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You are seeing a 10-year-old girl with sudden onset fever and abdominal pain of one day’s duration. You note that in her chart she has had 3 prior episodes, presenting to the ED with diffuse tenderness, guarding, and rebound; each time she was worked up for appendicitis but not found to have it, and her symptoms abated spontaneously in a few days. She has also had an episode of fever and intense chest pain. In addition to her abdominal pain, she has an erysipelas-like rash on her right ankle today. In a very tangential tribute to Thanksgiving, this disease process is more common in people from Turkey.
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December 10, 2024 at 12:24 am
D) Familial Mediterranean Fever
FMF is an autosomal recessive disease commonly found in individuals of Turkish, Armenian, Middle Eastern, and North African Jewish descent. It is characterized by recurrent attacks of fever and serositis (pericarditis, pleuritis, peritonitis, synovitis) and may include an erysipelas-like rash, typically on a lower extremity. Attacks start abruptly and last 1-3 days. Asymptomatic periods between attacks vary in length from days to months. Although flares of JIA and SLE may have fever, rash and serositis as symptoms, they usually last longer than a few days. While HSP sometimes presents with fever and can be recurrent, the skin finding is purpura rather than erysipelas, and abdominal pain is less frequently a feature. HSP symptoms also last longer than a few days. Interestingly, the incidence of HSP is much higher in patients with FMF. Job syndrome is characterized by atopic dermatitis and skin infections + recurrent sinopulmonary infections.