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Linear IgA dermatosis
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Linear IgA dermatosis

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Contributors: Philip I. Song MD, Vivian Wong MD, PhD, Susan Burgin MD
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Synopsis

Linear IgA bullous dermatosis (LABD) is an 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. There are idiopathic / spontaneous and medication-induced subtypes of this disease.

A number of drugs have been associated with LABD, most commonly vancomycin. Other causative agents include phenytoin, amoxicillin, NSAIDs, lithium, diclofenac, captopril, interleukin-2, interferon gamma, furosemide, and d-penicillamine.

LABD most commonly presents in the fifth decade of life, with annular or grouped vesicles or bullae, most frequently on the extensor surfaces. Mucosal erosions (which can affect any mucosal surface) are common and may be severe and result in scarring. Pruritus and burning may be associated with the eruption. Oral findings are present in 80% of cases.

Drug-induced LABD usually occurs 1-2 weeks after starting the offending medication, and resolves within several weeks of stopping the medication. It can present with more widespread bullous involvement than non-drug-induced disease. Localized or widespread macular erythema may be seen in association.

The pediatric variant of LABD, also called chronic bullous disease of childhood, usually presents before age 5 and appears as clusters or rings of tense bullae in the perioral and perineal regions.

Codes

ICD10CM:
L13.8 – Other specified bullous disorders

SNOMEDCT:
95330001 – Linear IgA dermatosis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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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: 11/13/2017
Last Updated: 12/14/2018
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Linear IgA dermatosis
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Linear IgA dermatosis : Grouped configuration, Tense bullae, Trunk, Pruritus, Annular configuration, Vesicles, Arms, Legs
Clinical image of Linear IgA dermatosis
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