TNF receptor-associated periodic syndrome (TRAPS) mimicking chronic spontaneous urticaria

Abstract

Tumor necrosis factor (TNF) receptor-associated periodic syndrome (TRAPS) is a rare (about one per million) autosomal dominant autoinflammatory disease being typically characterized by recurrent fever episodes accompanied by variable gastrointestinal, musculoskeletal, neurological, lymphoid, urogenital and skin symptoms (table 1) [1-3].

We report on a 21 year old non atopic male with prediagnosed IgA deficiency, Marfan syndrome and hypothyroidism who first presented in our university allergy centre with three year history of antihistamine resistant, only slightly itching urticarial rash being accompanied by fatigue. Histology showed superficial perivascular lymphocytic dermatitis with eosinophils and intravascular neutrophil granulocytes. Routine laboratory parameters were within normal range but due to persistently elevated antistreptolysin-titers, without any focal infection detectable, he unsuccessfully received antibiotic treatment. Urticarial rash was only improved by recurrent cycles of systemic methylprednisolone. Under the diagnosis of chronic spontaneous urticaria (CSU) we then treated him with omalizumab subcutaneously (300-600mg once monthly). Symptoms initially seemed to improve during summer time but therapy was stopped after 18 months due to complete recurrence of urticarial rash and persistence of fatigue.

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