Melkersson‐Rosenthal Syndrome

A 13 year‐old boy presented with a six‐month history of severe lip swelling. His past medical history was significant for eczema, multiple episodes of urticaria, and two episodes of anaphylaxis. Physical exam revealed severe cheilitis and labial angioedema. The cranial nerves were intact, and there was no evidence of a fissured tongue. A lip biopsy showed noncaseating granulomas throughout the dermis, with extension into the underlying skeletal muscle. Other features included dilated lymphatic channels, some closely approximating the granulomas, and a mild perivascular lymphoplasmacytic infiltrate. The epidermis was acanthotic with parakeratosis and focal acantholysis. Methenamine silver and acid‐fast stains were negative. A laboratory work‐up revealed an increased total eosinophil count, an increased serum IgE level, and a low complement C1q level. All other complement levels were normal. The patient also had an unremarkable chest x‐ray and a normal angiotensin converting enzyme level. This case represents the monosymptomatic form of Melkersson‐Rosenthal syndrome (MRS), also known as cheilitis granulomatosa Meischer. MRS is classically known as a triad of granulomatous cheilitis, facial nerve palsy, and fissured tongue. The onset of granulomatous cheilitis typically precedes the other symptoms, and when present alone, it is known as the monosymptomatic form of MRS.