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December 2013

Anti–Laminin γ1 Pemphigoid Accompanied by Autoantibodies to Laminin α3 and γ2 Subunits of Laminin-332

Author Affiliations
  • 1Department of Dermatology, Faculty of Medicine, The University of Tokyo, Tokyo, Japan
  • 2Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, Kurume, Fukuoka, Japan
JAMA Dermatol. 2013;149(12):1437-1439. doi:10.1001/jamadermatol.2013.5358

Anti–laminin γ1 pemphigoid (ALG1P) is an autoimmune subepidermal bullous disease characterized by autoantibodies to a 200-kDa acidic noncollagenous glycoprotein of the lower lamina lucida. In contrast, anti–laminin-332 mucous membrane pemphigoid (MMP) is an autoimmune blistering disease characterized by autoantibodies to various subunits of laminin-332 of the basement membrane. We report the first case to our knowledge of ALG1P with IgG autoantibodies for 3 distinct laminins: α3 and γ2 subunits of laminin-332 and laminin γ1.

A man in his 70s was referred for tense blisters and erosions on the trunk and extremities (Figure 1A and B). No mucosal involvement was observed. He had undergone a dialysis treatment for chronic glomerulonephritis. A skin biopsy specimen taken from a bulla on the right thigh at the previous hospital demonstrated subepidermal separation with infiltration of neutrophils and eosinophils in the blister cavity and the upper dermis (Figure 1C and D). Direct immunofluorescence (IF) of the perilesional skin showed linear deposition of C3 at the basement membrane zone (BMZ). Indirect IF of healthy control human skin showed IgG anti-BMZ antibodies at a 1:20 titer (Figure 1E), which reacted with the dermal side of 1M sodium chloride–split skin (Figure 1F). In immunoblotting of healthy control human dermal extracts, IgG antibodies reacted with the 200-kDa laminin γ1 (Figure 2A). Furthermore, immunoblotting of purified human laminin-332 demonstrated IgG autoantibodies to the 145-kDa and 165-kDa α3 subunits and the 105-kDa γ2 subunit of laminin-332 (Figure 2B). Traces of the 140-kDa β3 subunit were also detected, but the band was too faint to be significant. Although treatment with oral prednisolone, 25 mg/d (0.5 mg/kg/d), alleviated the symptoms, a few blisters still appeared. The addition of colchicine to his treatment regimen, 0.5 mg/d, suppressed blisters completely.