A case of linear IgA bullous dermatosis successfully treated with omalizumab

Barit

The Journal of Dermatology

et al. 2022

Pruritus, also known as itch, is classically defined as an uncomfortable sensation that provokes a desire to scratch. 1 Mild temporal pruritus is non-pathological and physiologically functional to remove external elements from one's own body. On the other hand, severe chronic pruritus is pathological and significantly affects quality of life. 2 Moreover, recurring pruritus leads to repeated scratching (itch-scratch cycle), aggravating disease morbidity by further damaging the skin and increasing proinflammatory cytokine release. 3 Therefore, the treatment of pruritus is important not only for relief from the uncomfortable sensation itself, but also for stopping the itch-scratch cycle, preventing disease exacerbation.Pemphigoid diseases (PDs) are a group of disorders characterized by tense blisters and edematous erythema, which include multiple diseases associated with severe pruritus, such as bullous pemphigoid (BP), epidermolysis bullosa acquisita (EBA), pemphigoid gestationis (PG), and linear IgA dermatosis (LAD). 4 Treatment targeting pruritus in PDs is important since itch in PDs is often intractable and significantly impairs patients' quality of life (QOL), with scratch behaviors

Section: Mechanisms Of Pruritusmentioning

confidence: 99%

“…44 Successful treatment with OMZ in LAD with an immediate improvement of pruritus has been demonstrated in a few case reports. 45,46 F I G U R E 1 Multiple pruritogens in pemphigoid diseases. Histamine induces pruritus through H1 and H4 receptor (H1R and H4R) activation on the cutaneous sensory neurons.…”

Section: Thymic Stromal Lymphopoietinmentioning

confidence: 99%

Pruritogens in pemphigoid diseases: Possible therapeutic targets for a burdensome symptom

Barit

The Journal of Dermatology

et al. 2022

“…Omalizumab is a monoclonal anti-IgE antibody approved for chronic idiopathic urticaria (CIU) and has also been successfully utilized in treatment for LABD patients in a few reports [67][68][69]. The efficacy of omalizumab alludes to a possible role of IgE in the pathogenesis of LABD.…”

Section: Omalizumabmentioning

confidence: 99%

Management Options for Linear Immunoglobulin A (IgA) Bullous Dermatosis: A Literature Review

Khan¹,

Park²,

Skopit³

2023

Cureus

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.

“…Monitoring for dapsone adverse reactions can be cumbersome as well. Numerous other treatments have been reported to be effective in the treatment of LAD, including topical corticosteroids, tetracyclines, dicloxacillin, oxacillin, erythromycin, sulfonamides, nicotinamide, rituximab, omalizumab, methotrexate, cyclosporine, etanercept, and intravenous immunoglobulin (IVIg) [1,[5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21]. This review provides updates on the diagnosis of LAD and emerging treatment modalities in order to assess their utility in the management of this disease.…”

Section: Introductionmentioning

confidence: 99%

Updates in the Diagnosis and Management of Linear IgA Disease: A Systematic Review

2021

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.