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What causes erythromelalgia?

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Posted on Wed, 6 May 2015
Question: Bonsoir,

je suis francophone résident au Québec Canada . J'ai été diagnostiqué érythromelalgia ... mon problème a commencé il y a 5 ans et personne n'a trouvé de solution à mon problème et je souffre beaucoup . Mes pieds deviennent très enflés et très rouges. Si je me laisse XXXXXXX pieds pendants pour plus de 10 minutes il deviennent noirs
je ne marche pratiquement plus et je passe la plupart de mon temps couché où XXXXXXX pieds étendus sur un divan.
on m'a dit qu' une personne sur 400 000 souffre de cette maladie.
J'ai consulté des médecins aux hôpitaux suivants : Sacré-Coeur de Montréal, Notre-Dame et hôpital général en médecine vasculaire, neurologie clinique anti-douleurs.
J'ai essayé plusieurs médicaments dont
Teba gabapentin naproxen acetylcisteine ketamine mexiletine bloc sympatique capssaicin oxcarbazepine 600mg trileptac
à venir : intratheca catheter

Je suis prêt à me faire soigner à l'extérieur du Canada

Bien à vous,
XXXX
1026 Neufchatel
Repentigny, Québec, Canada
J5Y 2A5
450-654-8004
doctor
Answered by Dr. Shafi Ullah Khan (39 minutes later)
Brief Answer:
English pleasr

Detailed Answer:
Thank you for asking

I am afraid the medium of language here is English to educate and counsel. Would you be darling enough to repeat th query in english please.

From the hints of previous one the neurological medications and intrathecal catheters indicate a neurological trouble and in order to help you better I need the details in english. Can you do that for me please?

Waiting for your response.

Regards
Khan
Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
doctor
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Follow up: Dr. Shafi Ullah Khan (36 hours later)
I have been diagnosticated of erythromelalgia. My problem started 5 years ago and nobody found a solution to help me and i am suffering a lot. My feet are swellen and there coming red. After 10 minutes, if I let them pending they are coming black. I am not able to walk and I am obliged to stay in bed or sitted with elongated feet. I have been told that there is a person out of 400 000 who have this problem. I went to 4 hospitals in Montréal (Québec, Canada). I have tried many médications like :
Teba gabapentin naproxen acetylcisteine ketamine mexiletine bloc sympatique capssaicin oxcarbazepine 600mg trileptac.

Nothing succed to help my case. Maybe somebody can help me.

Do you know this sickness ?

Regards,

XXXX
1026 Neufchatel
Repentigny, Québec, Canada
J5Y 2A5
450-654-8004

doctor
Answered by Dr. Shafi Ullah Khan (4 hours later)
Brief Answer:
Erythromelalgia needs a team of experts

Detailed Answer:
Thank you for getting back

Erythromelalgia is a rare disorder characterized by burning pain, warmth, and redness of the extremities. Despite the controversy regarding nomenclature and classification, a distinction is generally made between primary (idiopathic) and secondary erythromelalgia (most commonly associated with myeloproliferative disorders, neurological, hematological or vascular), as well as between early- and late-onset disease.

I want you to know that The name of this disorder is derived from three Greek words: erythros (“red”), melos (“limb”), and algos (“pain”). Till now what we know is the following causes as triggers and etiologies for erythromelalgia

Myeloproliferative disorders
Idiopathic origin
Medications
Infection
Other associated disorders
Genetic mutation
Mushroom poisoning

It is not one per 400,000. One zero is extra. According to Mayo clinic 1930 stats Every one US citizen out of 40 thousand XXXXXXX got this trouble.

If you don't have any bleeding disorder, ulcers, skin necrosis or any other extremities compromised vasculature you have nothing to worry about.

I want you to know that Treatment for erythromelalgia is primarily medical and supportive. Local measures, such as cooling or elevating the extremity, may relieve symptoms. Avoid excessive warming or dependency of the extremity. The environment should be modified so that it is not too hot.

Keep an eye on your blood cell count and consult your hematologist at slightest platelets or any other blood cells derangement.

Surgical sympathectomy has been attempted, with variable results. No specific dietary restrictions are necessary. Vigorous exercise may induce an acute episode and should therefore be avoided.


The treatment with medication for erythromelalgia depends on the cause.
1)For primary erythromelalgia, the best therapy is unknown.
2)Case reports of treatment with propranolol, epinephrine, biofeedback, sodium nitroprusside, gabapentin, and typhoid vaccine appear helpful.
3) high-dose magnesium, intravenous bupivacaine, the lidocaine patch, and lidocaine plus mexiletine.
4)Medications that affect voltage-gated sodium channels (eg, lidocaine and mexiletine) show promise.
5) Prostacyclin may provide some benefit; some patients achieve relief with gabapentin or high-dose magnesium.
6) I personally have no idea of any randomized trial of therapy for erythromelalgia. In a pilot study of 12 patients, iloprost (a synthetic prostacyclin analogue) improved symptoms.
7)compounded topical amitriptyline-ketamine has also helped in reduction of the pain.
8) In cases that are associated with other disorders, treating the original disease might improve symptoms. A recent case report described a patient with seronegative polyarthritis who developed erythromelalgia and was successfully treated with intravenous immunoglobulins.
9)For erythromelalgia related to thrombocytosis, aspirin is usually the treatment of choice. Other nonsteroidal anti-inflammatory drugs (NSAIDs) provide relief of short duration. Anagrelide may be an alternative. Other platelet-inhibiting agents (eg, ticlopidine and dipyridamole) have no effect.

Monitor for complications, response to treatment, and development of a myeloproliferative disorder because erythromelalgia often precedes the clinical appearance of polycythemia vera or essential thrombocythemia.

Nutshell it is a game of pain and multi organs need to be played by a team of rheumatologists, pain medicine experts and a hematologist.

I hope it helps. Take good care of yourself and dont forget to close the discussion please.

Regards
Khan

Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
doctor
Answered by
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Dr. Shafi Ullah Khan

General & Family Physician

Practicing since :2012

Answered : 3613 Questions

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What causes erythromelalgia?

Brief Answer: English pleasr Detailed Answer: Thank you for asking I am afraid the medium of language here is English to educate and counsel. Would you be darling enough to repeat th query in english please. From the hints of previous one the neurological medications and intrathecal catheters indicate a neurological trouble and in order to help you better I need the details in english. Can you do that for me please? Waiting for your response. Regards Khan