Omalizumab therapy for bullous pemphigoid

Abstract

Background: Bullous pemphigoid (BP) responds to a variety of immunosuppressive agents and usually controls, but does not cure, the disease. Omalizumab, Food and Drug Administration-approved for asthma, selectively suppresses the activity of IgE, an important immunoglobulin in the pathogenesis of BP.

Objective: We wished to determine if systemic omalizumab would have a therapeutic effect in patients with BP.

Methods: We treated 6 patients with BP using omalizumab and followed up their disease for up to 42 months.

Results: Although variable, 5 of the 6 patients with BP received therapeutic benefit from systemic omalizumab (the sixth terminated treatment because of intercurrent illness) with less use of other immunosuppressants, inhibition of new bullae, less pruritus, and dramatic decreases in eosinophil counts. None of the patients had untoward side effects from omalizumab.

Limitations: This was an open, uncontrolled study.

Conclusions: Omalizumab neutralizes the activity of IgE in patients with BP and improves the control of their disease activity.

Keywords: IgE; autoimmunity; bullous pemphigoid; omalizumab; pruritus.

Fig 1.

Response of a patient with bullous pemphigoid (BP) after 6 omalizumab injections as monotherapy. A, Involvement of the back of a steroid-refractory patient with BP before omalizumab treatment. B, Four months after beginning omalizumab as monotherapy, the inflammatory plaques have largely resolved, leaving postinflammatory hyperpigmentation and a small number of erosions and mild, transient erythema. The patient cleared completely after a second cycle of omalizumab treatment.

Fig 2.

Patient 4: autoantibody levels, eosinophil counts, and disease severity throughout omalizumab therapy. Patient 4 was assessed during 3 separate courses of omalizumab; arrows indicate the first dose of omalizumab in her treatment cycles. Bullous pemphigoid (BP)180 and BP230 IgG (A) autoantibody levels were measured in serum samples by enzyme-linked immunosorbent assay (MBL International, Woburn, MA). Disease activity was assessed on a 4-point scale: 4.5/4.0 = generalized disease >10% total body square area with/without immunosuppressive medications; 3–5/3 0 = localized disease <10% total body square area with/without medications; 2 = remission with medications; 1 = remission without medications. Peripheral eosinophil counts (B) paralleled disease activity more closely than autoantibody levels.