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

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.

Authors′ Reply

“…We thank you for your great interest in our article. [ 1 ] We agree with your view that immunofluorescence pattern is very important in making a diagnosis of linear IgA disease (LAD) in both children and adults. There is no problem in labeling cases with typical linear deposition of IgA at the basement membrane zone (BMZ) as LAD.…”

A Study on Cytoclinical Correlation in Immunobullous, Spongiotic, and Viral Vesicular Skin Lesions

“…A 3-year-old child with chronic bullous disease of childhood in this study had both neutrophils and eosinophils. The case reports of chronic bullous disease of childhood by Thappa et al 22 had shown only neutrophils while report by Haneef et al 23 had not shown any abnormality in Tzanck smear.…”

Chronic Bullous Disease of Childhood (Cbdc): (Case Report).

“…Haneef et al (6) stated that all pediatric cases with the typical finding of cluster of jewels sign considered as CBDC irrespective of the nature of the immune deposits, as there are cases show IgG predominance not IgA. Blister formation results from the activation of plasminogen to plasmin by keratinocytes as well as activation of neutrophils leading to the activation of promatrix metalloproteinase 9 and neutrophil elastase.…”