Chapter 155: Chronic Bullous Disease of Childhood

Patient Story

An 8 year-old girl presented with a two-day history of blisters on her face, trunk, and extremities (Figure 155-1). She complained of moderate discomfort from the blisters, but denied itching, burning, fever, and recent illness. She was not taking any medications. Biopsy demonstrated subepidermal vesicles and a rich neutrophilic infiltrate in the dermal papillary tips. Direct immunofluorescence revealed a linear deposition of IgA along the basement membrane confirming the diagnosis of chronic bullous disease of childhood.The patient was started on systemic therapy with dapsone and her lesions quickly resolved.

Introduction

Chronic bullous disease of childhood (CBDC) is the most common autoimmune bullous disorder in children.¹ This subepidermal vesiculo-bullous disorder is characterized by linear IgA deposits at the dermal-epidermal junction. It shares the same immunopathologic mechanism with its adult counterpart, adult linear IgA dermatosis. Although there is a great deal of overlap between the adult and childhood forms of linear IgA dermatosis, CBDC classically appears before the age of 5 years old, whereas adult linear IgA disease appears after age 60 years old. CBDC is considered a benign condition because it does not increase mortality; however, it may carry significant morbidity and, therefore, warrants treatment aimed at controlling the disease. Treatment with dapsone or sulfapyridine usually results in rapid resolution of the lesions. No correlation between the severity of blistering and the chronicity of disease exists. Most patients undergo spontaneous remission within months to years of initial presentation.

Synonyms

IgA bullous dermatosis of childhood, Linear IgA dermatosis, bullous disease of childhood, or chronic bullous dermatosis of childhood.

Epidemiology

• CBDC is a rare disorder with an incidence of 1:500,000.²

• It is the most common autoimmune bullous disorder in children.¹

• Age of onset generally ranges from 1 to 11 years old with a mean of 3.5 to 4.5 years;² however, some case reports have documented neonatal onset of disease.^3,4

• All races may be affected, although the disease appears to be more common in developing countries.²

• Data on the sex predilection of CBDC is heterogeneous and appears to vary with location.⁵ Both sexes are affected, and some of the literature notes a slight female predominance.^2,6

Etiology and Pathophysiology

• The etiology and exact pathophysiology of CBDC has not yet been defined, although both humoral and cellular immune responses are implicated in the disease process. The characteristic blister formation occurs as a result of IgA antibody formation against a ...