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What Is The Cause And Treatment For Schamberg Disease?

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Posted on Thu, 3 Jul 2014
Question: I am desperate. My brother is 60 has hep c, RA, on lactolos, methadone. just off Embril for RA. about 3 weeks ago he visited a podiatrist (mistake) because his feet were very dark purple up to his ankles. we did not know what this was - his Gastro said that the blood pools like this in some people, his Ortho said that the black was a fungus...funny thing we had been asking both of them about these black feet for 2 years. so the Podiatrist gave him gamapenten - 4 pills later both of my brothers legs blew up into a huge rash - looked like celulitis. ortho gave him antibiotic and it cleared up in 10 days. a week later it came back with a vengence. now he is not only inflamed but retaining fluid - swollen. his mind seems to be going. he is foggy weak. the Gastro prescribed diuretics and they don't seem to be working. he just tried to eat and went into a hip up spasm. I am just about hysterical with fear. the gastro told us to go to a dermotologist - but they are so backed up - I am trying to get in. emailing you in the meantime. I could text you a photo. Gastro says not cellulitis because he has no fever and it is both legs. help me. what the heck do you think this is- I feel like he is dieing.
doctor
Answered by Dr. Dr. Kakkar (43 minutes later)
Brief Answer:
Upload an Image

Detailed Answer:
Hello and welcome to healthcaremagic

I am Dr. Kakkar. I have gone through your query.

I totally empathise with you.

Kindly upload a few clear digital photographs of the area of concern, so that i may be able to guide you better.

Go to the reports section and upload the Images

Regards
Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
doctor
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Follow up: Dr. Dr. Kakkar (4 hours later)
I will try to do this tomorrow and thank you -
doctor
Answered by Dr. Dr. Kakkar (59 minutes later)
Brief Answer:
upload picture

Detailed Answer:
Ok.

Take care

Regards
Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
doctor
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Follow up: Dr. Dr. Kakkar (6 hours later)
how do I upload the photos please - I have 4 of them - sorry we had a graduation party last night. my brother was here and i honestly don't know how he made it. the day was horrid. he is also taking Morphine for pain and had reduced his dosage until this "rash" occurred. he was very out of it all day but that could be due to his liver issues and hep c too. I've seen him like this before he started taking his Lactolose. so I have the photos - need to download them asap. thank you Doctor - of course I am going to try to get him in next week to a Dermatologist - but they are so backed up here. I might have a Doc that can get him in - but you never know. Please excuse my spelling. OK - I figured out how to download the photos - I sent you 4 photos. I sincerely hope you can figure this out. My brother said that when he looks on line it almost seems like the symptoms of polio. of course his immune system is shot due to taking embril for at least a year for his RA. the Ortho doc said she thought that when his feet were blackish (for almost 2 years) that is was a fungus and now this red is a manifestation of this. Gastro Doc says - no it never was a fungus but a pooling of blood. Podiatrist RX's Gabapentin and after 4 pills brother broke out in this the first time - and now again with a vengeance. help me please. what do you think this is???
doctor
Answered by Dr. Dr. Kakkar (1 hour later)
Brief Answer:
Schamburg's disease

Detailed Answer:
Hi.

Thanks for the Images.

I would keep a possibility of Schamburg's Disease.

In Schamburg's disease the lesions are mostly seen on the lower limbs and consist of pinhead-sized reddish lesions, resembling grains of cayenne-pepper that further form irregular plaques of orange or brown pigmentation (owing to deposition of hemosiderin).

The patients are usually asymptomatic; slight itching may, however, be an associated feature.

No medical intervention is of proven benefit for the treatment of Schamburg's disease.

Schamburg's disease is chronic and the lesions persist or extend with time and may clear spontaneously in a period ranging from months to years. Recurrences though are not uncommon.

Regards
Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
doctor
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Follow up: Dr. Dr. Kakkar (10 minutes later)
Wow. I'm not so sure this is correct - he is NOT asymptomatic. His legs are swollen - retaining fluid. He says they are very painful. He can hardly walk. with that input - what would be your other diagnosis? we had to use a wheelchair yesterday to get him to the graduation. i just don't think this is it. we know it is not related to the cyrosis of the liver, the doc did not think it was an reaction to the gabepentin and the Morphine combination (what do you think about that? he did break out after taking 4 of the gabepentin)I just looked it up - this sure looks like it and there was reference to hepititis also and RA....maybe my brother is just imagining he is in pain around this and it is really his RA that is flaring up do to not being able to take Embril or Humara. so there is nothing we can do about this....it just runs its course. do you have any idea why prior to this his feet and ankles were blackish/purple? I really appreciate this. don't give up on me here - this is very helpful and of course we will go to a derm when we can get in.
doctor
Answered by Dr. Dr. Kakkar (26 minutes later)
Brief Answer:
Schamburg's disease

Detailed Answer:
Hi.

Swelling at the ankles and purplish discoloration could be due to venous incompetence and venous stasis. I would suggest a color doppler, B/L lower limbs to look for his venous blood flow, lower limbs.

Moreover, in Schamburg's disease venous insufficiency might play a role.

Regards
Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
doctor
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Follow up: Dr. Dr. Kakkar (15 minutes later)
ok - we will do this color doppler - he had an ultrasound last week - is the doppler yet another diagnostic that would be valuable in addition to this? Another question - don't you think this could possibly be brought on by the Gabapentin RX? I really think it was - brother burst out with this rash 4 pills into taking Gabapentin. Site states "Hypersensitivity to an unknown causal agent"."Drug eruption". "Any suspected precipitants should be withdrawn." (which we did immediately). he still states it is painful and then of course the swelling is horrendous. his feet look like they are going to burst. the photos that I sent you were the day prior to the swelling that came on board the next day and has remained even with the diuretics that were RX'd. if I can get a photo of the swelling I'll send that to you too.
doctor
Answered by Dr. Dr. Kakkar (1 hour later)
Brief Answer:
Possibility of vasculitis should be considered

Detailed Answer:
Hi.

Taking into account that he has pronounced swelling and pain associated with the rash (though I could'nt appreciate such a pronounced swelling in the pictures you posted) and there is a temporal history to gabapentin administration, I would keep another differential of 'drug induced small vessel vasculitis' (possibly to gabapentin). Small vessel vasculitis is a close differential of schamburg's purpura.

Drug induced small vessel vasculitis presents as palpable purpura, swelling, pain and burning sensation.

Points against schamburg's purpura are pronounced swelling, sudden appearance of the rash and temporal relation to gabapentin.

Schamburg's purpura is gradual and there are usually no symptoms or leg swelling

Treatment of vascultitis is administration of a short course of an oral steroids for a week or 10 days. OTC painkillers like ibuprofen can be given for symptomatic pain relief after discussion with hid gastro.

You can post me the recent photographs.

Regards
Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
doctor
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Follow up: Dr. Dr. Kakkar (1 hour later)
Oh my! this is IT! the first time he broke out the ortho doc RX'd antibiotic AND Steroid and he cleared up almost magically in about 9 - 10 days. this time ortho doc only RX'd Antibiotic (no steroid) and he is not clearing up. The Gastro stated - ".....well, finish the Antibiotic course - it won't hurt you - but I really don't think it will help you either" Gastro was correct. He then wanted a consult with a Dermo and of course getting into a Dermo in this town is impossible. thank God I found you! I am going down this afternoon to photograph my brothers legs and feet - that are so swollen you won't believe it. I wonder if he should stop the diuretic? because it certainly is NOT reducing the swelling. I can get the Ortho to order more steroids tomorrow if you think that your second opinion is more on the mark...and believe me, it totally believe that it is right on!!! THANK YOU! (stop the diuretic?) Photos to come this afternoon. I am so grateful for your time!!

PS - if it is "drug induced small vessel vasculitis" will this continue to haunt my brother ongoing or can there be a resolution at some point?

did you receive the photos and my last long email sent a couple of minutes ago?

i guess my last long email did not go through. Correction regarding steriods: Brother NEVER took steriods to clear this up - I imagine the Ortho felt that steriods might be dangerous with his hep c and RA. Gastro sort of alluded to this too. but last time this rash cleared up with Dicloxacillin 500mg 10 day course. this time he is on a 14 day course. Rash looks better and so does swelling today. you can see the pitting were I pressed with my finger. brother is a candidate for Sovaldi for his Hep c - but he has to be clear of this 'drug induced small vessel vasculitis' first. Brother is upping his methadone to manage horrid pain. he was down to 1 per day - now up to 3 per day 10mg and this affects his thinking and ability the converse and be clear. it is heartbreaking - but the pain is so bad. even his thighs hurt. gastro RX's Spironolactone 50mg and Fuosemide 20 mg diuretics to lower swelling.
doctor
Answered by Dr. Dr. Kakkar (13 hours later)
Brief Answer:
Schamburg's purpura

Detailed Answer:
Hi.

Thanks for the pictures. I have gone through all of them

I would still stick to my original diagnosis of schamburg's purpura.

Color doppler study is to required here to study venous circulation of the lower limbs. Venous stasis could be one of the causes of edema as well as schamburg's purpura

Regards
Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
doctor
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Follow up: Dr. Dr. Kakkar (4 hours later)
But what about the sever pain and the sudden onset? This does not go along with what you said about the difference between Schamburg's purpura and 'drug induced small vessel vasculitis' (pronounced swelling, sudden appearance of the rash and temporal relation to gabapentin). Why are you now thinking Schamburgs again?
doctor
Answered by Dr. Dr. Kakkar (52 minutes later)
Brief Answer:
Schamburg's purpura

Detailed Answer:
Hi.

I am in favor of schamburg's purpura purely on the basis of appearance. Schamburg's purpura can develop in a short span of time and usually has cayenne pepper appearance, which is what it looks like here.

Small vessel vasculitis on the other hand usually has purpura, vesicles, bullae, necrosis and the lesions are painful or tender. It is a differential.

Ideally, the lesions should be biopsied and confirmed histopathologically.

I strongly suspect venous insufficiency and thus venous stasis edema as the cause of swelling, therefore I suggested a color doppler study.

Swelling could be related to his other medical conditions as well and does'nt seem to be due to the skin condition

Regards
Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
doctor
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Follow up: Dr. Dr. Kakkar (8 minutes later)
ok - this makes sense to me. can I leave this discussion open for a bit please. I think I am about finished but am fearful of letting you go just yet.
doctor
Answered by Dr. Dr. Kakkar (50 minutes later)
Brief Answer:
Schamburg's purpura

Detailed Answer:
Hi

No medical intervention is of proven benefit in schamburg's purpura.

Associated venous stasis if present should be treated by compression and prolonged leg dependency should be avoided.

Topical steroids can be tried for a few weeks, although they may fail.

PUVA is useful in Schamburg's purpura.

Finally, I must stress upon that since he is HCV positive, therefore a defect of coagulation should also be ruled out(I hope it has been already ruled out by appropriate investigations)

Regards
Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
doctor
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Follow up: Dr. Dr. Kakkar (8 minutes later)
I will check and see if a defect of coagulation has been ruled out. This consult is just amazingly helpful.
doctor
Answered by Dr. Dr. Kakkar (8 minutes later)
Brief Answer:
Thanks for asking us on healthcaremagic

Detailed Answer:
You are welcome

Regards
take care
Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
doctor
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Follow up: Dr. Dr. Kakkar (5 hours later)
i am going to close this now. I have never had such a thorough consultation - even face to face with a doctor. My father was a doctor and my mom is a nurse. they are very old and I often need support in reaching out to docs. I will use this site often. YOU DR S - are amazing, compassionate, patient and provided attention to many details. thank you from the bottom of my heart. we have an appointment with a primary tomorrow and hope for a referral to a dermo soon. but I will share your input regarding my brothers legs with the primary. I will hit the close button in a minute - but want to make sure I don't lose any of this report
doctor
Answered by Dr. Dr. Kakkar (1 hour later)
Brief Answer:
You are welcome

Detailed Answer:
Thanks for your appreciation. We are always here to help you ,anytime

If you don't have any more queries and if you are satisfied with the reply, kindly close the discussion and rate my answer.

You won't lose any of this discussion.

Regards
Take care
Note: Hope the answers resolves your concerns, however for further guidance of skin related queries consult our Dermatologist.Click here to book a consultation

Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
doctor
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Dr. Dr. Kakkar

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What Is The Cause And Treatment For Schamberg Disease?

Brief Answer: Upload an Image Detailed Answer: Hello and welcome to healthcaremagic I am Dr. Kakkar. I have gone through your query. I totally empathise with you. Kindly upload a few clear digital photographs of the area of concern, so that i may be able to guide you better. Go to the reports section and upload the Images Regards