A rare case of Marshall syndrome

Marshall syndrome includes periodic fever، aphthus ulcer، pharyngitis and adenitis. This syndrome is known by recurrent fever and pharyngitis. General lymphadenopathy is not common. The etiology is still unknown. Disease onset is unually before the age of 5 years and generally resolves by adolescence. During acute episodes white blood cell and erythrocyte sedimentation rate are elevated. Fever lasts 3-6 days with a recurrence of symptoms every 3-8 weeks. Laboratory findings are normal until the next bout of fever. The acute febrile symptomatic episodes can be treated by single doses of prednisolone (1 to 2 mg/kg). In the past cimetidine was used for the treatment of disease. Also colchicine was used for prophylaxis of fever attack. There is controversy about tonsillectomy in the treatment of this syndrome. The disease is self limiting and usually resolved without sequelae. This case report is about a 28 year old lady with periodic fever and chills، pharyngitis and oral aphthus since 6 month prior to admission with diagnosis of Marshall syndrome. Symptoms were resolved dramatically after single dose of prednisolon. In the next attacks of fever، treatment was done with the same drug. This report is important because this syndrome is rare and due to unresponsive to current medication، its diagnosis is necessary.