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Chronic Bullous Disease Of Childhood with IgG Predominance: What is the locus standi?

Indian Journal of Dermatology
Medknow Publications
Publication Date
DOI: 10.4103/0019-5154.100505
  • Correspondence
  • Medicine


Sir, We read with great interest, the article ‘Chronic bullous disease of childhood with IgG predominance: What is the locus standi?’ by Haneef et al.[1] It's an interesting read, but we have certain reservations against some points raised in it and would like to address the same. The authors in their article have concluded that all the cases showing the typical clinical picture of ‘cluster of jewels’ should be included under the broad term of ‘chronic bullous disease of childhood,’ irrespective of the type of immune deposits. We strongly feel that this is a retrograde step and would undermine the importance of immunofluorescence in the diagnosis of immunobullous disorders. The ‘cluster of jewels’ or ‘string of pearl’ is a characteristic but not a pathognomonic sign of childhood linear IgA disease (LAD). None of the standard text books or article (including the one quoted by the authors)[2] mentions that it is a pathognomonic sign. We haven’t come across any studies looking at the specificity and sensitivity of this sign in the diagnosis of childhood LAD. By basing diagnosis on this important sign would mean that we could potentially under diagnose many cases of childhood LAD as this sign may not be present in all affected cases. Predominance of the type of immune deposits as recorded by direct immunofluorescence (DIF) is very subjective. It depends on many technical factors, and there may be inter-observer variation. Confirmation of the predominant immune deposition by objective criterion like indirect immunofluorescence (IIF) in serial dilutions, using salt split skin as a substrate, would have been more desirable. Circulating antibodies have been detected in 70-90% of children with LAD;[3] a previous study had suggested that possibility of detecting circulating anti-basement membrane zone (BMZ) is more when there is dual antibody response.[4] ‘Chronic bullous disease of childhood’ has been used synonymously with LAD in childhood, and the latter is the preferred terminology now. The ter

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