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Complement abnormality in Melkersson–Rosenthal

Melkersson–Rosenthal syndrome (MRS) is a disorder of unknown etiology characterized by one or more of the following: episodic lip swelling which progresses to chronicity, facial swelling, intermittent facial nerve palsy, and fissured tongue. Granulomatous infiltration is seen on histopathologic examination of lip tissue (1, 2). The first case of MRS with a complement abnormality is reported below. A 6-year-old Caucasian boy presented with a history of hives as an infant and toddler, and three episodes of urticaria over the preceding 3 years. He was again seen at 13 years of age with the complaint of 6 months of severe lip swelling and additional history of anaphylaxis at 2 and 7 years of age, eczema, and penicillin allergy manifest by angioedema. Onphysical examination, therewas severe cheilitis with local angioedema, patches of eczema and lop ears having poorly developed antihelical folds. The cranial nerves were intact and the tongue appearednormal. Pertinent laboratory results were a total eosinophil count of 480 per mm (normal 68%). A lip biopsy showed granulomas with multinucleate giant cells. Stains for fungi and acid-fast bacilli were negative. This case is compatible with the monosymptomatic form of MRS, which is also termed cheilitis granulomatosa Miescher (3). Other diseases in the differential diagnosis of granulomatous cheilitis seem unlikely in this patient: sarcoidosis because of the normal angiotensin-converting enzyme level and absence of pulmonary, lymphatic, ocular and joint findings and Crohns disease because of the absence of other gastrointestinal signs or symptoms. The patient also has an allergic diathesis. The complement studies revealed a low C1q and a C4 at the lowest limits of normal. Total complement, C2, C3 and C1 INH were all normal, as has been reported previously in MRS (4). The pathogenesis of the complement abnormality reported here has not been explored, however it could simply be an increased utilization through the classical pathway. If this be the case, then an ongoing immunologic reaction may be a feature of MRS and this would be worth evaluating in additional cases.