We report the case of a patient with a widespread bullous skin disease and
linear deposits of IgG and C3 at the dermal-epidermal junction using direct
immunofluorescence microscopy. Indirect immunofluorescence analysis demons
trated circulating IgG autoantibodies that stained, like autoantibodies to
laminin 5 and type VII collagen, the dermal side of 1 mol L-1 NaCl-split hu
man skin. By immunoblotting dermal extracts, the patient's serum, like seru
m samples from two control patients, reacted with a 200-kDa protein. Using
immunoelectron microscopy, the serum labelled a component of the lower lami
na lucida, but not the lamina densa/sublamina densa region, distinguishing
this from the type VII collagen localization pattern. By immunofluorescence
microscopy on skin sections from patients lacking either laminin 5 (Herlit
z's epidermolysis bullosa) or type VII collagen (recessive dystrophic epide
rmolysis bullosa of Hallopeau-Siemens), the patient's serum retained reacti
vity with these test substrates. The patient's disease responded rapidly to
the use of topical corticosteroids and lesions healed without scarring or
milia formation. Our results provide strong evidence for the hypothesis tha
t the 200 kDa autoantigen is different from laminin 5 and type VII collagen
. For this new disease, we propose the designation 'anti-p200 pemphigoid'.