Linear immunoglobulin (Ig) A bullous dermatosis of childhood, also known as chronic bullous disease of childhood, is a rare disease with highly characteristics skin lesions consisting of urticarial papules, vesicles, and tense blisters that usually arise in the perioral and genital regions and on the lower limbs. The lesions are sometimes circular or oval, showing a typical string of beads pattern at the periphery of emerging lesions. The image shown is from a 2-year-old girl who presented a 72-hour history of bullous lesions. The lesions mainly affected the limbs and genital region, with no mucosal involvement. Examination revealed tense vesicles and blisters containing a clear fluid, in an annular distribution with centrifugal growth, and there was the characteristic string of beads appearance, with concentric rings of vesicles, as may be observed in the photograph. Although diagnostic confirmation requires direct immunofluorescence that shows linear IgA deposits on the basement membrane and the presence of antibodies to the 97kD or 120kD fragments of bullous pemphigoid antigen 2 (BP180), this clinical image may be considered practically pathognomic (Fig. 1).

Figure 1

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