The cartilage matrix protein subdomain of type VII collagen is pathogenic for epidermolysis bullosa acquisita.

Epidermolysis bullosa acquisita (EBA) is an acquired bullous disease of the skin characterized by IgG autoantibodies against type VII (anchoring fibril) collagen. We previously defined four immunodominant antigenic epitopes within the noncollagenous 1 (NC1) domain of type VII collagen. In this study, we produced an additional recombinant fusion protein from the NC1 domain corresponding to the N-terminal 227 amino acids (residues 1 to 227), which contains homology with cartilage matrix protein (CMP).

Folliculosebaceous smooth muscle hamartoma.

Cutaneous hamartomas are a group of heterogenous benign skin conditions demonstrating epithelial and mesenchymal components in varying proportions. Folliculosebaceous (cystic) hamartomas comprise a distinct group of uncommon cutaneous tumor-like malformations. Histologically, these lesions demonstrate varying proportions of adnexal and stromal tissues.

Induction of epidermolysis bullosa acquisita in mice by passive transfer of autoantibodies from patients.

Epidermolysis bullosa acquisita (EBA) is an autoimmune sub-epidermal blistering disease characterized by autoantibodies to type VII (anchoring fibril) collagen. To date, however, direct evidence for a pathogenic role of human EBA autoantibodies has not been demonstrated. In this study, we affinity-purified anti-type VII collagen antibodies from EBA patients' sera and then injected them into adult hairless immunocompetent mice.