Hemidesmosomal protein 180 kDa BPAg2. Placenta may be the source of antigens.
Similar to bullous pemphigoid
Similar to bullous pemphigoid (refer to the Clinicopathologic
Correlation table, under the section "Bullous Pemphigoid").
Classification Variants, Mutations, and Other:
■ There is a high association with HLA-B8 and DR3, DR4 haplotypes.
■ There is a higher incidence of other autoimmune diseases (Hashimoto's, Graves, pernicious anemia) in individuals with pemphigoid gestationis.
■ Mucous membrane pemphigoid is a chronic blistering and scarring disorder involving mucosa, typically of elderly patients. Oral involvement is seen in 80%, ocular in 70%, and skin involvement in 20%. The oropharynx and nasopharynx are most commonly involved.
■ Eroded mucosal lesions are seen early, scarring is seen later (Fig. 5A). Eye involvement leads to symblepheron, where the scarred conjunctivae fuse together (Fig. 5B).
■ Mucosal-submucosal separation with submucosal fibrosis (Figs. 5C and D)
■ Blisters are uncommon, more typically, denuded submu-cosa and a strip of epithelium are present because the blister roof is sloughed (Fig. 5D)
■ Like BP, smooth not ragged blister roof
■ Submucosal infiltrates of lymphocytes, histiocytes, plasma cells, eosinophils, and neutrophils (Figs. 5E and F)
■ Neutrophils predominate over eosinophils compared with bullous pemphigoid
■ Bullous pemphigoid with mucosal involvement
■ DIF: linear IgG and/or IgA and C3 at the epidermal basement membrane zone (Fig. 5G)
■ IIF: low titer IgG and IgA antibodies that bind to the basement membrane zone
■ IIF-SSS: most patients have immunoreactants on the epidermal side of the salt-split skin (Fig. 5H); patients with antibodies to laminin have immunoreactants on the dermal side (base) of the salt-split skin.
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