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Chronic bullous disease of childhood in Child
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Chronic bullous disease of childhood in Child

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Contributors: John Zampella MD, Craig N. Burkhart MD, Dean Morrell MD, Susan Burgin MD
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Synopsis

Chronic bullous dermatosis of childhood (CBDC), also known as chronic bullous disease, is now recognized as the childhood counterpart to linear IgA bullous dermatosis. CBDC 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.

Onset typically occurs between the ages of 6 months and 10 years, with a 3:2 female-to-male predominance, but earlier onset has also been described. 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. Lesions tend to burn or itch, with the blistering becoming less severe with time. The bullae heal with pigmentary changes, infrequently with scarring, although there are reports of blindness and dysphagia in neonates with CBDC.

A small proportion of patients will have recurrences into adulthood.

Codes

ICD10CM:
L13.8 – Other specified bullous disorders

SNOMEDCT:
109250009 – Chronic bullous dermatosis of childhood

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Last Reviewed: 08/15/2019
Last Updated: 09/17/2019
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Chronic bullous disease of childhood in Child
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Chronic bullous disease of childhood : Fever, Oral erosions, Oral vesicles, Tense bullae, Tense vesicle
Clinical image of Chronic bullous disease of childhood
Tense vesicles, some umbilicated and some in arcuate arrays, and secondary crusting on the arm. 
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