A 6-year-old boy presented with a year-long history of recurrent itchy vesicles and bullae over the trunk and extremities. The lesions used to rupture spontaneously forming erosions and eventually crust. On examination, multiple, grouped vesicles and bullae, arranged in a manner depicting the characteristic “string of pearls” or “cluster of jewels” appearance of Chronic bullous disease of childhood (CBDC) [Figures 1 and 2]. The skin biopsy for histopathology and Direct Immunofluorescence (DIF) further confirmed the diagnosis revealing sub-epidermal bullae with predominantly neutrophilic infiltrate and linear IgA deposition along with C3 below the basement membrane respectively [Figure 3a and b]. He was managed with dapsone 0.5 mg/kg/day and topicals to which he responded well in 8 weeks.[1,2]
Figure 1.
Multiple, grouped vesicles and a few bullae in a “string of pearls” or “cluster of jewels” arrangement on the back
Figure 2.
Multiple bullae and a few vesicles depicting the “string of pearls” or “cluster of jewels” appearance of CBDC on the left upper limb
Figure 3.
(a) Sub-epidermal separation with a predominantly neutrophilic infiltrate in the dermis (H and E, 40×). (b) Direct immunofluorescence shows linear deposition of IgA along the sub-epidermal layer
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References
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