Immunopathology of epidermolysis bullosa acquisita. Association with mixed cryoglobulinemia

Abstract

We describe a case of epidermolysis bullosa acquisita with immunoglobulin and complement demonstrated at the dermal-epidermal junction. Essential mixed cryoglobulinemia was also present in the patient, reemphasizing the association of epidermolysis bullosa acquisita with other diseases.

Epidermolysis bullosa acquisita with pulmonary tuberculosis: a case report

“…The EBA-associated diseases occur with a compromised immune system and, Dahl (1979) points out, in many circulating immune complexes have been found. These diseases include diabetes mellitus (Wilson et al, 1980), chronic thyroiditis (Kushniruk, 1973), systemic lupus erythematosus (Dotsun et al, 1981), mixed cryoglobulinaemia (Krivo & Miller, 1978), and undifferentiated lymphoma (Roenigk et al, 1971;Wilson et al, 1980). With such a high incidence of underlying disease, it is important that any patient in whom EBA is diagnosed be thoroughly evaluated both clinically and by investigation in an effort to identify such a disease.…”

Epidermolysis bullosa acquisita: What’s new?

“…[53][54][55] EBA associated with systemic diseases have been also often reported, including rheumatoid arthritis and diabetes mellitus, as well as cryoglobulinemia and psoriasis. [56][57][58][59][60] An association between EBA and inflammatory bowel disease (IBD) has been extensively documented; in particular, Crohn's disease has been described in approximately 30% of EBA patients. [61][62][63][64][65] However, to date, their relevance for the pathogenesis of both IBD and EBA is still unclear.…”

Autoimmunity against type VII collagen in inflammatory bowel disease

“…Autoantibodies against type VII collagen are also responsible for the skin blistering in a subgroup of patients with systemic lupus erythematosus [10–19]. Occasionally, EBA is associated with further autoimmune diseases, including rheumatoid arthritis and diabetes mellitus [20], as well as cryoglobulinemia [21] and psoriasis [22–24].…”