Jonathan Kay, MD


August 19, 2014

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Hello. I am Dr. Jonathan Kay, Professor of Medicine and Director of Clinical Research in the Division of Rheumatology at UMass Memorial Medical Center and the University of Massachusetts Medical School, both in Worcester, Massachusetts.

I would like to share with you a case that I saw recently in my clinical practice -- a very interesting and pleasant 87-year-old woman who came in with a rash and no muscle weakness. This woman experienced progressive erythema on her face, around her eyelids, shoulders, upper back, anterior chest, and on the extensor surfaces of her extremities. The clinical presentation was similar to that of dermatomyositis but without any muscle involvement. Her medical history was notable for hypertension, hyperlipidemia, and some mild degenerative arthritis, but she was a very active individual. In fact, she was extremely active and up-to-date with modern technology. She was disabled by this rash because it was pruritic and disfiguring, and it worsened when she was exposed to the sun.

She was evaluated by a dermatologist, who considered the rash to be consistent with a myopathic dermatomyositis. Her muscle enzyme levels were normal and her clinical muscle strength was also relatively normal. She had elevation of acute-phase reactants. Her antinuclear antibody titer was positive by nuclear image florescence, confirming the clinical diagnosis of dermatomyositis.

We treated her with hydroxychloroquine 200 mg orally, taken twice daily, but her rash worsened immediately upon initiating hydroxychloroquine therapy. She returned to her dermatologist who felt that the exacerbation of her skin rash was a photosensitive eruption in response to the hydroxychloroquine therapy. She stopped hydroxychloroquine, her rash worsened, and she was initiated on low-dose prednisone therapy.

Her lack of response to medical therapy suggested to me that there might be an underlying malignancy, although she felt quite well and had no focal symptoms. Her general physical examination, other than some exaggerated kyphosis of her dorsal spine owing to osteoporosis, was relatively normal. She had no abdominal tenderness, her lungs were clear, and there were no symptoms such as cough or fever. There was no palpable lymphadenopathy. An MRI scan of her chest revealed no abnormalities. An MR study of her abdomen was not interpreted as showing any abnormalities, but there was motion artifact.

Given how recalcitrant her skin disease was to medical therapy, I ordered an MRI of her pelvis to look for a pelvic malignancy. This MRI scan showed abnormalities in her mid-abdomen, with a large, 7-cm diameter mass, some lesions in her liver, and a stable cyst at the tail of her pancreas. These findings were distressing to me because this was a very nice woman and I did not want to have to evaluate her for a malignancy. However, the potential of a malignancy that might be resected or treated with chemotherapy might lead to remission of her dermatomyositis.

She is currently undergoing evaluation to obtain tissue and determine what type of mass this might be. I hope it's not a malignancy, but it probably is. Once this is determined, we can proceed with treatment, which I hope will lead to resolution not only of her cutaneous symptoms, but also to prolongation of her very productive and fulfilling life.

I'm Jonathan Kay. Thank you very much, and I look forward to seeing you again on Medscape.


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