Mucosal linear IgA disease with esophageal involvement responsive to ustekinumab

Jimenez, Amber; Topham, Christina; Varedi, Danny; Leiferman, Kristin M.; Hull, Christopher M.

doi:10.1016/j.jdcr.2021.06.021

Cited by 3 publications

(3 citation statements)

References 10 publications

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“…13 Other biologics, including omalizumab and ustekinumab, have been successfully used to treat LABD. 14,15…”

Section: Discussionmentioning

confidence: 99%

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Adult‐onset linear IgA bullous dermatosis: a retrospective single‐center cohort study of 81 patients and literature review

Wang,

Lehman,

Todd

et al. 2024

Int J Dermatology

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BackgroundLinear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disorder that may be drug‐induced or paraneoplastic. We aim to characterize features of LABD and determine differentiating factors among idiopathic, drug‐induced, or malignancy‐associated diseases.MethodsWe conducted a single‐center retrospective chart review of adult patients with linear IgA bullous dermatosis at a large tertiary referral center and a literature review of adult linear IgA bullous dermatosis.ResultsEighty‐one patients were included in the study. Ten patients (12.3%) had comorbid malignancy and nine (11.1%) had inflammatory bowel disease. Median disease duration was significantly shorter in both drug‐induced (1.2 vs. 48.8 months; P < 0.001) and malignancy‐associated (1.7 vs. 48.8 months; P < 0.001) LABD compared with idiopathic LABD. Recurrent episodes occurred significantly more often in idiopathic LABD compared to those with drug‐induced (76.1 vs. 11.5%; P < 0.001) or malignancy‐associated disease (76.1 vs. 33.3%; P = 0.019). Time to diagnosis was significantly shorter in the drug‐induced (0.2 vs. 5.4 months; P < 0.001) and malignancy‐associated groups (0.7 vs. 5.4 months; P = 0.049) compared with idiopathic; similarly, time to improvement was significantly shorter in both drug‐induced (0.4 vs. 3.0 months; P < 0.001) and malignancy‐associated disease (1.1 vs. 3.0 months; P = 0.016). Clinical morphology was indistinguishable between groups. Limitations included retrospective data collection, data from tertiary referral centers, and limited racial and ethnic diversity.ConclusionScreening for underlying malignancy, as well as for a predisposing medication or possibly inflammatory bowel disease, may be advisable in patients with LABD, particularly when it is newly diagnosed.

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“…13 Other biologics, including omalizumab and ustekinumab, have been successfully used to treat LABD. 14,15…”

Section: Discussionmentioning

confidence: 99%

“…So far, case reports have described dupilumab's effectiveness in children 13 . Other biologics, including omalizumab and ustekinumab, have been successfully used to treat LABD 14,15 …”

Section: Discussionmentioning

confidence: 99%

Adult‐onset linear IgA bullous dermatosis: a retrospective single‐center cohort study of 81 patients and literature review

Wang,

Lehman,

Todd

et al. 2024

Int J Dermatology

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“…Ustekinumab is a monoclonal antibody that specifically binds the IL-12/23 p40 subunit. Jimenez et al [ 77 ] reported the case of successful utilization of ustekinumab in an LABD patient with a history of Crohn’s disease and common variable immune deficiency, resistant to other therapies [ 77 ]. The dose utilized was 90 mg subcutaneously every eight weeks with successful maintenance of remission at one year.…”

Section: Reviewmentioning

confidence: 99%

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

Khan¹,

Park²,

Skopit³

2023

Cureus

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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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Multiple drugs

2021

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Mucosal linear IgA disease with esophageal involvement responsive to ustekinumab

Cited by 3 publications

References 10 publications

Adult‐onset linear IgA bullous dermatosis: a retrospective single‐center cohort study of 81 patients and literature review

Adult‐onset linear IgA bullous dermatosis: a retrospective single‐center cohort study of 81 patients and literature review

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

Multiple drugs

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