maintenance infusions every six months. A marked and sustained clinical improvement became evident from the second treatment cycle, allowing complete withdrawal of systemic corticosteroids and doxycycline, and at the latest follow-up the patient remained clinically stable on dapsone monotherapy, with only minimal residual cutaneous activity. This case highlights rituximab as a promising steroid-sparing rescue therapy for severe refractory adult LABD and contributes to the limited evidence supporting its use in this uncommon autoimmune bullous disease; further studies are needed to establish its optimal treatment regimen and long-term efficacy.
2026-05-01 | D104-11 Fatal Linear IgA Bullous Dermatosis Masquerading as Steven-Johnson Syndrome
Abstract Introduction Linear IgA bullous dermatosis (LABD) is a rare autoimmune disorder with generally good prognosis. Here we present for the first time, a case of drug induced LABD, initially thought to be Steven Johnson Syndrome (SJS), which eventually led to necrotizing pneumonia and a fatal outcome. Case A 66-year-old lady with chronic diarrhea due to lymphocytic colitis presented for tachycardia and hypovolemia. She received one dose of ciprofloxacin and metronidazole and was resuscitated with intravenous fluids. The next day, a diffuse petechial rash appeared which developed into bullous lesions within 24 hours. The patient was presumed to have SJS. A skin biopsy was performed, and etanercept was initiated. The biopsy was consistent with LABD, showing inflammatory subepidermal blister with neutrophils, lymphocytes and occasional eosinophils and necrotic keratinocytes with strong linear deposition of IgA present along the basement membrane zone on immunofluorescence, and the patient was started on dapsone and a prednisone taper. During this time, the patient developed new oral and cutaneous lesions due to herpes reactivation [positive for HSV-1 DNA in nasopharynx and in skin lesions]. Her clinical condition worsened secondary to right lower lobe MRSA pneumonia per pulmonary infiltrates and bacteremia. After an initial course of cefepime and linezolid for sepsis and high dose acyclovir for diffuse cutaneous herpes, the antibiotics were narrowed to ceftaroline. Oxygenation initially improved but the patient was persistently bacteremic. Repeat imaging showed cavitary lesions in the right lower lobe. The patient’s condition progressed to shock, ARDS, and was complicated by pneumothorax and acute renal failure. Despite multiple life support treatments, she did not show clinical improvement, and the family transitioned to comfort-oriented care. Discussion LABD is a rare condition affecting 0.2-2.3 cases/million individuals/year, among which fatal complications have rarely been reported. Our patient likely developed LABD due to ciprofloxacin exposure, which is an uncommon association. Her mucosal disruptions likely led to bacterial translocation, and necrotizing pneumonia. Due to her weakened immune status from both LABD and etanercept and prednisone, despite aggressive therapies, she remained persistently bacteremic, which lead to multiorgan failure and to her demise. Our case highlights the importance of prompt evaluation of blistering dermatoses for accurate treatment, and appropriate wound care to prevent infections which can lead to severe complications. This abstract is funded by: None
2026-05-01 | Vancomycin-induced linear IgA bullous dermatosis mimicking Stevens–Johnson syndrome/toxic epidermal necrolysis treated with etanercept
Linear IgA bullous dermatosis (LABD) is a rare subepidermal autoimmune blistering disorder defined by linear IgA deposition at the basement membrane zone of the skin or mucous membranes.1 Drug-induced LABD (DILAD) constitutes a significant proportion of LABD cases, with vancomycin identified as the most common precipitating agent.1,2
2026-04-01 | Administration of dupilumab for the treatment of paediatric linear IgA dermatosis inadequately controlled with dapsone and prednisolone
BackgroundOral lichen planus (OLP) is one of the most common non-infectious oral mucosal diseases. Currently, corticosteroid therapy (CST) is regarded as the first-line treatment for OLP, however, long-term use of CST may lead to adverse effects and be ineffective for a minority of patients. Objectives: To systematically evaluate the efficacy of photodynamic therapy (PDT) versus CST in the treatment of OLP.Materials & MethodsA comprehensive search was conducted in Chinese- and English-language electronic databases, including China National Knowledge Infrastructure, Wanfang Data, VIP, PubMed, Embase, and the Cochrane Library, for studies that compared the efficacy of PDT and CST, with the search timeframe set from the establishment of each database to October 2025. Data from eligible studies were analysed using Stata 15.0 software.ResultsIn total,13 randomised controlled trials were included, involving 587 patients (296 in the PDT group and 291 in the CST group). Pooled analysis revealed no statistically significant differences between the two groups in the total effective rate for OLP treatment or in the improvement in Visual Analogue Scale (VAS) score, Thongprasom (TH) sign score, or lesion area (all p>0.05). Subgroup analysis indicated that country and photosensitiser type were the sources of heterogeneity among the studies. Moreover, subgroup analyses revealed that in studies conducted in non-Chinese countries and in studies using phenothiazine dye-based photosensitisers (e.g. toluidine blue), CST showed significantly superior efficacy to PDT in improving VAS score, TH score, and lesion area (p<0.05).ConclusionCurrent evidence indicates that PDT and CST have comparable efficacy in the treatment of OLP. This equivalence positions PDT as a viable non-steroidal alternative, particularly valuable for patients who are intolerant to, have contraindications against, or seek to avoid the local and systemic side effects associated with long-term corticosteroid use.
2026-03-01 | Refractory linear IgA dermatosis in childhood: a successful response to rituximab
Refractory linear IgA dermatosis in childhood: a successful response to rituximab
small molecules
2026-06-16 | Management of Recurrent Linear IgA Dermatosis of Childhood in the Absence of Histopathology and Immunofluorescence: A Case Report from a Low-Resource Setting in South-Western Uganda.
Linear IgA Dermatosis of Childhood (LAD of childhood) is a rare autoimmune sub-epidermal blistering disorder classically confirmed by direct immunofluorescence demonstrating linear IgA deposition along the basement membrane zone. However, in resource-limited settings, access to immunopathological diagnostics is often restricted, posing significant challenges to timely diagnosis and management. We report the case of a 12-year-old girl from rural South-Western Uganda with a history of recurrent vesiculobullous eruptions beginning at 2 years of age, with subsequent episodes at 7 years and a current severe presentation at 12 years. The disease demonstrated a progressive increase in severity with each recurrence. On examination, she had multiple large, tense bullae arranged in annular and polycyclic patterns over the trunk, limbs, thighs, and groin, with a characteristic "string-of-pearls" configuration and no mucosal involvement. Due to unavailability and unaffordability of histopathology and immunofluorescence studies, a clinical diagnosis of LAD of childhood was made based on history, morphology, distribution, recurrence pattern, and exclusion of close differentials. The patient was treated with dapsone, systemic corticosteroids, antibiotic prophylaxis, and local wound care. Within one week of initiating therapy, there was marked clinical improvement, with cessation of new blister formation, resolution of existing bullae, and progressive re-epithelialization. This case underscores the critical role of clinical acumen in diagnosing LAD of childhood in resource-constrained settings where gold-standard investigations are inaccessible. It highlights that a thorough history, careful physical examination, recognition of characteristic lesion patterns, and therapeutic response to dapsone can provide sufficient diagnostic confidence. Importantly, lack of advanced diagnostic tools should not delay initiation of appropriate treatment. This report also emphasizes the need to strengthen diagnostic capacity in low-resource settings while reinforcing the value of clinical judgment in managing rare dermatological conditions.
2026-03-06 | Autoimmune Bullous Diseases Associated With Immune Checkpoint Inhibitors: An Analysis Based on a Systematic Review.
Autoimmune bullous diseases (AIBDs) constitute a rare yet potentially life-threatening subset of immune-related adverse events induced by immune checkpoint inhibitors (ICIs). However, the characteristics of ICI-induced AIBDs (ICI-AIBDs) and the factors influencing patient survival remain incompletely characterized. Therefore, we aimed to synthesize the available information on ICI-AIBD patients and sought to explore factors potentially influencing the survival outcome of this population. A systematic search of 5 databases was conducted. Cox regression analysis was used to identify potential factors affecting patient survival outcomes. Finally, a total of 188 studies with 319 participants were analyzed. The spectrum of AIBDs comprised bullous pemphigoid (n = 254, 79.6%), lichen planus pemphigoides (n = 34, 10.7%), mucous membrane pemphigoid (n = 11, 3.4%), pemphigus group (n = 7, 2.2%), linear IgA bullous dermatosis (n = 5, 1.6%), primarily induced by programmed cell death protein 1 inhibitors. In the exploratory analysis, female (hazard ratio [HR], 2.35; 95% confidence interval [CI], 1.10-5.02; p = 0.020) and pemphigus group (HR, 7.09; 95% CI, 1.87-26.96; p = 0.003) were potentially associated with higher mortality, whereas topical glucocorticoid therapy was potentially protective (HR, 0.44; 95% CI, 0.21-0.93; p = 0.025). In conclusion, we delineate the full clinical spectrum of ICI-AIBDs, tentatively exploring factors potentially affecting patients' survival, which provides insights for individualized therapy and may inform future clinical practice.
2026-01-13 | Linear IgA Bullous Dermatosis in an Elderly Patient Following Meloxicam and Candesartan Use: A Case Report.
Linear immunoglobulin A (IgA) bullous dermatosis (LABD) is a rare autoimmune blistering disorder characterized by subepidermal blisters and linear IgA deposition along the dermo-epidermal junction (DEJ). We report the case of a 61-year-old woman who developed multiple tense vesicles and bullae on an erythematous base affecting both upper and lower extremities after initiating meloxicam for postoperative analgesia following knee arthroplasty. A punch biopsy showed subepidermal blistering, while direct immunofluorescence demonstrated linear IgA deposition along the basement membrane zone, confirming LABD. After discontinuation of the suspected culprit drug and adjustment of her antihypertensive therapy, she was treated with oral dapsone 1 mg/kg (50 mg) daily for two weeks, resulting in clinical improvement. This case underscores the importance of thorough drug history taking and early recognition of drug-induced LABD, particularly in patients on multiple medications, where identifying the true offending agent may be challenging.
2026-01-01 | A Rapidly Evolving Vesiculobullous Eruption in a Toddler: A Case of Linear IgA Bullous Dermatosis
Linear immunoglobulin A (IgA) bullous dermatosis (LABD) is a rare autoimmune neutrophilic dermatosis that can mimic viral or molluscoid eruptions, thereby delaying treatment. We report a 2‐year‐old girl with a 2‐week history of pruritic vesiculobullous rash that began as a single “mosquito bite.” Multiple emergency department visits led to empiric acyclovir and topical steroids. Histopathologic confirmation of LABD, essential for diagnosis, was achieved by a skin biopsy performed on Day 8, which revealed a subepidermal bulla with 3+ linear IgA deposition at the dermoepidermal junction. She improved after dapsone dosed daily at 1 mg/kg −1 , plus a tapering course of prednisolone. Therefore, early consideration of LABD in toddlers with disseminated evolving vesicular lesions can prevent unnecessary treatments and expedite more targeted immunosuppressive therapy.
2025-12-31 | A Rare Type of Bullous Disorder in a Child. Can It Be Chronic Bullous Disease of Childhood?
Chronic bullous disease of childhood (CBDC), also known as childhood linear IgA bullous dermatosis, is a rare autoimmune disorder characterized by sub-epidermal blistering and linear deposition of immunoglobulin A at the demo-epidermal junction. Clinical manifestations in children include tense bulla (arranged annularly in the form of ring of pearl) and vesicles over the erythematous skin. Diagnosis is confirmed by skin biopsy from lesions showing sub-epidermal blisters in histopathological examination and linear deposition of IgA at the dermal-epidermal junction in direct immunofluorescence. Here, we report a case of CBDC in a 5-year-old child who was treated successfully with oral dapsone showing improvement in the skin lesions.
proteins
2023-10-10 | Retrospective analysis of autoimmune bullous diseases in Middle Franconia
Autoimmune bullous diseases (AIBDs) are a group of rare cutaneous disorders affecting cornified skin and mucous membranes. They are characterized by tense or flaccid blistering and erosions due to autoantibodies against desmosomal and hemidesmosomal structural proteins of the skin. This group of disorders can be divided into those of pemphigoid and those of pemphigus diseases. If left untreated, these autoimmune diseases can cause serious or even life-threatening complications such as loss of fluid, superinfections or impaired food intake. Due to modern standardized serological assays, the diagnosis of AIBDs can usually be confirmed in combination with their clinical appearance. Whereas for a long time corticosteroids were the major players in the treatment of these diseases, with the approval of rituximab and other immunosuppressive agents, the therapy has increasingly improved.In this study, we aimed to investigate epidemiologic and clinical features as well as diagnostics and therapy of bullous autoimmune diseases in Middle Franconia, a governorate within the German federal state of Bavaria. Patients diagnosed or treated because of a AIBDs between 01.04.2013 and 31.03.2019 at the dermatological department of the university hospital Erlangen were included in this retrospective study (n = 242). Patients were either diagnosed for the first time (n=176) or the diagnosis has been confirmed (n=66) at the department. The respective incidence was calculated among the 176 subjects who had been diagnosed at the center in this period. Data was taken from patient records and analyzed with Microsoft® Excel. The evaluation included the diagnoses of pemphigus vulgaris (PV), pemphigus foliaceus (PF), bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), linear IgA dermatosis (LAD), epidermolysis bullosa acquisita (EBA), and dermatitis herpetiformis (DH).This study shows that the incidence of each AIBDs in Middle Franconia is low and comparable (PV, PF, LAD, EBA) or lower (BP, MMP, DH) than in other studies and regions. BP is the most common newly diagnosed AIBD in Middle Franconia.Due to the chronic and sometimes severe course of AIBDs, repeated in-house treatments are often necessary. To date, mainly topically and systemically applied corticosteroids in combination with immunomodulators are used as first-line therapy.
2023-09-14 | Linear IgA bullous dermatosis induced by nemvaleukin alfa, an engineered interleukin 2 molecule, in a patient with treatment-refractory metastatic melanoma
Linear IgA bullous dermatosis (LABD) is characterized by linear deposition of IgA at the dermoepidermal junction. It is associated with medications, malignancies, and vaccinations.1 Here, we present a case of a patient with LABD treated with nemvaleukin alfa, a novel, engineered interleukin 2 (IL-2) receptor therapy currently in phase 2 investigation for treatment of advanced melanoma.
2021-08-31 | Updates in the Diagnosis and Management of Linear IgA Disease: A Systematic Review.
Background and Objectives: Linear IgA disease (LAD) is a rare autoimmune blistering disease with linear IgA deposits along the basement membrane zone. Direct immunofluorescence remains the gold standard for diagnosis, but other diagnostic measures reported in recent literature have proven useful in the setting of inconclusive preliminary results. Dapsone is a commonly used treatment, but many therapeutic agents have emerged in recent years. The objective of this study is to provide a comprehensive overview of updates on the diagnosis and management of LAD. Materials and Methods: A literature search was conducted from May to June of 2021 for articles published in the last 5 years that were related to the diagnosis and management of LAD. Results: False-negative results in cases of drug-induced LAD and the presence of IgG and IgM antibodies on immunofluorescence studies were reported. Serration pattern analysis has been reported to be useful in distinguishing LAD from sublamina densa-type LAD. Rituximab, omalizumab, etanercept, IVIg, sulfonamides, topical corticosteroids, and others have been used successfully in adult and pediatric patients with varying disease severity. Topical corticosteroids were preferred for pediatric patients while rituximab and IVIg were used in adults with recalcitrant LAD. Sulfonamides were utilized in places without access to dapsone. Conclusion: In cases where preliminary biopsy results are negative and clinical suspicion is high, repeat biopsy and additional diagnostic studies should be used. Patient factors such as age, medical comorbidities, and disease severity play a role in therapeutic selection.
2021-06-23 | Intravenous Immunoglobulins in a Series of 32 Rare and Recalcitrant Immune Dermatoses.
Intravenous immunoglobulins are an effective and well-tolerated treatment option for immune dermatological diseases. However, they are primarily used to treat diseases with a severe course and are mostly used off-label. Therefore, it is important to document case series on the use of intravenous immunoglobulins in rare immune dermatological diseases. We present here 32 patients who were treated with intravenous immunoglobulins in our clinic between 2015 and 2020. The indications were dermatomyositis, including amyopathic and paraneoplastic forms, as well as overlap-syndromes (n = 18), pemphigus vulgaris (n = 2), mucous membrane pemphigoid (n = 2), linear IgA dermatosis (n = 1), necrotizing vasculitis (n = 1), urticarial vasculitis (n = 1), systemic scleroderma (n = 1), Stevens-Johnson syndrome (n = 1), pyoderma gangrenosum (n = 3), and livedoid vasculopathy (n = 2). The data from this case series confirm the efficacy and tolerability of intravenous immunoglobulins as an adjuvant treatment option for rare therapy-resistant immune dermatoses.
2011-09-19 | Intravenous immunoglobulin in the treatment of autoimmune bullous dermatoses: An update
High-dose intravenous immunoglobulin (IVIg) is being increasingly utilized as an off-label therapy for a variety of autoimmune and inflammatory conditions across various specialties. Numerous reports have shown that it is an effective treatment for autoimmune skin blistering disorders. Unlike most therapies for blistering disorders, IVIg is not immunosuppressive and has a favorable side effect profile. This has allowed its use to expand dramatically over the last decade. However, due to the rarity and severity of autoimmune skin blistering diseases, well-designed prospective trials are generally lacking. This work highlights major research developments and the best evidence to date regarding the treatment of autoimmune pemphigus, bullous pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, pemphigoid gestationis, and linear IgA dermatosis with IVIg, providing an update on its efficacy, proposed mechanisms of action, side effect profile, and indications for use.
antibodies
2026-08-08 | Rituximab for Refractory Adult Linear Iga Bullous Dermatosis: A Case Report
Linear IgA bullous dermatosis (LABD) is a rare autoimmune bullous disease characterized by linear IgA deposition along the basement membrane zone. While dapsone remains the cornerstone of treatment, a subset of adult patients develops severe, treatment-refractory disease requiring prolonged systemic corticosteroid therapy, and the role of rituximab in this setting remains poorly documented. We report the case of a 34-year-old woman with severe mucocutaneous LABD who failed to achieve disease control despite 18 months of treatment with dapsone, high-dose systemic corticosteroids, and doxycycline, resulting in persistent corticosteroid dependence and significant impairment in quality of life. Following confirmation of the diagnosis by repeat histopathology and direct immunofluorescence, rituximab was initiated using the pemphigus protocol (two 1-g infusions administered two weeks apart), followed by maintenance infusions every six months. A marked and sustained clinical improvement became evident from the second treatment cycle, allowing complete withdrawal of systemic corticosteroids and doxycycline, and at the latest follow-up the patient remained clinically stable on dapsone monotherapy, with only minimal residual cutaneous activity. This case highlights rituximab as a promising steroid-sparing rescue therapy for severe refractory adult LABD and contributes to the limited evidence supporting its use in this uncommon autoimmune bullous disease; further studies are needed to establish its optimal treatment regimen and long-term efficacy.
2026-05-01 | D104-11 Fatal Linear IgA Bullous Dermatosis Masquerading as Steven-Johnson Syndrome
Abstract Introduction Linear IgA bullous dermatosis (LABD) is a rare autoimmune disorder with generally good prognosis. Here we present for the first time, a case of drug induced LABD, initially thought to be Steven Johnson Syndrome (SJS), which eventually led to necrotizing pneumonia and a fatal outcome. Case A 66-year-old lady with chronic diarrhea due to lymphocytic colitis presented for tachycardia and hypovolemia. She received one dose of ciprofloxacin and metronidazole and was resuscitated with intravenous fluids. The next day, a diffuse petechial rash appeared which developed into bullous lesions within 24 hours. The patient was presumed to have SJS. A skin biopsy was performed, and etanercept was initiated. The biopsy was consistent with LABD, showing inflammatory subepidermal blister with neutrophils, lymphocytes and occasional eosinophils and necrotic keratinocytes with strong linear deposition of IgA present along the basement membrane zone on immunofluorescence, and the patient was started on dapsone and a prednisone taper. During this time, the patient developed new oral and cutaneous lesions due to herpes reactivation [positive for HSV-1 DNA in nasopharynx and in skin lesions]. Her clinical condition worsened secondary to right lower lobe MRSA pneumonia per pulmonary infiltrates and bacteremia. After an initial course of cefepime and linezolid for sepsis and high dose acyclovir for diffuse cutaneous herpes, the antibiotics were narrowed to ceftaroline. Oxygenation initially improved but the patient was persistently bacteremic. Repeat imaging showed cavitary lesions in the right lower lobe. The patient’s condition progressed to shock, ARDS, and was complicated by pneumothorax and acute renal failure. Despite multiple life support treatments, she did not show clinical improvement, and the family transitioned to comfort-oriented care. Discussion LABD is a rare condition affecting 0.2-2.3 cases/million individuals/year, among which fatal complications have rarely been reported. Our patient likely developed LABD due to ciprofloxacin exposure, which is an uncommon association. Her mucosal disruptions likely led to bacterial translocation, and necrotizing pneumonia. Due to her weakened immune status from both LABD and etanercept and prednisone, despite aggressive therapies, she remained persistently bacteremic, which lead to multiorgan failure and to her demise. Our case highlights the importance of prompt evaluation of blistering dermatoses for accurate treatment, and appropriate wound care to prevent infections which can lead to severe complications. This abstract is funded by: None
2026-05-01 | Vancomycin-induced linear IgA bullous dermatosis mimicking Stevens–Johnson syndrome/toxic epidermal necrolysis treated with etanercept
Linear IgA bullous dermatosis (LABD) is a rare subepidermal autoimmune blistering disorder defined by linear IgA deposition at the basement membrane zone of the skin or mucous membranes.1 Drug-induced LABD (DILAD) constitutes a significant proportion of LABD cases, with vancomycin identified as the most common precipitating agent.1,2
2026-04-01 | Administration of dupilumab for the treatment of paediatric linear IgA dermatosis inadequately controlled with dapsone and prednisolone
BackgroundOral lichen planus (OLP) is one of the most common non-infectious oral mucosal diseases. Currently, corticosteroid therapy (CST) is regarded as the first-line treatment for OLP, however, long-term use of CST may lead to adverse effects and be ineffective for a minority of patients. Objectives: To systematically evaluate the efficacy of photodynamic therapy (PDT) versus CST in the treatment of OLP.Materials & MethodsA comprehensive search was conducted in Chinese- and English-language electronic databases, including China National Knowledge Infrastructure, Wanfang Data, VIP, PubMed, Embase, and the Cochrane Library, for studies that compared the efficacy of PDT and CST, with the search timeframe set from the establishment of each database to October 2025. Data from eligible studies were analysed using Stata 15.0 software.ResultsIn total,13 randomised controlled trials were included, involving 587 patients (296 in the PDT group and 291 in the CST group). Pooled analysis revealed no statistically significant differences between the two groups in the total effective rate for OLP treatment or in the improvement in Visual Analogue Scale (VAS) score, Thongprasom (TH) sign score, or lesion area (all p>0.05). Subgroup analysis indicated that country and photosensitiser type were the sources of heterogeneity among the studies. Moreover, subgroup analyses revealed that in studies conducted in non-Chinese countries and in studies using phenothiazine dye-based photosensitisers (e.g. toluidine blue), CST showed significantly superior efficacy to PDT in improving VAS score, TH score, and lesion area (p<0.05).ConclusionCurrent evidence indicates that PDT and CST have comparable efficacy in the treatment of OLP. This equivalence positions PDT as a viable non-steroidal alternative, particularly valuable for patients who are intolerant to, have contraindications against, or seek to avoid the local and systemic side effects associated with long-term corticosteroid use.
2026-03-01 | Refractory linear IgA dermatosis in childhood: a successful response to rituximab
Refractory linear IgA dermatosis in childhood: a successful response to rituximab
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Drug Discovery Landscape
1 orphan drug designation for Linear IgA dermatosis.
1 orphan drug designation for Linear IgA dermatosis.
Drug | Therapy type | Regulator | Orphan designation | Approval | Sponsor |
|---|---|---|---|---|---|
Human IgG4k monoclonal antibody against CD89 | antibodies | EMA | 2022-10-11 | — | Jjp Biologics Sp. z o.o. |
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