A case of linear IgA disease in a child with IgA and IgG circulating antibodies directed to BPAg2. 2007

Gianmaria Viglizzo, and Emanuele Cozzani, and Paolo Nozza, and Corrado Occella, and Aurora Parodi
Section of Dermatology, Dipartimento di Scienze Endocrinologiche e Metaboliche (DiSEM), University of Genoa, Viale Benedetto XV 7, 16132, Genoa, Italy.

A 7-year-old Caucasian girl had multiple bullae on the trunk, upper and lower limbs (Fig. 1a,b) for 10 days. The lesions were large, tense, and asymptomatic, and mucosae were not involved. Laboratory findings were all normal. Histopathology revealed a subepidermal blister containing numerous neutrophils, eosinophils, and fibrin. Direct immunofluorescence of perilesional skin disclosed linear deposition of IgA and slight linear deposits of IgM at the basement membrane zone (Fig. 2). Indirect immunofluorescence on monkey esophagus and on human salt-split skin was negative. Immunoblot assay (IB), performed with an epidermal extract, revealed IgA and IgG antibodies directed to BPAg2 antigen (Fig. 3), while it was negative when performed on dermal extract. Enzyme-linked immunosorbent serologic assay using a commercial kit (MBL, Naka-Ku Nagoya, Japan) with the noncollagenous domain (NC16A) of the BPAg2 antigen was negative for both IgA and IgG. A diagnosis of linear IgA disease (LAD) was made and a treatment with dapsone (50 mg/day) and prednisolone (30 mg/day) was initiated. One month later, lesions had cleared.

UI MeSH Term Description Entries
D007070 Immunoglobulin A Represents 15-20% of the human serum immunoglobulins, mostly as the 4-chain polymer in humans or dimer in other mammals. Secretory IgA (IMMUNOGLOBULIN A, SECRETORY) is the main immunoglobulin in secretions. IgA,IgA Antibody,IgA1,IgA2,Antibody, IgA
D007074 Immunoglobulin G The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of IgG, for example, IgG1, IgG2A, and IgG2B. Gamma Globulin, 7S,IgG,IgG Antibody,Allerglobuline,IgG(T),IgG1,IgG2,IgG2A,IgG2B,IgG3,IgG4,Immunoglobulin GT,Polyglobin,7S Gamma Globulin,Antibody, IgG,GT, Immunoglobulin
D007159 Immunologic Tests Immunologic techniques involved in diagnosis. Diagnosis, Immunological,Immunodiagnosis,Diagnosis, Immunologic,Immunologic Diagnosis,Immunologic Test,Immunological Tests,Tests, Immunologic,Diagnoses, Immunologic,Diagnoses, Immunological,Immunodiagnoses,Immunologic Diagnoses,Immunological Diagnoses,Immunological Diagnosis,Immunological Test,Test, Immunologic,Test, Immunological,Tests, Immunological
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000097805 Collagen Type XVII A 220 kDa non-fibrillar collagen and basement membrane component. It is involved in healing wounds of the EPITHELIUM, CORNEAL. 180 kDa Bullous Pemphigoid Autoantigen,BP180 Antigen,BP180 Autoantigen,Bullous Pemphigoid Autoantigen, BP180,Collagen Alpha-1(XVII) Chain,Collagen XVII,Collagen, Type XVII, Alpha 1 Chain,Type XVII Collagen
D001323 Autoantibodies Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them. Autoantibody
D001324 Autoantigens Endogenous tissue constituents with the ability to interact with AUTOANTIBODIES and cause an immune response. Autoantigen,Autologous Antigen,Autologous Antigens,Self-Antigen,Self-Antigens,Antigen, Autologous,Antigens, Autologous,Self Antigen,Self Antigens
D012867 Skin The outer covering of the body that protects it from the environment. It is composed of the DERMIS and the EPIDERMIS.

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