We describe a 53-year-old man with a two-year history of bullous disease. He had also had stage IV
gastric cancer for 3 years. He presented with cutaneous
erythemas and
blisters, showing an annular arrangement. Histopathological examination revealed intraepidermal pustules of eosinophils and neutrophils without apparent
acantholysis. Indirect immunofluorescence (IIF) analysis showed
IgG anti-keratinocyte cell surface
antibodies. The result of IIF on rat bladder was positive.
IgG enzyme-linked
immunosorbent assays failed to detect
antibodies to either anti-desmoglein-1 (Dsg1), Dsg3, or BP180. Immunoblot analysis with normal human epidermal extract revealed
IgG reactivity with 120, 110, and 100 kDa species. Immunofluorescence analysis using COS-7 cells that expressed
desmocollin (Dsc) 1, 2, and 3 demonstrated that
IgG autoantibodies in the patient's serum reacted with all Dsc1-3. A heterogeneous
autoantibody profile including
IgG reactivity against Dsc1-3 implicated association with
cancer-related
pemphigoid, although the findings did not fulfill the diagnostic criteria of paraneoplastic
pemphigus. A review of the literature revealed that rare
autoantibodies to Dsc, most of which were
IgA class, were detected in 7 reported bullous diseases. In 5 out of 7 cases, they were combined with
autoantibodies to
bullous pemphigoid or
pemphigus vulgaris. This is the first case that has
IgG autoantibodies to all Dsc1~3.