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Linear IgA bullous dermatosis

Authors
Russell P Hall, III, MD
Caroline L Rao, MD
Section Editor
John J Zone, MD
Deputy Editor
Abena O Ofori, MD

INTRODUCTION

Linear IgA bullous dermatosis (LABD), also known as linear IgA disease, is a rare, idiopathic or drug-induced autoimmune blistering disease characterized by the linear deposition of IgA at the dermoepidermal junction. Although the clinical features of this disorder can be difficult to distinguish from dermatitis herpetiformis (another autoimmune blistering disease), the distinct immunopathologic findings in LABD and the absence of an associated gluten-sensitive enteropathy confirm the status of LABD as a distinct disease [1].

LABD occurs in both adults and children, and since the late 1980s, the disorder designated as chronic bullous disease of childhood has been recognized as the childhood form of LABD [2]. Children classically present with widespread annular blisters that exhibit a predilection for the lower abdomen, thighs, and groin. In adults, annular lesions are less common and the extensor extremities, face, and trunk are frequently affected. Mucosal lesions, manifesting as inflammation, erosions, or scarring, may also occur in patients with LABD.

The clinical features, diagnosis, and management of LABD in adults and children will be reviewed here. A general approach to the evaluation of patients with blistering skin lesions is reviewed separately. (See "Approach to the patient with cutaneous blisters".)

EPIDEMIOLOGY

Linear IgA bullous dermatosis (LABD) is a rare disorder. Reports of disease incidence from various countries have ranged from less than 0.5 to 2.3 cases per million individuals per year [3]. A predilection for LABD based upon ethnicity or sex has not been definitively established [3,4].

Adults frequently develop LABD in the later stages of life, with many cases occurring after the age of 60 [2,3]. In children, the disease usually presents between the ages of 6 months and 10 years; in a series of 25 affected children, the average age of onset was 4.5 years [2]. Rarely, neonates are affected [5,6].

                               

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Literature review current through: Nov 2016. | This topic last updated: Mon Apr 25 00:00:00 GMT+00:00 2016.
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