IgG Antibasement Membrane Zone Antibodies Research Articles

Circulating IgA and IgE autoantibodies in antilaminin-332 mucous membrane pemphigoid

Antilaminin-332 mucous membrane pemphigoid (MMP) is a chronic autoimmune bullous disease that is often associated with internal malignancy. IgG autoantibodies against laminin-332 in patients with MMP are well documented; however, IgA and IgE autoantibodies against laminin-332 have not yet been described. To characterize IgA and IgE autoantibodies binding to laminin-332 in sera from patients with antilaminin-332 MMP. Sera and skin samples from four patients who met the following criteria were used: (i) subepidermal blistering lesions present on the mucous membranes; (ii) in vivo deposition of IgG along the epidermal basement membrane zone of sampled skin; (iii) circulating IgG antibasement membrane zone antibodies that react with the dermal side of salt-split normal human skin; and (iv) circulating IgG autoantibodies that do not show positivity against type VII collagen or 200-kDa protein (p200 antigen) in immunoblot analysis using dermal extracts. Circulating IgG/IgA/IgE class autoantibodies against laminin-332 were determined by immunoblotting. Circulating IgG autoantibodies against the gamma2, alpha3/gamma2, alpha3 and alpha3/beta3/gamma2 subunits of laminin-332 were demonstrated in sera from four patients, respectively. Serum from one of the four patients showed IgA reactivity with the alpha3/beta3/gamma2 subunits of laminin-332. Serum from one of the four patients showed IgE reactivity with the gamma2 subunit of laminin-332. The control sera failed to display IgG/IgA/IgE reactivity to laminin-332. In addition to IgG autoantibodies, circulating IgA and IgE autoantibodies against laminin-332 are detectable in a subset of patients with antilaminin-332 MMP.

Childhood Bullous Pemphigoid Successfully Treated with Diaminodiphenyl Sulfone

Bullous pemphigoid (BP) is an acquired autoimmune blistering disease which predominantly affects the elderly. It is rare in children and infants. We reported a 14-year-old girl presenting with a month history of relapsing tense bullae on the face and extremities. Histopathological examination of the lesional skin revealed a subepidermal bulla with infiltration of eosinophils, neutrophils, and lymphocytes. Direct immunofluorescence showed linear deposits of IgM and C3 at the basement membrane zone. Indirect immunofluorescence using normal human skin sections as a substrate detected IgG anti-basement membrane zone antibodies in the patient's serum and that using 1M NaCl split skin sections showed that the patient's antibodies bound to the epidermal side of the split skin. Immunoblot analysis using normal human epidermal extracts demonstrated the presence of autoantibodies against the 230-kDa BP antigen. Furthermore, the patient's serum reacted with the recombinant protein of the NC16a domain of the 180-kDa BP antigen by immunoblot analysis and enzyme-linked immunosorbent assay. Our patient showed significant improvement of the skin lesions with systemic administration of diaminodiphenyl sulfone.

Pyodermatitis-pyostomatitis vegetans with circulating autoantibodies to bullous pemphigoid antigen 230

W e describe a woman from Korea with pyodermatitis-pyostomatitis vegetans associated with ulcerative colitis. On immunofluorescence examination, she demonstrated in vivo bound and circulating IgG antibasement membrane zone antibodies. The immunoelectron microscopy and immunoblot analysis showed that the antibodies reacted with the bullous pemphigoid antigen 230. We consider that the circulating autoantibodies to the bullous pemphigoid antigen 230 in this patient were an epiphenomenon, resulting from epidermal damage induced by inflammation of pyodermatitis-pyostomatitis vegetans.

A case of generalized pustular psoriasis followed by bullous disease: and atypical case of bullous pemphigoid or a novel bullous disease?

We describe a 31-year-old Japanese woman with generalized pustular psoriasis treated with PUVA who subsequently developed a bullous disease. Throughout the disease course, there was no phase of psoriasis vulgaris. Although several reports describe coexistence of psoriasis vulgaris and bullous disease such as bullous periphigoid, coexistence of generalized pustular psoriasis without any phase of psoriasis vulgaris and bullous disease is rare. As for the bullous disease, direct immunofluorescence study showed IgG and C3 deposition along the basement membrane zone. Indirect immunofluorescence disclosed IgG antibasement membrane zone antibodies. Indirect immunofluorescence on 1 mol/l sodium chloride-split skin demonstrated linear IgG staining almost exclusively on the dermal side of the split. Western immunoblot analysis revealed that the antibody was directed to neither epidermolysis bullosa acquisita antigen nor bullous pemphigoid antigens. Considering the unusual clinical course, we suspect the possibility of a novel autoimmune blistering disease.

Localization of bullous pemphigoid antibody--an indirect immunofluorescence study of 228 cases using a split-skin technique.

We have re-examined the site of deposition of IgG anti-basement membrane zone antibodies in 228 sera from suspected cases of bullous pemphigoid (BP), using 1.0 M sodium chloride split-skin as substrate for indirect immunofluorescence. Nine sera (4%) produced fluorescence on the floor of the split suggesting sub-basal lamina antibody deposition compatible with a diagnosis of epidermolysis bullosa acquisita (EBA). This ultrastructural localization was confirmed in only three of these nine by immuno-electron microscopy. Clinical details were available on six of these nine cases, and none had clinical features suggesting EBA. We have found the split-skin immunofluorescence technique unhelpful in identifying 'missed' cases of EBA. The prevalence of EBA presenting as BP would appear to be very low indeed in the U.K.