A 36 year old presents with several months of fever, weight loss, malaise, fatigue, and a new wrist drop. All of his medical problems started about 16 years ago, he says. He was diagnosed with allergic rhinitis when he was 20, eczema when he was 24, and asthma when he was 28. At age 32, he was admitted with pulmonary opacities, an eosinophilic pleural effusion, and peripheral eosinophilia. They didn't know what to make of it, diagnosed him with an asthma exacerbation, and sent him home.
His allergic rhinitis has been evaluated by an ENT and the endoscopic image is shown above. The white arrow points to the middle turbinate and the red arrow points to the nasal septum. The black arrows represent the findings. The patient also says he gets recurrent sinusitis.
Most recently, the patient complains of the skin finding shown above on his elbow. It is tender, nodular, and subcutaneous. On exam, you also note a foot drop.
Challenge: Making the diagnosis is important as cardiac complications can be serious. What do you think this is?
His asthma is poorly controlled on moderate doses of inhaled corticosteroids. He has required several courses of systemic glucocorticoids to control his symptoms. He's had multiple exacerbations requiring ED visits, hospitalizations, but no intubations.
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2 comments:
churg strauss?
Nicely done!
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Asthma III
This is Churg-Strauss syndrome, also called allergic granulomatosis and angiitis, characterized by chronic rhinosinusitis, asthma, and peripheral eosinophilia. It is a small and medium sized vasculitis. The prodromal phase presents with atopic disease, allergic rhinitis, and asthma. The eosinophilic phase presents with peripheral and visceral eosinophilia. The vasculitic phase is heralded by constitutional symptoms. Cardiac involvement includes heart failure and arrhythmias. Neurologic involvement is a peripheral mononeuritis multiplex (nerve damage in two or more nerves in separate parts of the body; here, radial nerve and sciatic or peroneal nerve). Other organ involvement includes renal failure, gastrointestinal symptoms, myalgias, and lymphadenopathy. This disease is often ANCA-positive. The first image shows nasal polyposis. The second image shows the vasculitis finding of subcutaneous nodules on the extensor surfaces of the arm.
Sources: UpToDate; knol.google.com. First image courtesy of Andrew Murr, second image courtesy Talmadge King - both UCSF faculty!
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