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. 2012 Jul;57(4):285-7.
doi: 10.4103/0019-5154.97670.

Chronic bullous disease of childhood with IgG predominance: what is the locus standi?

Nayeem Sadath Haneef  1 , S Ramachandra, Arun Kumar Metta, L Srujana
Affiliations

Chronic bullous disease of childhood with IgG predominance: what is the locus standi?

Nayeem Sadath Haneef et al. Indian J Dermatol. 2012 Jul.

Abstract

Linear IgA disease (LAD) is an acquired, autoimmune, subepidermal, blistering disease, characterized by linear deposition of IgA along the dermoepidermal junction on immunofluorescence. Some cases known as 'mixed immunobullous disease' show weak staining with other immune reactants like IgG, IgM or C3. We report a rare case of a child having typical manifestations of LAD (chronic bullous disease of childhood), but with IgG predominance rather than IgA. Obviously it is improper to term this as linear IgA disease. Such cases are reported in literature as variants of LAD, with a multitude of terms like mixed immune bullous disease, linear IgG / IgA disease, linear IgA / IgG disease, and so on. In view of the tremendous confusion that these multiple terms cause in the absence of any practical benefit, we propose that the broad term 'chronic bullous disease of childhood' be applied to all childhood cases, irrespective of the nature of the immune deposits.

Keywords: Chronic bullous disease of childhood; linear IgA disease; linear IgG / IgA disease; mixed immunobullous disease.

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Conflict of interest statement

Conflict of Interest: Nil.

Figures

Figure 1
Figure 1
Multiple vesicles, bullae, and erosions present all over the body
Figure 2
Figure 2
Characteristic annular arrangement of vesicles / bullae around a crusted, erythematous plaque, appearing as a ‘string of pearls’ or ‘cluster of jewels’
Figure 3
Figure 3
The ‘string of pearls’ or ‘cluster of jewels’ sign, pathognomonic of chronic bullous disease of childhood
Figure 4
Figure 4
Subepidermal bulla with predominantly neutrophilic infiltration within the bulla and the dermis [H and E, 10×]
Figure 5
Figure 5
Linear deposition of IgG, IgA, and C3 along the dermoepidermal junction on direct immunofluorescence
See this image and copyright information in PMC

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