A String of Pearls

A
n 11-month-old girl presented with a rapidly progres-
sive, pruritic eruption. Physical examination revealed
generalized skin lesions composed of coalescing
vesicles and bullae on an erythematous base in an annular
and polycyclic arrangement (Figure, A and B). Histology of
lesional skin demonstrated subepidermal blistering and
a predominantly neutrophilic dermal infiltrate. Direct
immunofluorescence of perilesional skin revealed linear
deposition of IgA along the basement membrane zone
(Figure, C) and confirmed the diagnosis of linear IgA
disease, also known as chronic bullous disease of childhood.
Treatment was started with diamino-diphenyl sulfone
(Dapsone, Teva Pharmachemie, Haarlem, The
Netherlands); after the addition of erythromycin, all skin
lesions resolved completely.
Linear IgA disease is the most common autoimmune bul-
lous skin disease of childhood, associated with IgA antibodies
against basement membrane proteins, including collagen
VII.1,2 The characteristic annular arrangement of coalescing
vesicles, often referred to as the ‘‘string of pearls’’ or ‘‘cluster
of jewels’’ sign, should raise the suspicion of linear IgA
disease. n

Douwe Vellinga, MD
Rám Sukhai, MD, PhD
Patty Michaëla Jansen, MD, PhD
Remco van Doorn, MD, PhD
Departments of Dermatology, Pediatrics, and Pathology
Leiden University Medical Center
Leiden, The Netherlands

References

1. Wojnarowska F, Marsden RA, Bhogal B, Black MM. Chronic bullous

disease of childhood, childhood cicatricial pemphigoid, and linear IgA
disease of adults: a comparative study demonstrating clinical and immu-
nopathologic overlap. J Am Acad Dermatol 1988;19:792-805.
2. Ghohestani RF, Nicolas JF, Kanitakis J, Claudy A. Linear IgA bullous
dermatosis with IgA antibodies exclusively directed against the 180- or
230-kDa epidermal antigens. J Invest Dermatol 1997;108:854zA-8.

Figure. A, Characteristic annular pattern of tense bullae, re-

sembling a string of pearls. B, Several lesions exhibiting
multiple concentric rings. C, Direct immunofluorescence of
perilesional skin revealing a linear band of IgA along the
basement membrane zone. (Original magnification 40.)

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