Immunopathologic study of herpes gestationis in mother and infant

Abstract

In vivo bound C3 was demonstrated by immunofluorescence in lesions of both maternal and infant skin in an immunopathologic study of herpes gestationis. Immunoglobulins and other complement components of both classical and alternative pathways were not found. The serum was also negative for circulating antibody to basement membrane of skin. Maternal serum had a factor present that was able to deposit C3 from fresh normal serum onto the basement membrane zone of skin. Hemolytic complement titrations showed a decrease of CH50 levels in serum of both classical and alternative pathways in the mother and the classical pathway in the infant, with a decrease of C2 and C4 in both. To our knowledge, this is the first reportof a decrease in serum complements in herpes gestationis and indicates that complement may not be activated locally.

Pemphigoid Gestationis in a Third Trimester Pregnancy

“…The presence under DIF (direct immunofluorescence) of a linear deposition of complement component protein C3, with or without IgG, along the basement membrane zone of perilesional skin, is an essential component for the diagnosis of PG. Depositions of C3 and IgG are also seen in the placenta and fetal skin [8]. Although indirect immunofluorescence reveals the presence of circulating IgG autoantibodies in 20–60% of cases, a complement fixation test demonstrates the presence of these specific autoantibodies in 90% of PG patients [5, 9].…”

Immunopathology and molecular diagnosis of autoimmune bullous diseases

“…The autoimmune response in pemphigoid gestationis is directed against BP180 and, less frequently, BP230 [73–75]. The pathogenic relevance of autoantibodies to BP180 is emphasized by the fact that passive transfer of autoantibodies from mothers suffering from pemphigoid gestationis to the foetus may induce transient skin blistering in the newborn [76, 77]. Like in bullous pemphigoid, the non-collagenous 16 th A domain of BP180 has been identified as the major target of autoantibodies and is recognized by more than 80–90% of pemphigoid gestationis sera [54, 55, 78, 79].…”

Pemphigoid gestationis and intravenous immunoglobulin therapy

“…PG is a rare, subepidermal, autoimmune bullous disease that occurs during pregnancy or early postpartum period ( Katz et al, 1977 ). PG is the third most common bullous disease after pemphigus and bullous pemphigoid.…”

“…PG is characterized by protein- and tissue-bound IgG autoantibodies that bind to the NC16A domain of BP180 antigen and is located in the hemidesmosomes of the dermo-epidermal junction, which is the same autoantigen that is found in most cases of bullous pemphigoid. The diagnosis of PG is the identification of a subepidermal blister on histopathology and C3, IgA, IgG, IgM, and fibrinogen deposition in the basement membrane on DIF studies ( Intong and Murrell, 2011a , Katz et al, 1977 ).…”