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Linear IgA bullous dermatosis
Other Resources UpToDate PubMed

Linear IgA bullous dermatosis

Contributors: Erin X. Wei MD, Philip I. Song MD, Vivian Wong MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

This summary discusses adult patients. Linear IgA bullous dermatosis of childhood is addressed separately.

Linear IgA bullous dermatosis (LABD) is a rare immune-mediated disorder that occurs in both children and adults. The disease is defined by the presence of a homogenous deposition of IgA in a linear band at the epidermal-dermal junction. The 3 main types of LABD include the drug-induced, pediatric, and adult forms of the disease.

In the adult form, LABD most commonly presents in the fifth decade of life. Clinical presentation is highly variable, ranging from urticarial plaques, papules, and papulovesicles to large erosions, and the rash can be focal or generalized. Mucosal erosions (which can affect any mucosal surface) are common and may be severe and result in scarring. LABD may be associated with ulcerative colitis and, rarely, with hematologic and solid organ malignancies.

The drug-induced LABD variant usually occurs 1-4 weeks after starting the offending medication and resolves within several weeks of stopping the medication. It resembles classic LABD clinically. It can present with more widespread bullous involvement than non-drug-induced disease. Localized or widespread macular erythema may be seen in association. Localized, morbilliform, erythema multiforme-like with targetoid lesions, and Stevens-Johnson-like presentations have also been described. A number of drugs have been associated with LABD, most commonly vancomycin. Other causative agents include a variety of antibiotics (eg, trimethoprim-sulfamethoxazole, amoxicillin), NSAIDs, simvastatin, cyclosporine, furosemide, lithium, captopril, phenytoin, interleukin-2, interferon gamma, and amiodarone. IgA nephropathy can be rarely associated with the cutaneous eruption of drug-induced LABD.

Most patients with LABD (of any type) have IgA1 antibodies to a 97-kDa and a 120-kDa fragment of the extracellular portion of bullous pemphigoid antigen 2 (BP180 / type XVII collagen), typically the 15 collagenous domain and less frequently the NC16A epitope. Reactivity to collagen VII, laminin-332, and laminin gamma-1 have also been described. Collagen VII has been shown to be the antigen target in a small group of patients with vancomycin-induced LABD.

Codes

ICD10CM:
L13.8 – Other specified bullous disorders

SNOMEDCT:
95330001 – Linear immunoglobulin A dermatosis dermatosis

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed:02/10/2020
Last Updated:03/08/2020
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Linear IgA bullous dermatosis
Linear IgA bullous dermatosis : Bullae, Oral erosions, vancomycin, Pruritus, Urticarial plaque, Annular configuration, Tense vesicles
Clinical image of Linear IgA bullous dermatosis
A close-up of pink, scaly papules and plaques with tense vesicles and crusts at their borders.
Copyright © 2021 VisualDx®. All rights reserved.