Pain, Papular Lesions, a Trip to Pakistan: Crack the Case

Stephen Paget, MD


August 19, 2015

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My name is Dr Stephen Paget. I'm the physician-in-chief emeritus at Hospital for Special Surgery and in the Division of Rheumatology.

I'd like to talk to you about a patient who I saw yesterday. This was a 43-year-old Pakistani woman who presented to me with significant joint pain, finger discomfort, and some cognitive issues, with approximately a 5-year history.

This is the story. Five years ago, she was visiting her family in Pakistan, and when she came back from there, she developed some diarrhea and abdominal discomfort. Since then, she has never been normal. For 3 years after she returned from her trip to Pakistan, she had continued abdominal pain, discomfort, diarrhea, and bloating. Eventually, 3 years later, she was diagnosed with Giardia infection and treated with the appropriate antibiotic medication with resolution of the gastrointestinal symptoms. However, she has never "felt well" since.

How has she not felt well? First of all, she has developed some papular lesions on her mouth, particularly her lips, and also her fingers which are quite tender. They are herpes simplex negative. She has developed enthesitis in the Achilles tendon, the elbows, and other areas. She has had severe fatigue and cognitive problems. The most important sentinel finding is that she has had three episodes of ischemic colitis spread 6 months apart over the past 2 years, each of which was self-limited and responded to intravenous antibiotics and hydration.

When I saw her yesterday, she had some enthesitis in her elbows and her feet and local tenderness. She had 4+ over 5 proximal muscle weakness in the arms with normal distal strength. She claimed to have some cognitive problems, and a recent MRI showed white matter abnormalities, which most probably relate to her underlying disease. Her laboratory tests have all been negative or normal, including antinuclear antibody, antibodies to extractable nuclear antigens, C3 and C4 complement, rheumatoid factor, anti-cyclic citrullinated peptide, etc.

When I examined her, interestingly enough, I touched the pulps of her fingertips. They were quite tender, with these same papular lesions, which to my mind was probably localized vasculitis. My diagnosis was systemic vasculitis, probably initially triggered by Giardia, which is not a common trigger, in my experience. I treated her with high doses of oral steroids, and she had significant improvement in 24 hours.

This was a long illness, probably triggered by an infection that we are not used to seeing as a trigger, in someone who developed central nervous system, joint, skin, and other organ system abnormalities that I think will respond to steroids.


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