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Erythromelalgia:     more detail

Small fiber neuropathy underlies erythromelalgia.(Skin Disorders): An article from: Family Practice News by Jane Salodof MacNeil, 2006-04-01 Red scrotal syndrome: a localized phenotypical expression of erythromelalgia.(CASE REPORTS)(Case study): An article from: Journal of Drugs in Dermatology by Noel Prevost, Joseph C., III English, 2007-09-01 A case of erythromelalgia: good response to treatment with gabapentin.(CASE REPORTS)(Clinical report): An article from: Journal of Drugs in Dermatology by Ali Murat Ceyhan, Ipek Gurses, et all 2010-05-01 Two cases of erythromelalgia (Mitchell) by Daniel Webster Prentiss, 1897 A case of erythromelalgia,: With microscopical examination of the tissue from an amputated toe, (American journal of the medical sciences) by S. Weir Mitchell, 1899 The occurrence of erythromelalgia in diseases of the spinal cord: An account of ten cases by James Collier, 1898 Erythromelalgia: Report of two cases, with cure. From the clinic of Dr. Otto Lerch, professor of medical diagnosis, postgraduate department, Tulane University by Albert Emile Fossier, 1913 Hysterical rapid respiration, with cases: Peculiar form of rupial skin disease in an hysterial woman by S. Weir Mitchell, 1893

lists with details

1. The Erythromelalgia Association
   A nonprofit organization dedicated to research and education regarding thispainful disorder. Offers details about the disorder, the organization,  
   http://www.erythromelalgia.org/
   
   Extractions: Erythromelalgia (EM) is a rare condition that can cause redness and pain in all parts of the body, most frequently the feet and hands (extremities), but also in the face, ears, limbs and torso. It is characterized by intense, burning pain, severe redness (erythema), and increased skin temperature that may be episodic or almost continuous in nature. Although Erythromelalgia typically affects both sides of the body (bilateral), it may sometimes involve only one side (unilateral). In addition, the disease course may be extremely variable from case to case. For example, in some individuals, symptom onset may be gradual, with the condition potentially remaining relatively mild for years. However, in others, it may have a sudden onset, possibly spreading and becoming severe over weeks. The specific underlying cause of erythromelalgia remains unknown. However, the condition is thought to result from vasomotor abnormalities or dysfunction in the normal constriction and dilation of the diameter of certain blood vessels, leading to abnormalities of blood flow to the extremities. Erythromelalgia may be an isolated, primary condition or occur secondary to various underlying disorders.

2. THE MERCK MANUAL, Sec. 16, Ch. 212, Peripheral Vascular Disorders
   A basic description of this disorder, which causes severe burning, pain, and redness, primarily in the hands and feet.  
   http://www.merck.com/pubs/mmanual/section16/chapter212/212f.htm
   
   Extractions: This Publication Is Searchable The Merck Manual of Diagnosis and Therapy Section 16. Cardiovascular Disorders Chapter 212. Peripheral Vascular Disorders Topics [General] Peripheral Arterial Occlusion Thromboangiitis Obliterans Raynaud's Disease and Phenomenon ... Lipedema Erythromelalgia A rare syndrome of paroxysmal vasodilation with burning pain, increased skin temperature, and redness of the feet and, less often, the hands. The cause of primary erythromelalgia is unknown. Secondary erythromelalgia may occur in patients who have myeloproliferative disorders, hypertension, venous insufficiency, diabetes mellitus, SLE, RA, lichen sclerosus et atrophicus, gout, spinal cord disease, or multiple sclerosis. Diagnosis of erythromelalgia is based on the relationship of complaints to increased skin temperature. Secondary types should be differentiated from the rare primary type because correction of an underlying disorder may relieve symptoms. Differential diagnosis includes posttraumatic reflex dystrophies, shoulder-hand syndrome, causalgia, Fabry's disease, and bacterial cellulitis. Because erythromelalgia may precede a myeloproliferative disorder (even by several years), frequent blood counts may be indicated to allow early diagnosis and treatment of the latter. Attacks can be avoided or aborted by rest, elevation of the extremity, and cold applications. Therapy is not always successful. In primary erythromelalgia, aspirin 650 mg 1 to 4 times/day may provide prompt, prolonged relief. Avoiding factors that produce vasodilation is usually helpful, and vasoconstrictors (eg, ephedrine 25 mg po, propranolol 10 to 40 mg po qid, methysergide 1 mg po q 4 h) may also provide relief. In secondary erythromelalgia, the underlying disease should be treated.

3. NORD - National Organization For Rare Disorders, Inc.
   Sample report, plus links to organisations. Fee required for full report.  
   http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Erythromelalg

4. The Erythromelalgia Association
   Welcome To The erythromelalgia Association. What's New? The erythromelalgia Association (TEA) is an international, allvolunteer organization  
   http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

5. Erythromelalgia Diagnosis
   Dr. Cohen further says “even mild erythromelalgia can greatly affect normalfunctioning and Raynaud s Syndrome and erythromelalgia at the same time  
   http://www.erythromelalgia.org/Diagnosis.asp
   
   Extractions: “EM results from a maldistribution of blood flow with some areas not getting enough blood and calling for more. The extra blood gets through other open vessels, including arteriovenous anastomosis, that is, the connection between the arterial and venous systems. This vicious cycle continues to worsen until the overall appearance of the skin is of too much blood flow. Vasodilating drugs sometimes work to open the shut vessels. When that happens, the vessels stop calling for more blood and the overall system may normalize or at least improve its function.” Dr. Cohen further says: “even mild erythromelalgia can greatly affect normal functioning and quality of life. Patients avoid warm weather and limit their activities to cool or air conditioned locations. Some move to cooler climates...many patients cannot wear socks or closed shoes even in winter. ” When patients are severely affected, they can be house bound because of the continually painful symptoms. Elevation of the affected limbs becomes necessary and normal life is disrupted. Several cases of bed confinement due to unrelenting EM symptoms are known.

6. Erythromelalgia Treatment
   erythromelalgia Treatment. Though there is no single treatment known for EM, there are several medications that are usually helpful in controlling  
   http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

7. EMedicine - Erythromelalgia : Article By Robert J Nardino, MD
   erythromelalgia erythromelalgia is a rare disorder characterized by burningpain and warmth and redness of the extremities.  
   http://www.emedicine.com/med/topic730.htm
   
   Extractions: (advertisement) Home Specialties Resource Centers CME ... Patient Education Articles Images CME Patient Education Advanced Search Consumer Health Link to this site Back to: eMedicine Specialties Medicine, Ob/Gyn, Psychiatry, and Surgery Hematology Last Updated: March 10, 2004 Rate this Article Email to a Colleague Synonyms and related keywords: erythermalgia, myeloproliferative disorder, arteriolar fibrosis, idiopathic erythromelalgia, platelet-mediated erythromelalgia, aspirin-sensitive erythromelalgia, primary erythermalgia, secondary erythermalgia, polycythemia vera, essential thrombocytosis AUTHOR INFORMATION Section 1 of 11 Author Information Introduction Clinical Differentials ... Bibliography Author: Robert J Nardino, MD , Director, Internal Medicine Residency Program, Assistant Clinical Professor, Department of Internal Medicine, Hospital of Saint Raphael, Yale University School of Medicine Coauthor(s): Andrea LM Silber, MD , Fellowship Director of Oncology, Hospital of St Raphael; Clinical Assistant Professor, Department of Medicine, Section of Hematology-Oncology, Yale University School of Medicine Robert J Nardino, MD, is a member of the following medical societies:

8. The Merck Manual Erythromelalgia
   erythromelalgia A rare syndrome of paroxysmal vasodilation with burning pain, increased skin temperature, and redness of the feet and, less often  
   http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

9. Erythromelalgia
   erythromelalgia If you have RED, BURNING, FEET or HANDS, check this page out!!!  
   http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

10. THE MERCK MANUAL--SECOND HOME EDITION, Functional Peripheral Arterial Disease In
    erythromelalgia (erythermalgia) is a rare syndrome in which arterioles of A rare hereditary form of erythromelalgia starts at birth or during childhood.  
    http://www.merck.com/mmhe/sec03/ch034/ch034c.html
    
    Extractions: var externalLinkWarning = "The link you have selected will take you to a site outside Merck and The Merck Manuals.*n*nThe Merck Manuals do not review or control the content of any non-Merck site. The Merck Manuals do not endorse and are not responsible for the accuracy, content, practices, or standards of any non-Merck sources."; Search The Second Home Edition , Online Version Search Index A B C D ... Z Sections Accidents and Injuries Blood Disorders Bone, Joint, and Muscle Disorders Brain, Spinal Cord, and Nerve Disorders ... Women's Health Issues Resources Anatomical Drawings Multimedia Pronunciations Weights and Measures ... , Online Version Section Heart and Blood Vessel Disorders Chapter Peripheral Arterial Disease Topics Introduction Functional Peripheral Arterial Disease Occlusive Peripheral Arterial Disease Functional Peripheral Arterial Disease Buy The Book Print This Topic Email This Topic Pronunciations acrocyanosis angina arterioles arthritis ... ultrasonography Functional peripheral arterial disease is much less common than occlusive peripheral arterial disease. Normally, the arteries of the arms and legs widen (dilate) and narrow (constrict) in response to changes in the environment, such as a change in temperature. Functional peripheral arterial disease usually occurs when the normal mechanisms that dilate and constrict these arteries are exaggerated. The affected arteries constrict more tightly and more often. These changes in constriction can be caused by an inherited defect in the blood vessels, by disturbances of the nerves that control the dilation and constriction of arteries (sympathetic nervous system), by injuries, or by drugs.

11. EMedicine - Erythromelalgia Article By Robert J Nardino, MD
    erythromelalgia erythromelalgia is a rare disorder characterized by burning pain and warmth and redness of the extremities. Confusion exists in the  
    http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

12. Introduction: Erythromelalgia - WrongDiagnosis.com
    Introduction to erythromelalgia as a medical condition including symptoms,diagnosis, misdiagnosis, treatment, prevention, and prognosis.  
    http://www.wrongdiagnosis.com/e/erythromelalgia/intro.htm
    
    Extractions: Researching symptoms of Erythromelalgia: Further information about the symptoms of Erythromelalgia is available including a list of symptoms of Erythromelalgia , or alternatively return to research other symptoms in the symptom center Causes of Erythromelalgia: Research more detailed information about the causes of Erythromelalgia , other possibly hidden causes of Erythromelalgia , or other general information about Erythromelalgia

13. Lessons From Erythromelalgia, Raynauds, RSD, Causalgia, Cluster
    What is erythromelalgia? erythromelalgia is a painful condition that has a very distinctive pattern of symptoms.  
    http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

14. Symptoms Of Erythromelalgia - WrongDiagnosis.com
    Symptoms of erythromelalgia including signs, symptoms, incubation period, duration,and correct diagnosis.  
    http://www.wrongdiagnosis.com/e/erythromelalgia/symptoms.htm
    
    Extractions: Feedback General information about symptoms of Erythromelalgia: The symptom information on this page attempts to provide a list of some possible symptoms of Erythromelalgia. This symptom information has been gathered from various sources, may not be fully accurate, and may not be the full list of symptoms of Erythromelalgia. Furthermore, symptoms of Erythromelalgia may vary on an individual basis for each patient. Only your doctor can provide adequate diagnosis of symptoms and whether they are indeed symptoms of Erythromelalgia. List of symptoms of Erythromelalgia: The list of symptoms mentioned in various sources for Erythromelalgia includes: More symptoms of Erythromelalgia: In addition to the above information, to get a full picture of the possible symptoms of this condition and its related conditions, it may be necessary to examine symptoms that may be caused by complications of Erythromelalgia

15. Symptoms Of Erythromelalgia - WrongDiagnosis.com
    Symptoms of erythromelalgia including signs, symptoms, incubation period, duration, and correct diagnosis.  
    http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

16. ERYTHROMELALGIA
    Calcium channel blockers worsened his erythromelalgia symptoms as noted in I suspect this is erythromelalgia (sometime called erythermelalgia) and can  
    http://dermatology.cdlib.org/rxderm-archives/erythromelalgia
    
    Extractions: ERYTHROMELALGIA - I have a 60 y.o. male with a one-year history of erythromelalgia confined to the feet. The pt. does not have an elevated platelet count. He has hypertension, controlled with beta blockers. Calcium channel blockers worsened his erythromelalgia symptoms as noted in the literature. Aspirin and other NSAIA's had no effect. Pushing beta blockers had no effect. Any suggestions for treatment? Ethan D. Nydorf, M.D. - I have had great sucess with 40% urea in aquaphor. Bob Aylesworth M.D. - It sounds like the patient might have a Trental deficiency. Haines Ely MD - I have been a silent observer for a few months and have found the discussions interesting and occasionally helpful (esp IRBD). It takes a while to become comfortable with this modality - but the potential for good is great. Yesterday, I saw an 18 year-old woman with burning tender feet for two to three weeks. She's in good general health; a college student active in dance and cheerleading, she takes no medications other than OCP. The exam showed bright red warm feet, no papules or plaques, hands were normal. No edema. I suspect this is erythromelalgia (sometime called erythermelalgia) and can only remember seeing one similar case in the distant past. Three textbooks painted different pictures - not surprising since this is realtively rare. I obtained cbc and chemistries and ua (since thrombocythemia, diabetes and a host of other disease have been associated); held off on any more esoteric studies - recommended elevation, cold compresses and ASA 650 mg qid and will see her back in a week. May use an NSAID if ASA is not helpful. Does this picture suggest any other diagnosis (we see chilblains here commonly, and this looks quite different) and has anyone treated a number of these patients - and what seems to work best? Thank you for your help. David J. Elpern Your address (Hawaii) seems to rule against cold induced diseases such as erythromelalgia. The most common cause of what you are seeing is a mixed corynebacterial infection in sweaty feet. The best treatment is a month or so of erythromycin, orally. Careful exam of the hands usually reveals some fingertip erythema and desquamation as well. Haines Ely Quick search lead to piroxicam rx (JAAD l991-Jan), propranolol rx, and I was wondering about Trental as a possibility? Note that it has been associated with SLE and as a sign of internal malignancy- Diane Thaler Haines, would you say keratolysis exfoliativa is corynebacterium as well? What do you use in people who cannot tolerate erythro? And why do some people get it only in the late Fall through Winter-at least here in cold country. If David's patient does have erthromelalgia, what do you think of Trental rx? Diane Thaler No suggestion for other dxs. However, an elderly woman (already on aspirin) complained of burning feet. Exam showed increased erythema otherwise normal. KOH was neg. Tried Zostrix cream and warned of burning sensation with application. Patient thrilled!! Burning of feet completely resolved within one week. Currently tapering, so do not know if "cured" or controlled by Zostrix cream. Mary K. Cullen I suggest "neuropeptidogenic acral dermatitis" as described in the International Journal of Dermatology by Italian Dermatologists several years ago. Zostrix was very useful. Robert I. Rudolph, M.D., FACP There is a condition called symmetric lividities of the feet. I'm sure it was described in the JAAD about 1987. I've seen it several times usually in young males who are very active in sports. Your patient may have this condition. James G. Rothschild The proper name is acral neuropeptidogenic dysesthesia. Robert I. Rudolph, M.D., FACP If ASA not helpful, would consider something like Zyrtec to treat this as possible pressure urticaria(?from the dancing and cheerleading?) Hal Rehbein Excerpt from Rook's Textbook of Dermatology on CD-Rom "Diffuse erythematous eruptions/Scarlatiniform eruptions Scarlatiniform eruptions (Fig 41-8 . The known causes include the exotoxins of the haemolytic streptococcus and staphylococcus causing scarlet fever, and certain drugs (Chapters 23 and 74). The onset is usually acute and the eruption may be generalized or it may be restricted to localized areas, e.g. the palms and soles. There is a diffuse erythema which may be associated with a burning sensation in the skin and sometimes some itching. There may be fever and variable systemic symptoms. Spontaneous resolution usually occurs in 10-21 days and is often followed by desquamation. There may also be shedding of nails and sometimes of hair. In those cases where no cause is detected recurrences are not infrequent. The presence of an exanthem helps to distinguish scarlet fever from other scarlatiniform eruptions but is not completely reliable. Intradermal injections of a specific streptococcal antitoxin will cause blanching of the erythema in scarlet fever (Schultz-Charlton test), a test now very seldom used." Erythermalgia occurs in short attacks precipitated by heat which would seem to exclude it from the diagnosis Philip Bekhor Diane, I'm writing the next in the series of my Cutis articles on this very topic. Keratosis exfoliativa is corynebacterial, but it comes on in October and May more than any other months. SO deos herpses zoster. I have pondered this timing for 20 years. WHy? Maybe som one smart on the list knows the answer. As to erythromelalgia: I like Trental, DIltiazem, and a baby aspirin in combination. Of course, keeping warm is the best bet. Haines Ely I have treated several patients with erythromelalgia. Assuming a negative systemic workup, I have had success with combination therapy using beta blockers, trental and tricyclic antidepressants. This in patients who have failed aspirin therapy. Ethan D. Nydorf, M.D. I'd like to thank those who gave me advice on the young woman with erythromelalgia. I saw her back yesterday (after five days) and to my surprise she is symptom free on ASA 650 mgs qid. Feet no longer hot, slight erythema. Labs normal. I'm surprised this was so simple, but improvement may have nothing to do with medications. My second choice based on advice and readings would have been to add periactin. The suggestion of a localized scarletiniform eruption was interesting, and reaction to bacterial exotoxin similar to that seen with TSS and SSSS should have been considered. What I learned: 1) It's erythermalgia not erythromalgia 2) There's primary erythromelalgia, secondary erythromelalgia and erythermalgia. Much more complicated than I thought. Medline had 48 references in the past 3 years. David J. Elpern Erythromelalgia/erythermalgia Caper: In the Lancet, 1990: Vol 336, pp 183-4, JJ Michiels made the following observation: "The erythromelalgia patient finds: warmth intensifies discomfort a single dose of aspirin gives complete relief for several days the patient has a chronic myoproliferative disease with thrombocytopenia. platelet clumping leads to thrombi in the small vessels with resultant acrocyanosis and finally necrosis. "The erythermalgia patient experiences the same redness and burning pain of the extremities brought on by heat or exercise, but by contrast finds: no response to aspirin no progression to necrosis" The conclusion: "Watch your vowels or you'll get the diagnosis wrong. " See also JAAD 1989, Vol 21, pp 1128-30 on Idiopathic erythermalgia. Jerry Litt

17. Erythromelalgia - New Treatments, January 22, 2005
    New Treatments for erythromelalgia, January 22, 2005  
    http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

18. The Use Of Regional Anaesthetic Blockade In A Child With Recurrent
    The use of regional anaesthetic blockade in a child with recurrent erythromelalgia C M Harrison1 , J M Goddard2 and C D Rittey1  
    http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

19. Erythromelalgia
    erythromelalgia If you have RED, BURNING, FEET or HANDS, check this page out!!!  
    http://www.homestead.com/lareisaplaza/erythromelalgia.html

20. The Use Of Regional Anaesthetic Blockade In A Child With Recurrent
    The use of regional anaesthetic blockade in a child with recurrent erythromelalgia C M Harrison1 , J M Goddard2 and C D Rittey1  
    http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

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