Mucocutaneous Lymph Node Syndrome (MLNS) in the Continental United States

Abstract

A case of mucocutaneous lymph node syndrome (MLNS) was described. The chief clinical features of MLNS include: young age; prolonged fever; conjunctivitis; red mouth and lips; red swollen palms and soles followed by peeling; generalized rash; and nonsuppurating cervical lymphadenopathy. No single feature is present in all instances. Taken together the clinical, laboratory and pathologic features seem to constitute a cohesive and unique entity. This case seems to fit within this description. Not previously described was the abdominal crisis suffered by this patient and the elevated serum IgM [immunoglobulin M] level. The similarity of this syndrome to infantile periarteritis nodosa (IPN) has been noted. Originally thought to be benign, there seems to be a small but consistent 1.7% overall mortality in the Japanese experience with MLNS. Deaths have all been due to myocardial infarction and related to coronary thromboarteritis. Coronary, renal and bronchial arteritis together with clinical features such as rash and prolonged fever are found in IPN and MLNS and underline the similarity between these 2 diseases. The small number of cases of IPN reported in the Western literature, together with its apparently high mortality, is in striking contrast to the far larger Japanese experience with MLNS and their much lower death rate. The etiology of MLNS was hypothesized.