Melkersson–Rosenthal syndrome (MRS) is a rare disorder classically presenting with a triad of recurrent orofacial swelling, facial palsy and a fissured tongue. This case report describes a case of MRS in a patient with isolated immunoglobulin E (IgE) hypogammaglobulinaemia. The 52-year-old woman presented with puffy eyelids, lower lip swelling and right facial nerve palsy. Fissures of the tongue were also noted. On investigation she was found to have a markedly low serum IgE level. This case report clearly indicates that IgE-mediated reactions do not play a significant role in the development of MRS.
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