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Linear IgA bullous dermatosis of childhood in Infant/Neonate
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Linear IgA bullous dermatosis of childhood in Infant/Neonate

Contributors: Erin X. Wei MD, John Zampella MD, Craig N. Burkhart MD, Dean Morrell MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Linear IgA bullous disease (LABD) of childhood, also known as chronic bullous dermatosis of childhood (CBDC) and chronic bullous disease, is recognized as the childhood counterpart to linear IgA bullous dermatosis. LABD is an immunobullous dermatosis due to immunoglobulin A (IgA) autoantibodies against antigens in the basement membrane that cause subepidermal blister formation. It has clinical similarities to childhood bullous pemphigoid, and some believe them to be on a clinical spectrum of disease.

Childhood LABD usually presents around age 5 years, but it has been seen in neonates up to children aged 10 years. There is a 3:2 female-to-male predominance. This condition is usually self-limited over several months, up to 2-4 years, following a less chronic course than childhood pemphigoid, pemphigus, or dermatitis herpetiformis. While the majority of cases resolve before puberty, a small proportion of patients will have recurrences into adulthood.

Classically, the lesions appear as clusters or rings of tense bullae in the perioral and perineal regions. Lesions tend to burn or itch, with the blistering becoming less severe with time. The bullae heal with pigmentary changes and infrequently with scarring. Mucosal involvement is rarer in children than in adults.

Drug-induced LABD may be seen in childhood. Clinical presentation is typically indistinguishable from the classic idiopathic form with the exception that large erosions may be more commonly seen in the drug-induced compared with the idiopathic form. Localized or widespread macular erythema may be seen in association. A number of drugs have been associated with LABD, most commonly a variety of antibiotics (eg, trimethoprim-sulfamethoxazole, amoxicillin-clavulanate, and amoxicillin alone), NSAIDs, and cephalosporins.

Most patients with childhood LABD 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.

Codes

ICD10CM:
L13.8 – Other specified bullous disorders

SNOMEDCT:
109250009 – Chronic bullous dermatosis of childhood

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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:02/13/2020
Last Updated:03/24/2020
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Linear IgA bullous dermatosis of childhood in Infant/Neonate
Linear IgA bullous dermatosis of childhood : Fever, Oral erosions, Oral vesicles, Tense bullae, Tense vesicle
Clinical image of Linear IgA bullous dermatosis of childhood
Tense vesicles, some umbilicated and some in arcuate arrays, and secondary crusting on the arm. 
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