Ann Dermatol.  2011 May;23(2):209-212. 10.5021/ad.2011.23.2.209.

A Case of Chronic Bullous Disease of Childhood That Was Reactive to the Antigen of 120 kDa (LAD-1)

• Han JH
• Yun SJ
• Kim SJ
• Lee SC
• Won YH
• Lee JB

• KMID: 2156665
• DOI: http://doi.org/10.5021/ad.2011.23.2.209

Abstract

Chronic bullous disease of childhood (CBDC) is an autoimmune blistering disease that is characterized by Immunoglobulin A (IgA) deposits at the basement membrane zone. IgA autoantibodies (aAbs) from the serum of patients with CBDC react with antigens of 97 kDa (LABD97) and 120 kDa (LAD-1), and both of which are fragments of the extracellular domain of bullous pemphigoid 180 (BP180, type XVII collagen). The CBDC sera reacts with the immunodominant NC16a domain of BP180, which is the major region recognized by IgG aAbs in patients with bullous pemphigoid. A five-year-old boy presented with multiple pruritic tense blisters on the umbilical and inguinal areas for six weeks. The direct immunofluorescence of the perilesional area demonstrated linear deposits of IgA at the basement membrane zone. Using immunoblotting and an enzyme linked immunosorbent assay (ELISA), we identified the IgA aAbs reactive to antigens with a molecular weight of 120 kDa (LAD-1), which is a fragment of the extracellular domain of BP180.

Keyword

Chronic bullous disease of childhood; LAD-1; Linear IgA bullous dermatosis

MeSH Terms

Autoantibodies
Basement Membrane
Blister
Enzyme-Linked Immunosorbent Assay
Fluorescent Antibody Technique, Direct
Humans
Immunoblotting
Immunoglobulin A
Immunoglobulin G
Linear IgA Bullous Dermatosis
Molecular Weight
Pemphigoid, Bullous
Transcutaneous Electric Nerve Stimulation
Autoantibodies
Immunoglobulin A
Immunoglobulin G

Figure

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  Fig. 1 (A) Clustering of tense bullae and erythematous scaly patches on the right inner thigh. (B) Grouped blisters on the umbilicus with erythematous erosive patches.

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  Fig. 2 Histology of the vesicles shows subepidermal blisters with a mild inflammatory cellular infiltration in the bulla and upper dermis (H&E, ×100).

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  Fig. 3 Indirect immunofluorescence using NaCl-split human foreskin as the substrate reveals binding of IgA aAbs to the epidermal side of the lamina lucida (×20).

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  Fig. 4 Immunoblotting analysis using epidermal extracts showed IgA aAbs bound to the 120 kDa antigen. M: protein molecular marker, BP: bullous pemphigoid serum, PV: pemphigus vulgaris serum, Pt: patient serum.

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