Late-onset TNF receptor-associated periodic syndrome presenting as recurrent urticarial rash and lymph nodes

Description 

A 35-year-old Caucasian woman was referred by a general practitioner to our medical ward because she was suffering from recurrent arthralgia, urticarial rash with mild pruritus and cervical lymph nodes. There was no sign of arthritis, any fever, chills or night sweats, but she had lost 2.5 kg in 3 months. The average lengths of symptoms were more than 6 weeks.

She presented the same symptoms with cervical lymph nodes when she was 24 years old. She received doxycycline for a suspicion of Lyme disease and the evolution was favourable after 2 weeks of treatment. However, serology was not compatible with recent Lyme disease. Since then, the frequencies of the attacks were rare but occurred once a year.

The routine blood tests were normal except a slightly elevated C reactive protein at 12 mg/L (normal value <5 mg/L). Infectious serologies for Cytomegalovirus, Epstein-Barr virus, HIV, hepatitis C and B, Brucella, Bartonella henselae and...

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