Biological therapies may help manage hard-to-treat EBA

Report shows biologicals eased symptoms of epidermolysis bullosa acquisita

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

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The use of biological anti-inflammatory therapies effectively controlled epidermolysis bullosa acquisita (EBA) in four people with the disease, a recent study reports.

“According to our clinical experience and literature data in severe EBA cases with rapid progress, biologicals should be considered promptly,” the researchers wrote. They added that the use of these therapies can help “to avoid complications, such as finger contractures or narrowing of the esophagus, as well as to improve patients’ quality of life.”

The study, “Case Report: Biological treatment of epidermolysis bullosa acquisita: report on four cases and literature review,” was published in Frontiers in Immunology.

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This case study illustration shows two healthcare providers holding a giant magnifying glass in front of a patient.

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EBA is an autoimmune disorder caused by antibodies that target type VII collagen, a protein that lends structural support to skin tissue. This autoimmune attack leads to disease symptoms like skin fragility and blistering.

Treatment of EBA generally focuses on anti-inflammatory therapies that can dampen the disease-driving autoimmune attack. Commonly used medications include corticosteroids and dapsone, but many people with EBA don’t respond well to those older medications.

Newer biological therapies (medications like monoclonal antibodies that are made using living cells) used in a range of immune-mediated inflammatory diseases, have been tried in severe EBA cases, Here, researchers in Poland reported on outcomes for four EBA patients — two females and two males —  treated with biological therapies.

Therapies included rituximab and IVIG

The specific biological therapies used included rituximab (sold as Rituxan and biosimilars), infliximab (sold as Remicade and biosimilars), and intravenous immunoglobulin (IVIG), a type of therapy that delivers specific antibodies purified from healthy people to restore the levels and/or help regulate immune responses in patients.

Three of the patients each were treated with one of these three therapies, while the fourth patient received IVIG first and then, when this didn’t fully control symptoms, also was given rituximab. These biological therapies were added to or followed treatment with other, more standard anti-inflammatory therapies.

Marked easing of EBA symptoms

In all four cases, although standard treatments like corticosteroids had not controlled the patients’ disease, treatment with these biological therapies led to a marked easing of symptoms. The patient given IVIG and then rituximab experienced complete disease remission, which, according to the investigators, “confirms that combined therapy with rituximab and IVIG may be more beneficial than monotherapy in exceptional cases.”

The other three patients saw a substantial reduction in symptoms like blistering following treatment with biological therapy.

Reviewing the existing scientific literature, the scientists found there have been more than 20 published cases in which rituximab was used to treat EBA. Results have been generally positive, though the researchers noted that “it is difficult to compare the effectiveness of rituximab and other biologicals in EBA across studies … due to the difference in dosing regimens and clinical subtypes and the varying severity and duration of the disease.”

For infliximab and IVIG, meanwhile, the scientists noted that study results are in line with prior observations that combination therapy with rituximab is more effective than stand-alone treatment.

The researchers highlighted a need to continue gathering data on the use of these and other biological therapies in EBA, especially as the use of these new medicines becomes more commonplace.