I am Dr Stephen Paget, physician-in-chief emeritus at the Hospital for Special Surgery, and professor of medicine at Weill Cornell Medical College, both in New York City.
Today I want to describe an interesting case I saw recently. A 45-year-old woman came to me with painful sclerotic lesions in her right pelvis for about 6 months. When she was 14 years of age, she developed fever, joint pains, and skin rash and was diagnosed as having an undifferentiated connective tissue disease. This was treated with a short course of steroids and some antimalarial drugs. During the next 10 or 15 years, she experienced intermittent flares for which she would take short courses of steroids.
About 2 years ago, she developed a nodular lesion in her right upper arm. The lesion was eventually biopsied and found to be a granulomatous panniculitis. The whole lesion was excised and became a non-problem. Then, around 6 or 8 months ago, she developed the onset of severe left pelvic pain; an x-ray showed sclerotic lesions, and she was told that she had cancer. She eventually went to Memorial Sloan Kettering Cancer Center in New York and physicians there biopsied the lesion. The biopsy showed lymphocytes, some mild granulomatous changes, and no signs of cancer. However, because she had a mildly elevated sedimentation rate and a small monoclonal protein, she underwent a bone marrow biopsy that showed no sign of malignancy or multiple myeloma and no monoclonal disease.
Putting the Puzzle Together
As is common, the patient was sent to me, a rheumatologist, to try to figure this out. I reviewed the x-rays. She had three or four large sclerotic lesions in her right pelvis. I knew that one of them had been biopsied and showed no signs of cancer. I asked: How do you put these all together? And when did this start? Did this start when she was 14 years old? Is this the same illness? Is there a connection between her right upper arm lesion and her right pelvic lesion?
This is how I put it together. I believed she probably had a granulomatous-type disorder, systemic and inflammatory, of a sarcoid type. I thought that because the lesion in the right upper arm showed a granulomatous panniculitis most consistent with a septal panniculitis or erythema nodosum, not an uncommon problem in the setting of sarcoidosis. I felt reasonably comfortable that the breadth of her disease ruled out infection and underlying malignancy, and I started her on prednisone 10 mg twice daily, for a total dose of 20 mg daily. If she improved and her lesions disappeared within a certain amount of time, I could say with certainty that she had a steroid-responsive problem and most likely a granulomatous inflammatory disorder. Indeed, after 1 month her 10/10 pain score dropped to zero, and on a CT scan of her pelvis, the lesions had completely cleared.
What is the take-home lesson from this? First, the issue is how you put facts together. Do facts that begin at 14 years of age connect to ones 30 years later? Certainly, she clearly did not have an infection or cancer, as defined by the length of her illness and the negative studies; thus, I was comfortable making a diagnosis of an inflammatory disorder, probably sarcoid-like, and treating her with steroids, with an excellent response.
Thank you for listening. This is Dr Stephen Paget, speaking for Medscape.
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Cite this: A Puzzling Case of Painful Sclerotic Pelvic Lesions - Medscape - Mar 03, 2015.