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Year : 2012  |  Volume : 57  |  Issue : 5  |  Page : 416-417
Chronic Bullous Disease Of Childhood with IgG Predominance: What is the locus standi?

Department of Dermatology, Kasturba Medical College, Manipal, India

Date of Web Publication3-Sep-2012

Correspondence Address:
Raghavendra Rao
Department of Dermatology, Kasturba Medical College, Manipal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-5154.100505

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How to cite this article:
Petrolwala SM, Rao R. Chronic Bullous Disease Of Childhood with IgG Predominance: What is the locus standi?. Indian J Dermatol 2012;57:416-7

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Petrolwala SM, Rao R. Chronic Bullous Disease Of Childhood with IgG Predominance: What is the locus standi?. Indian J Dermatol [serial online] 2012 [cited 2021 Dec 4];57:416-7. Available from:


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 term 'chronic' is best avoided as the disease in children is usually self-remitting and subsides within 3-6 years. Wojnarowska et al reported that 64% of patients remitted, mostly within 2 years. [5] Labeling this potentially self- limiting condition as 'chronic' may have profound psychological implication on the patients and their parents. Also, LAD is the only disease in dermatology, which is named after an investigation (or its finding), and it should maintain 'status quo' position.

Petersen et al[6] (reference 5 in the article by Haneef et al) reported a case of 76-year-man who had pruritic vesiculobullous eruption and had initial predominant IgG deposition; he was initially diagnosed as a case of 'bullous pemphigoid (BP)' and not as 'LAD' as claimed by the authors. Subsequently, he showed both clinical and immunopathological features of LAD (i.e., three years later).

We feel that additional investigations should have been done in this interesting case before arriving at the diagnosis. Chan et al studied four adult patients of LAD who had dual IgA and IgG antibody response and found out by immunoblotting test that both IgA and IgG bound to 97 kDa ('LAD antigen'). [4] The presence of both IgG and IgA are explained by a complex immunologic process involving both T and B lymphocytes. Though antibodies are produced by B lymphocytes after transforming into plasma cells, it is the T cells that direct their production process. Th2 helper cells stimulate B cells via interleukin-5 (IL-5) to produce IgA; another Th2 cytokine IL-4 greatly enhances the IgA production. At the same time, IL-4 also stimulates B-cells to produce IgG subclass 1 (IgG1). Thus, at least in few cases, both IL-4 and IL-5 are secreted, which result in the dual antibody response. On the other hand, Th1 secretes interferon γ (INF- γ), which inhibits the effect of IL-4 , but not IL-5, thereby interfering with production of IgG1, leaving the IgA production intact, which results in isolated IgA deposition at BMZ.[4] .

   References Top

1.Haneef NS, Ramachandra S, Metta AK, Srujuna L. Chronic bullous disease of childhood with IgG predominance: What is the locus standi? Indian J Dermatol 2012;57:285-7.  Back to cited text no. 1
[PUBMED]  Medknow Journal  
2.Thappa DM, Jeevankumar B. Chronic bullous dermatosis of childhood. Postgrad Med J 2003;79:437.  Back to cited text no. 2
3.Darling TN, Cardenas AA, Beard JS, Sau P, Yee CL, Zone JJ, et al. A child with antibodies targeting both linear IgA bullous dermatosis and bullous pemphigoid antigens. Arch Dermatol 1995;131:1438-42.  Back to cited text no. 3
4.Chan LS, Traczyk T, Taylor TB, Eramo LR, Woodley DT, Zone JJ. Linear IgA bullous dermatosis. Characterisation of a subset of patients with concurrent IgA and IgG anti basement membrane autoantibodies. Arch Dermatol 1995;131:1432-7.  Back to cited text no. 4
5.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 immunological overlap. J Am Acad Dermatol 1988;19:792-805.  Back to cited text no. 5
6.Petersen MJ, Gammon WR, Briggaman RA. A case of linear IgA disease presenting initially with IgG immune deposits. J Am Acad Dermatol 1986;14:1014-9.  Back to cited text no. 6


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