![]() ![]() No, in our hands the diagnosis is questioned in patients with skin rash Recurrent attacks of fever often regularly associated with sign and symptoms according to the acronym PFAPA in the absence of upper respiratory tract infection Sometimes erysipelas-like erythema, most often in the ankle regionĬolchicine (IL-1 blockade in refractory cases or colchicine side-effects)Įyes: conjunctivitis, uveitis and papilloedemaĪbdominal pain, diarrhoea, lymphadenopathy, splenomegalyĪrthralgia, headaches, aphthous stomatitis, abdominal pain Large joints: serositis, peritonitis, pleuritis and/or arthritis PFAPA is a relative common and important differential diagnosis for many infections occurring in preschool children, and every paediatrician will encounter PFAPA several times during her or his career.Įyes: conjunctivitis, periorbital oedema, pleuritis, pericarditis ![]() This review discusses the emerging field of autoinflammatory conditions, with a special emphasis on periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome. The concept was suggested in the same paper that described the genetic background of the TNF receptor-associated periodic syndrome (TRAPS), but was also linked to the identification, two years earlier, of mutations that cause familial Mediterranean fever (FMF) (Table 1) 1, 3. Autoinflammatory diseases were defined as ‘conditions characterised by seemingly unprovoked episodes of inflammation, without high-titre autoantibodies or antigen-specific T-cells' 1, 2. When Michael McDermott and Daniel Kastner coined the concept of autoinflammatory diseases in 1999, it marked a paradigm shift as it depicted an entirely new group of immunological diseases in conceptual, clinical and mechanistic terms. ![]()
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