Melkersson-Rosenthal syndrome: a form of pseudoangioedema.

Background: Melkersson-Rosenthal syndrome is an unusual cause of facial swelling that can be confused with angioedema.

Objective: To describe a young woman with facial swelling initially considered to be angioedema.

Methods: A biopsy specimen of the eyelid demonstrated findings consistent with Melkersson-Rosenthal syndrome.

Results: After reviewing the differential diagnosis of pseudoangioedema, a presumptive diagnosis of Melkersson-Rosenthal syndrome was made. The patient was successfully treated with infliximab for Melkersson-Rosenthal syndrome. Owing to medication adverse effects, infliximab treatment was discontinued. Treatment was then continued with adalimumab, with good effect and without adverse events.

Conclusions: We report the case of a patient with Melkersson-Rosenthal syndrome presenting as angioedema. Furthermore, we report the first successful treatment of Melkersson-Rosenthal syndrome with adalimumab.