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Biopsy showed osteoma cutis and dermatomyositis. Nodules and blisters on knuckles. Whom should I consult?

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Practicing since : 2006
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Hello, I have recently received 2 biopsies from my face - one indicating osteoma cutis 9 purportedly quite rare and once suggestive of dermatomyositis. Both were taken via the punch method and when I had a rash on my face. I also have had over 15 episodes of vasodilation triggered by the sun when I have blood pressure readings in the range of 60 over 42 and I begin to go into shock accompanied by tachycardia. I have muscle weakness and my right upper arm muscle is atrophying and is visibly getting smaller. I had an electromyography that indicated early recruitment in my right arm. I cannot hold my arms up long enough to blow my hair dry because of weakness - then I have to bring them down. I itch all the time. Nodules on the underside of my feet. Blisters on the top of my knuckles and hands that look like spider bites. Pustules on my arms, back, face, scalp (blisters and scales) wrist and ankle pain as well as many kinds of fluorescent lights are also a trigger for an attack.
Also have difficulty with swallowing from time to time - especially after sun exposure. Recently, my left thumb became very flaccid in the MCP joint. And most alarming I an developing devits in my nose and forehead. I live in a small town and the doctor is puzzled after all the tests. With my permission, he invoked a sun triggered attack while my husband was present and I vasodilated and had tachycardia after 7 minutes and headed into shock. The doctor had to keep me on his examining table for over an hour while I stabilized. . After sun exposure I have very painful stomach problems. The rash has a butterfly appearance on my face but the other breakout is more like a "bite" appearance on my arms, back and face. Does this constellation of symptoms indicate anything to you. Any help would be appreciated as he says whatever this is is life threatening to me and now I have epipens at all times. I am going to go to the city and he is trying to find the right specialist but is sure to whom I should be referred.
Posted Tue, 30 Apr 2013 in Skin Hair and Nails
Answered by Dr. Kalpana Pathak 33 minutes later

Thanks for writing to us.

The constellation of features mentioned. Proximal muscle weakness( difficulty in raising arm,blow drying hair), Difficulty in swallowing, Photosensitivity with typical red rashes over face, knuckles, hands
Nodules in feet( calcinosis), points towards an autoimmune disease dermatomyositis. In Dermatomyositis we get calcinosis or calcification in skin that is why one of your skin biopsies diagnosed it as osteoma cutis( which is one of the differential diagnosis for calcinosis).

As you are having cardiac (heart ) symptoms too, I would advise that you XXXXXXX a rheumatologist at the earliest to begin with the specific treatment.

Hope that answers your query.
Wish you a speedy recovery.
Above answer was peer-reviewed by
Follow-up: Biopsy showed osteoma cutis and dermatomyositis. Nodules and blisters on knuckles. Whom should I consult? 49 minutes later
Thank you. I have seen a rheumatologist and he said that all my tests were normal except for an XXXXXXX of 80 speckled. Will this disease not show in rheu. testing?

Thank you very much. From all you see would you believe this is more dermato. than mast cell disease or can they co-exist?

Thank you again.
Answered by Dr. Kalpana Pathak 21 hours later
I apologise for the delay from my side.
Raised XXXXXXX titre points towards an autoimmune disease( SLE, Dermatomyositis, rheumatoid arthritis scleroderma etc). Low XXXXXXX titres of less than 80 may be found in normal individuals too though rarely. In your case there are few points suggestive of Dermatomyositis
1) clinical presentation
2) skin biopsy
3) raised XXXXXXX
CPK is found to be raised in dermatomyositis but its not reliable ie can be normal too.
To confirm dermatomyositis other specific antibody tests like anti Jo1 and anti Mi 2 can be done. Though the positive tests confirm dermatomyositis but these are positive only in 25 percent cases. Another possibility to be ruled out in your case should be SLE as it has overlapping features with dermatomyositis with raised XXXXXXX in speckled pattern.
The clinical symptoms are not suggestive of mastocytosis in your case.
I would definitely advise a second opinion in your case from a rheumatologist.
If you are not able to get the same in your locality, you may use this site and post your query, specifically for a rheumatologist. Make it a point to attach all your available reports and images for a better advise.
Hope that helps.
Take care
Above answer was peer-reviewed by
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