Melkersson-Rosenthal syndrome with isolated immunoglobulin E hypogammaglobulinaemia.
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.
Department of Paediatrics, Faculty of Medicine, University of Yamanashi, Chuo, Japan. Tnakane99@aol.com
T
T
Nakane
K
K
Hatakeyama
K
K
Nakamura
M
M
Aihara
S
S
Nakazawa
D000361:Agammaglobulinemia / Q000276:immunology*; D005260:Female; D006801:Humans; D007073:Immunoglobulin E / Q000172:deficiency*; D008556:Melkersson-Rosenthal Syndrome / Q000175:diagnosis / Q000276:immunology / Q000503:physiopathology; D008875:Middle Aged
D007073:Immunoglobulin E