Erytromelalgia
Objawy

Erytromelalgia (EM) to rzadkie schorzenie naczyniowo-nerwowe charakteryzujące się triadą objawów: zaczerwienieniem skóry, podwyższoną temperaturą oraz palącym bólem, najczęściej w obrębie kończyn dolnych (dotyczy około 90% pacjentów) i górnych (około 25%). Objawy mogą mieć charakter obustronny lub asymetryczny, a epizody zaostrzeń trwają od kilku minut do dni, często pojawiając się wieczorem i w nocy. Zaostrzenia wywołują czynniki takie jak ciepło (temperatura otoczenia powyżej 29°C), wysiłek fizyczny, długotrwałe stanie, stres, odwodnienie, a także pikantne potrawy i alkohol. W przebiegu choroby mogą wystąpić dodatkowe objawy, takie jak obrzęk, zmiany w poceniu się, mrowienie, a także fioletowe zabarwienie skóry między epizodami, co wskazuje na wazokonstrykcję. EM może prowadzić do poważnych powikłań, w tym owrzodzeń, martwicy, zgorzeli oraz infekcji wtórnych, szczególnie u pacjentów z zaburzeniami hematologicznymi.

Objawy Erytromelalgii

Erytromelalgia (EM), znana również jako choroba Mitchella, jest rzadkim schorzeniem naczyniowo-nerwowym charakteryzującym się triadą objawów: zaczerwienieniem, podwyższoną temperaturą skóry oraz palącym bólem, najczęściej występującym w obrębie kończyn. Objawy te mogą pojawić się nagle lub rozwijać się stopniowo na przestrzeni miesięcy, a nawet lat.12

Klasyczna triada objawów

Podstawowe objawy erytromelalgii tworzą charakterystyczną triadę:34

56

Lokalizacja objawów

Erytromelalgia najczęściej występuje w kończynach dolnych i górnych, przy czym:78

  • Stopy są dotknięte u około 90% pacjentów
  • Dłonie i palce u około 25% pacjentów
  • Rzadziej objawy mogą występować na twarzy, uszach, kolanach, klatce piersiowej, szyi czy narządach płciowych (2-3% przypadków)

910

Objawy najczęściej występują obustronnie (dotyczą obu stron ciała), choć mogą być asymetryczne. W rzadkich przypadkach mogą pojawiać się jednostronnie.1112

Dodatkowe objawy

Oprócz głównej triady objawów, u pacjentów z erytromelalgią mogą występować:1314

1516

Zaostrzenia i przebieg choroby

Charakter zaostrzeń

Charakterystyczną cechą erytromelalgii są epizody zaostrzeń (tzw. flare-ups), które:1718

  • Mogą wystąpić nagle lub rozwijać się stopniowo
  • Trwają od kilku minut do kilku dni
  • Najczęściej pojawiają się wieczorem i w nocy
  • Mogą być wywołane przez zwiększenie temperatury ciała

1920

Pacjenci opisują odczucia podczas zaostrzeń jako:21

  • Uczucie ściskania, jakby skóra była zbyt ciasna
  • Wrażenie przebywania w ogniu lub poparzenia gorącą wodą
  • Ból przypominający cięcie skóry żyletkami
  • Uczucie podobne do ciężkiego poparzenia słonecznego

22

Czynniki wyzwalające

Epizody zaostrzeń erytromelalgii najczęściej są wywoływane przez:2324

  • Ciepło – wzrost temperatury otoczenia powyżej 29°C, gorąca kąpiel, noszenie ciepłego obuwia lub skarpet
  • Wysiłek fizyczny – ćwiczenia, intensywna aktywność
  • Długotrwałe stanie lub utrzymywanie kończyn w pozycji opuszczonej
  • Stres emocjonalny lub ekscytacja
  • Odwodnienie
  • Pikantne potrawy i alkohol

2526

Progresja choroby

Erytromelalgia może przebiegać różnie u poszczególnych pacjentów:2728

  • U niektórych objawy mogą pozostać łagodne przez lata
  • U innych mogą stopniowo nasilać się i rozprzestrzeniać
  • W przypadku pierwotnej erytromelalgii objawy często rozpoczynają się w dzieciństwie i mogą pozostać stabilne lub progresować z wiekiem
  • W wtórnej erytromelalgii, związanej z innymi schorzeniami, objawy mogą poprzedzać diagnozę choroby podstawowej nawet o 2,5 roku

2930

Z wiekiem i postępem choroby, u niektórych pacjentów:31

  • Dłonie i stopy mogą być stale zaczerwienione
  • Objawy mogą rozprzestrzeniać się z dłoni na ramiona, barki i twarz
  • Objawy mogą rozprzestrzeniać się ze stóp na całe nogi

32

Powikłania i wpływ na jakość życia

Powikłania medyczne

Erytromelalgia może prowadzić do poważnych powikłań, szczególnie u pacjentów z chorobą o podłożu związanym z płytkami krwi:3334

  • Owrzodzenia skóry – mogą rozwijać się w odpowiedzi na powtarzające się zanurzanie kończyn w zimnej wodzie lub inne formy długotrwałego chłodzenia
  • Martwica (necrosis) – śmierć tkanek
  • Zgorzel (gangrene) – szczególnie w obrębie kończyn dolnych
  • Infekcje wtórne – w miejscach uszkodzenia skóry
  • Hipotermia – związana z ciągłym chłodzeniem kończyn wymaganym do kontroli objawów

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Wpływ na jakość życia

Erytromelalgia może znacząco wpływać na jakość życia pacjentów:3738

  • Ograniczenie aktywności fizycznej i społecznej
  • Trudności w wykonywaniu codziennych czynności
  • Problemy ze snem z powodu zaostrzeń występujących w nocy
  • Niemożność noszenia skarpet lub zamkniętego obuwia, nawet zimą
  • Unikanie ciepłej pogody, a nawet przeprowadzka do chłodniejszego klimatu
  • Konieczność używania wózka inwalidzkiego w ciężkich przypadkach
  • Ograniczenie normalnego funkcjonowania, nawet do stanu przykucia do domu

3940

Aspekty psychologiczne

Cierpienie związane z przewlekłym bólem i ograniczeniami może prowadzić do:4142

  • Depresji – często związanej z lękiem przed wystąpieniem epizodów
  • Zaburzeń lękowych
  • Problemów behawioralnych
  • Myśli samobójczych – w skrajnych przypadkach

4344

Różnice między pierwotną i wtórną erytromelalgią

Erytromelalgia pierwotna

Pierwotna forma erytromelalgii może występować:4546

47

Charakterystyka pierwotnej erytromelalgii:48

  • Objawy często zaczynają się w dzieciństwie (wczesny początek)
  • Napady bólu rozpoczynają się od świądu i przechodzą w silny, palący ból
  • Epizody mogą trwać od minut do dni
  • Choroba często jest oporna na leczenie i ma charakter przewlekły

4950

Erytromelalgia wtórna

Wtórna erytromelalgia rozwija się w przebiegu innych schorzeń, takich jak:5152

5354

Charakterystyka wtórnej erytromelalgii:5556

  • Objawy często są łagodniejsze niż w formie pierwotnej
  • Mogą ustąpić po prawidłowym leczeniu choroby podstawowej lub odstawieniu wywołującego leku
  • W przypadku związku z zaburzeniami mieloproliferacyjnymi, choroba często dobrze reaguje na leczenie aspiryną

5758

Trudności diagnostyczne

Diagnostyka erytromelalgii może być wyzwaniem, ponieważ:5960

  • Jest to rzadka choroba, często nieznana wielu lekarzom
  • Objawy są czasami mylone z innymi schorzeniami
  • Pacjenci często mają normalne wyniki badań między epizodami
  • Brak jednolitych kryteriów diagnostycznych
  • Duża zmienność w prezentacji klinicznej

61

Pacjenci pediatryczni z erytromelalgią spotykają się średnio z trzema specjalistami zanim otrzymają właściwą diagnozę. Choroba może pozostać niezdiagnozowana przez wiele lat, co opóźnia rozpoczęcie właściwego leczenia.6263

Prognozy dla pacjentów

Rokowanie w erytromelalgii zależy od kilku czynników:6465

  • Rodzaju erytromelalgii – pierwotna vs wtórna
  • Wieku wystąpienia – wczesny początek choroby jest zwykle trudniejszy w leczeniu
  • Odpowiedzi na leczenie – szczególnie na aspirynę w przypadku wtórnej erytromelalgii związanej z zaburzeniami mieloproliferacyjnymi
  • Występowania chorób współistniejących

66

W niektórych przypadkach:6768

  • Mogą wystąpić samoistne remisje
  • Choroba może pozostać stabilna przez wiele lat
  • Choroba może postępować z wiekiem
  • Ciężkie przypadki mogą prowadzić do znacznego upośledzenia funkcjonowania

69

Mimo że erytromelalgia może znacząco wpływać na jakość życia, rzadko jest chorobą śmiertelną. Większość pacjentów dożywa późnej dorosłości, choć jakość ich życia może być znacząco obniżona.7071

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  1. 09.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Erythromelalgia – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK557787/
    Erythromelalgia is a rarely occurring disease entity characterized by a triad of erythema, warmth, and recurrent burning pain, most notably affecting the extremities. […] The classic presentation of erythromelalgia is a triad of redness, warmth, and burning pain in the extremities that occur in episodes. Erythromelalgia most commonly affects the feet (in 90% of patients) and less commonly the fingers and hands (in 25% of patients). […] The symptoms often begin with an itching sensation of the affected area, progressing to a more severe burning pain. The involvement is typically bilateral but can be asymmetric. […] Myeloproliferative disorders like essential thrombocytosis and polycythemia vera have a median onset of approximately 2.5 years after the onset of clinical symptoms of erythromelalgia.
  • #2 Living With Erythromelalgia | FASA
    https://fasafw.com/blog/living-with-erythromelalgia/
    Erythromelalgia, also known as Mitchells disease, is a rare neurovascular condition affecting the extremities, most commonly the feet. […] An intense, burning sensation in the feet is erythromelalgias leading characteristic. Extreme redness of the skin and increased skin temperatures in affected areas are other typical symptoms. […] For most people, the symptoms are intermittent. Those living with erythromelalgia experience erratic episodes of painful, red-hot extremities. These symptoms can appear gradually, and sometimes the disease remains mild for years. […] Erythromelalgia can also have a sudden onset, with symptoms becoming severe in a matter of weeks or months. […] Look for the following erythromelalgia symptoms: Swelling, Burning pain that typically starts as itching, Sweating too much or too little, Warm or hot skin, Redness, the shade of which can vary depending on skin tone. […] An erythromelalgia flare can feel like blades cutting your skin, severe sunburn, tightness or a scald from hot water.
  • #3 Erythromelalgia: Symptoms, Causes, Treatment
    https://my.clevelandclinic.org/health/diseases/22752-erythromelalgia
    Erythromelalgia symptoms, which often include burning pain, worsen over time. […] The three primary erythromelalgia symptoms are: Increase in skin temperature, Pain that ranges from mild tingling to severe burning, Skin redness. […] Symptoms may occur on one or both sides of your body. They can range from mild to intense. Some people experience their first flare in childhood, while others aren’t affected until adulthood. […] Flares usually occur when you experience a sudden increase in body temperature. […] Symptom flares can affect your quality of life, but they are rarely fatal. Most people live well into older adulthood. […] People who have the disease say that symptom flares can feel like: A binding sensation, as if your skin is too tight, Being on fire or scalded by hot water, Razor blades cutting your skin, Severe sunburn.
  • #4 Erythromelalgia
    https://www.nhs.uk/conditions/erythromelalgia/
    Erythromelalgia usually affects the feet, but can also happen in the hands, arms, legs, ears and face. […] The main symptoms are: burning pain, which often starts as itching; redness this can look different on brown or black skin; warm or hot skin. […] Other symptoms include: swelling; sweating more than normal. […] The pain can either be constant or come and go. When the pain comes, it might be called a flare-up. A flare-up can come on gradually or suddenly, and last anywhere from a few minutes to a few days. […] Your skin may feel cold after a flare-up and look blue or grey. […] A flare-up of your symptoms is triggered by an increase in your body temperature. […] Erythromelalgia can be difficult to treat. You may need to try different combinations of treatments to find the ones that work for you.
  • #5 The Erythromelalgia Association | Information, Research, Support
    https://burningfeet.org/
    The three hallmark symptoms of EM are: […] Redness, caused by excessive blood flow to the area […] Heat, meaning that the skin will feel warm or hot to the touch, also due to increased blood flow […] Pain, which may range from mild tingling to severe burning […] Additional symptoms may include swelling caused by the buildup of fluid in the affected body part, changes in perspiration, or purple discoloration when not actively flaring. EM flares are most commonly triggered by warmth, physical activity, or stress.
  • #6 Erythromelalgia Clinical Presentation: History, Physical Examination, Complications
    https://emedicine.medscape.com/article/200071-clinical
    The classic description of erythromelalgia is a triad of redness, pain, and warmth in the extremities, brought on by warming or dependency and relieved by cooling. […] Paroxysmal burning pain has also been reported as a presenting symptom of erythromelalgia. […] Symptomatic episodes may last minutes to days. They often begin with an itching sensation and later progress to more severe pain with a burning quality. Pain may be so intense that the patient cannot walk; some must even keep their feet immersed in ice water. […] The lower extremities are affected more often than the upper extremities. The soles of feet and toes are most commonly involved. In rare cases, involvement may reach as high as the knees. Involvement is usually bilateral, though not necessarily symmetric. Warming the extremity or placing and maintaining the extremity in a dependent position can exacerbate symptoms; cooling and elevating the extremity can relieve symptoms.
  • #7 Erythromelalgia – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK557787/
    Erythromelalgia is a rarely occurring disease entity characterized by a triad of erythema, warmth, and recurrent burning pain, most notably affecting the extremities. […] The classic presentation of erythromelalgia is a triad of redness, warmth, and burning pain in the extremities that occur in episodes. Erythromelalgia most commonly affects the feet (in 90% of patients) and less commonly the fingers and hands (in 25% of patients). […] The symptoms often begin with an itching sensation of the affected area, progressing to a more severe burning pain. The involvement is typically bilateral but can be asymmetric. […] Myeloproliferative disorders like essential thrombocytosis and polycythemia vera have a median onset of approximately 2.5 years after the onset of clinical symptoms of erythromelalgia.
  • #8 Erythromelalgia: Definition, symptoms, and management
    https://www.medicalnewstoday.com/articles/erythromelalgia
    Erythromelalgia is a rare condition that causes episodes of burning pain in the extremities. It mostly affects the feet, but people can also experience symptoms in the arms, hands, ears, legs, and face. […] EM is a rare disorder that can cause episodes of pain, swelling, and severely flushed skin, or erythema, in different parts of the body. […] The main symptoms of EM include: Erythema: This redness, which is often not apparent in darker skin tones, occurs due to excessive blood flow to the area. Heat: The extra blood flowing to the affected body part can cause the skin to be warm to the touch. Pain: The type of pain can vary, and a person may experience mild tingling, a pins-and-needles sensation, itchiness, or a strong burning feeling. […] According to The Erythromelalgia Association (TEA), other symptoms can include: swelling of the affected area, sweating more or less than the person usually would, but only in the affected area, purple discoloration when they are not experiencing an episode.
  • #9 Erythromelalgia – MD Searchlight
    https://mdsearchlight.com/blood-disorders/erythromelalgia/
    Erythromelalgia is a rare condition that leads to redness, warmth, and a burning sensation, often affecting the arms and legs. Its most frequently found in the lower part of the body, namely the feet, but can also occur in the upper part such as the hands. People with this condition usually experience symptoms after physical activities and find relief when they cool down the affected parts. While erythromelalgia usually affects both sides of the body, it can occasionally affect just one side. In some very rare cases, symptoms can occur only in the face, which can often lead to misdiagnosis. […] Erythromelalgia is a rare condition where parts of the body, especially the feet, become red, warm, and experience a burning pain. This typically occurs in episodes, and the classic symptoms are redness, warmth, and burning pain. The feet are most commonly affected, with about 90% of patients experiencing it here. Hands and fingers may also be affected in roughly 25% of cases, while a very small percentage (2-3%) may experience symptoms in areas such as the head, genitals, or neck region.
  • #10 Pediatric erythromelalgia from multidisciplinary perspectives: a scoping review | Pediatric Research
    https://www.nature.com/articles/s41390-025-03817-4
    Erythromelalgia is a rare, chronic pain disorder characterized by the triad of intense burning sensation, warmth, and redness, primarily involving the hands and feet, and usually alleviated by cold and worsened by heat. […] Patients with these findings commonly experience prolonged delay in diagnosis, suffer from missed diagnosis or remained undiagnosed. […] The studies reported cases mainly affected in their hands (C=236), feet (C=358), and ears (C=45), or hands and feet (C=230). […] Most of the studies did not report any inciting event (S=130) or reported the spontaneous onset of erythromelalgia symptoms (C=10). […] Psychosocial factors were assessed in 23 studies, but findings were reported in only 21 studies, which primarily included anxiety, behavioral problems, depression, and suicidal ideation.
  • #11 Erythromelalgia – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK557787/
    Erythromelalgia is a rarely occurring disease entity characterized by a triad of erythema, warmth, and recurrent burning pain, most notably affecting the extremities. […] The classic presentation of erythromelalgia is a triad of redness, warmth, and burning pain in the extremities that occur in episodes. Erythromelalgia most commonly affects the feet (in 90% of patients) and less commonly the fingers and hands (in 25% of patients). […] The symptoms often begin with an itching sensation of the affected area, progressing to a more severe burning pain. The involvement is typically bilateral but can be asymmetric. […] Myeloproliferative disorders like essential thrombocytosis and polycythemia vera have a median onset of approximately 2.5 years after the onset of clinical symptoms of erythromelalgia.
  • #12 Anyone out there with Erythromelalgia? | Mayo Clinic Connect
    https://connect.mayoclinic.org/discussion/erythromelalgia/
    The erythromelalgia started April 24,2018. It is absolutely stopping everything I am able to do, even walking inside, because of the burning heat & pain. I am searching for new medicine, on Gabapentin now and it does nothing, and researching for a successful treatment! […] My hands sting and burn in heat, but my feet are th worse. I have been diagnosed since April 2018 with Erythromelalgia, and also Peripheral Neuropathy. I am taking Gabapentin and CBD oil, but nothing but “cold” helps! […] I amon Gabapentin and Cymbalta. Burning has moved up my intire right side. I wonder why one side?
  • #13 Erythromelalgia
    https://www.nhs.uk/conditions/erythromelalgia/
    Erythromelalgia usually affects the feet, but can also happen in the hands, arms, legs, ears and face. […] The main symptoms are: burning pain, which often starts as itching; redness this can look different on brown or black skin; warm or hot skin. […] Other symptoms include: swelling; sweating more than normal. […] The pain can either be constant or come and go. When the pain comes, it might be called a flare-up. A flare-up can come on gradually or suddenly, and last anywhere from a few minutes to a few days. […] Your skin may feel cold after a flare-up and look blue or grey. […] A flare-up of your symptoms is triggered by an increase in your body temperature. […] Erythromelalgia can be difficult to treat. You may need to try different combinations of treatments to find the ones that work for you.
  • #14 What Is Erythromelalgia?
    https://www.webmd.com/heart-disease/what-is-erythromelalgia
    Erythromelalgia Symptoms include an elevated skin temperature, pain that can be mild to severe in nature, and reddening skin. […] Other symptoms may be present as well. These symptoms include: Itching, Skin that seems cold in between flare-ups, Excessive sweating, Swelling, Blotchy skin that is tender, Feelings of tingling. […] Symptoms may present themselves on one side of your body or on both sides and can range in severity from mild to severe.
  • #15 Erythomelalgia | SRUK
    https://www.sruk.co.uk/scleroderma/erythomelalgia/
    Symptoms of Erythromelalgia (EM) become apparent during an attack- or as it’s known with Erythromelalgia- a 'flare-up’: Redness – caused by excessive blood flow to the affected area. The medical term for this is erythema. Hot to the touch. Pain – discomfort may range from mild tingling to severe burning. Others may feel sensations such as pins and needles or itching. […] Other symptoms may include: Swelling – the increased blood flow may cause a build-up of fluid in the affected body part. The medical term is edema. Change in perspiration – some people with erythromelalgia can sweat less (hidrosis) in the affected body part while others may sweat more (hyperhidrosis). Purple discoloration and/or cold to the touch – some may experience cold and/or bluish skin when they are not experiencing a flare-up. This may be related to the narrowing of the blood vessels, known as vasoconstriction. Some people may find that their symptoms appear on the face, ears, knees or other parts of the body. […] In some cases, when the underlying condition is treated, EM symptoms will improve or resolve completely.
  • #16 Clinical Characterization of Pediatric Erythromelalgia: A Single-Center Case Series
    https://www.mdpi.com/2227-9067/10/8/1282
    Burning and itching/tingling were the most common pain descriptors, though most patients reported multiple pain qualities and other sensory symptoms. […] The swelling of feet and/or hands was reported for 17 patients (40%). […] The age of onset tended to be younger for SCN9A+ patients (n = 3, median = 4 years, range = 1–5 years) than SCN9A− patients (n = 37, median = 13 years, IQR = 3.75–14.25 years). […] No treatments emerged as consistently effective.
  • #17 Erythromelalgia
    https://www.nhs.uk/conditions/erythromelalgia/
    Erythromelalgia usually affects the feet, but can also happen in the hands, arms, legs, ears and face. […] The main symptoms are: burning pain, which often starts as itching; redness this can look different on brown or black skin; warm or hot skin. […] Other symptoms include: swelling; sweating more than normal. […] The pain can either be constant or come and go. When the pain comes, it might be called a flare-up. A flare-up can come on gradually or suddenly, and last anywhere from a few minutes to a few days. […] Your skin may feel cold after a flare-up and look blue or grey. […] A flare-up of your symptoms is triggered by an increase in your body temperature. […] Erythromelalgia can be difficult to treat. You may need to try different combinations of treatments to find the ones that work for you.
  • #18 Erythromelalgia: Symptoms, Causes, Treatment & More
    https://www.healthline.com/health/erythromelalgia
    Erythromelalgia is a rare skin condition that affects the feet, hands, arms, and legs. Symptoms include episodes of redness, burning, and pain in the areas affected. […] Some people with erythromelalgia experience mild tingling pain, while others experience severe burning pain. Erythromelalgia episodes are called flare-ups. They occur when your body is exposed to certain triggers. […] Treatment for erythromelalgia depends on the severity, cause, and progression of the condition. […] Symptoms might be mild or severe. When symptoms are mild, you may be able to manage them without significant disruption to your daily life. When erythromelalgia symptoms are severe, the pain can make it difficult to walk or even sleep normally. […] Even with treatment, erythromelalgia can sometimes be progressive and get worse over time. Your outlook will depend on your specific case.
  • #19
    https://journals.lww.com/dmms/fulltext/2023/18040/rare_case_of_systemic_lupus_erythematosus.35.aspx
    Erythromelalgia (EM) is a characteristic triad of symptoms, including erythema, burning pain, and local rise of temperature. […] Secondary EM in patients with systemic lupus erythematosus (SLE) is a unique presentation; EM can be preceding or only clinical manifestation of systemic lupus for as long as 4 years or it may develop in a patient with well-established SLE. […] EM is a rare clinical syndrome characterized by a triad of symptoms of redness, warmth, and burning pain, most notably affecting the extremities. […] The episodic attacks can last from minutes to days or may become chronic persistent and tend to be precipitated by heat, exercise, and physical dependency. […] It is thought that EM in SLE may be on the same spectrum as Raynaud phenomenon (RP), with RP having a more prominent vasoconstrictive phase and EM having a more prominent reactive hyperemia. […] Although well established, occurrence of EM in SLE patients is a rare entity with exact incidence is not being known.
  • #20 Erythromelalgia – Clinical Tree
    https://clinicalpub.com/erythromelalgia/
    Erythromelalgia is a rare condition of the extremities characterized by the triad of redness, warmth, and pain. The symptom complex of intermittent acral warmth, pain, and erythema that defines erythromelalgia has been well documented in the medical literature for more than 150 years. The essential elements of this clinical syndrome, as described by its name, are intermittent (occasionally continuous) redness of an acral area (i.e., extremities, head and neck area) associated with heat and pain. Common terms used to describe the pain include piercing, burning, and discomfort. The pain and burning sensation can be extremely severe. In the majority of patients, symptoms are intermittent; episodes, precipitated by specific triggers, can last from minutes to hours. In a minority of patients, erythromelalgia symptoms are continuous, although they may wax and wane. The specific precipitant for erythromelalgia varies from person to person, but the most frequent precipitant is an increase in temperature of the affected acral area. Symptoms can also be precipitated or intensified by lowering the affected part. In the largest reported series (168 patients), symptoms predominantly involved feet (148 patients, 88.1%) and hands (43 patients, 25.6%).
  • #21 Erythromelalgia: Symptoms, Causes, Treatment
    https://my.clevelandclinic.org/health/diseases/22752-erythromelalgia
    Erythromelalgia symptoms, which often include burning pain, worsen over time. […] The three primary erythromelalgia symptoms are: Increase in skin temperature, Pain that ranges from mild tingling to severe burning, Skin redness. […] Symptoms may occur on one or both sides of your body. They can range from mild to intense. Some people experience their first flare in childhood, while others aren’t affected until adulthood. […] Flares usually occur when you experience a sudden increase in body temperature. […] Symptom flares can affect your quality of life, but they are rarely fatal. Most people live well into older adulthood. […] People who have the disease say that symptom flares can feel like: A binding sensation, as if your skin is too tight, Being on fire or scalded by hot water, Razor blades cutting your skin, Severe sunburn.
  • #22 Living With Erythromelalgia | FASA
    https://fasafw.com/blog/living-with-erythromelalgia/
    Erythromelalgia, also known as Mitchells disease, is a rare neurovascular condition affecting the extremities, most commonly the feet. […] An intense, burning sensation in the feet is erythromelalgias leading characteristic. Extreme redness of the skin and increased skin temperatures in affected areas are other typical symptoms. […] For most people, the symptoms are intermittent. Those living with erythromelalgia experience erratic episodes of painful, red-hot extremities. These symptoms can appear gradually, and sometimes the disease remains mild for years. […] Erythromelalgia can also have a sudden onset, with symptoms becoming severe in a matter of weeks or months. […] Look for the following erythromelalgia symptoms: Swelling, Burning pain that typically starts as itching, Sweating too much or too little, Warm or hot skin, Redness, the shade of which can vary depending on skin tone. […] An erythromelalgia flare can feel like blades cutting your skin, severe sunburn, tightness or a scald from hot water.
  • #23 Erythromelalgia: Definition, symptoms, and management
    https://www.medicalnewstoday.com/articles/erythromelalgia
    Although these symptoms are usually present in the feet, they can also occur in the hands, face, or other parts of the body. […] The symptoms usually present bilaterally, meaning that they occur on both sides of the body. However, in some cases, they can occur on one side only. […] EM is a rare condition that can cause flushed skin, pain, and swelling. […] Flare-ups of the condition typically occur due to an increase in body temperature.
  • #24 Rare erythromelalgia causes burning in extremities | UCLA Health
    https://www.uclahealth.org/news/article/rare-erythromelalgia-causes-burning-in-extremities
    Erythromelalgia is a condition marked by periodic episodes of stinging or burning pain that occurs in the extremities, along with a rise in skin temperature and visible flushing in the affected area. […] Erythromelalgia typically affects the feet. However, it can sometimes progress to involve the legs, arms or hands, and, very rarely, the ears or the face. The pain it causes is often intermittent and ranges from mild to severe. […] Additional symptoms may include swelling of the affected body part and a purplish cast to the skin between flare-ups. The condition usually affects both sides of the body. […] Symptoms are often triggered by an increase in body temperature. This can happen following exercise, standing for a period of time, from a too-warm bath or shower, on a hot day, eating spicy food, stress or excitement, becoming dehydrated, or even wearing a warm pair of socks. Ambient temperatures in the low to mid-80s and above are associated with the onset of symptoms.
  • #25 Erythromelalgia: Causes, Symptoms, and Treatment
    https://www.medicoverhospitals.in/diseases/erythromelalgia/
    Erythromelalgia is a rare and often debilitating condition characterized by episodes of intense burning pain, warmth, and redness of the extremities. […] The symptoms of erythromelalgia can vary significantly in severity and frequency. Common symptoms include: […] Burning Pain: The hallmark of erythromelalgia is intense burning pain in the affected areas. This pain can be constant or episodic. […] Redness: The skin in the affected areas often turns red or purple, reflecting increased blood flow. […] Warmth: The affected areas may feel unusually warm to the touch. […] Swelling: Swelling can accompany the other symptoms, adding to the discomfort. […] Certain factors can trigger or exacerbate symptoms, including: […] Heat: Warm weather or exposure to heat can worsen the symptoms.
  • #26 What Can Trigger Erythromelalgia?
    https://www.bergenfootgroup.com/blogs/item/112-what-can-trigger-erythromelalgia
    A rare condition that is known as erythromelalgia can cause a consistent, intense burning sensation in the feet. It can alter the quality of life, despite the fact it is not a fatal condition. Additional symptoms that are associated with this condition can include swelling, tenderness, and a burning feeling that can radiate to the entire foot. The symptoms often affect both feet and can be severe. […] This condition may develop from genetic reasons, and can happen as attacks. They can begin with itching, and may progress to severe pain. These attacks can be triggered by warm weather, sweating, exercise, or being immobile for long periods of time. If you are experiencing any of these symptoms, it is strongly suggested that you are under the care of a podiatrist who can properly diagnose and prescribe medication that may help the symptoms of erythromelalgia.
  • #27 Erythromelalgia – CheckOrphan
    https://checkorphan.org/disease/erythromelalgia/
    The signs and symptoms of erythromelalgia typically begin in childhood, although mildly affected individuals may have their first pain episode later in life. […] As individuals with erythromelalgia get older and the disease progresses, the hands and feet may be constantly red, and the affected areas can extend from the hands to the arms, shoulders, and face, and from the feet to the entire legs. […] Many people with primary erythromelalgia have stable symptoms, however cases of progressive disease (symptoms worsening overtime) have also been described.
  • #28 Erythromelalgia: Symptoms, Causes, Treatment & More
    https://www.healthline.com/health/erythromelalgia
    Erythromelalgia is a rare skin condition that affects the feet, hands, arms, and legs. Symptoms include episodes of redness, burning, and pain in the areas affected. […] Some people with erythromelalgia experience mild tingling pain, while others experience severe burning pain. Erythromelalgia episodes are called flare-ups. They occur when your body is exposed to certain triggers. […] Treatment for erythromelalgia depends on the severity, cause, and progression of the condition. […] Symptoms might be mild or severe. When symptoms are mild, you may be able to manage them without significant disruption to your daily life. When erythromelalgia symptoms are severe, the pain can make it difficult to walk or even sleep normally. […] Even with treatment, erythromelalgia can sometimes be progressive and get worse over time. Your outlook will depend on your specific case.
  • #29 Erythromelalgia – Heart and Blood Vessel Disorders – Merck Manual Consumer Version
    https://www.merckmanuals.com/home/heart-and-blood-vessel-disorders/peripheral-arterial-disease/erythromelalgia
    Erythromelalgia usually develops 2 to 3 years before another underlying disorder is diagnosed. […] Symptoms include burning pain in the feet or hands, which feel hot and appear red. Attacks are usually triggered by environmental temperatures of over 84 F (over about 29 C). Symptoms may remain mild for years or may progress and become completely incapacitating.
  • #30 Erythromelalgia
    https://www.pcds.org.uk/clinical-guidance/erythromelalgia
    Erythromelalgia presents before the appearance of the myeloproliferative disorder in 85% of cases and by a median time of 2.5 years. […] The classic description of erythromelalgia is painful, red, warm hands or feet, precipitated by exercise, heat or hanging the limb downward, and relieved with cooling and elevation. […] Symptoms often become so bad that normal functioning and quality of life are greatly affected. Patients avoid warm weather and may even relocate to cooler climates. Many cannot wear socks or closed shoes even in winter. Some patients become virtually housebound by frequent flare-ups. […] The prognosis varies. Patients who respond to aspirin have limited morbidity. Spontaneous remissions may occur. Early-onset disease is relatively unresponsive to treatment and generally unremitting.
  • #31 Erythromelalgia: MedlinePlus GeneticsLock
    https://medlineplus.gov/genetics/condition/erythromelalgia/
    Erythromelalgia is a condition characterized by episodes of pain, redness, and swelling in various parts of the body, particularly the hands and feet. […] The signs and symptoms of erythromelalgia typically begin in childhood, although mildly affected individuals may have their first pain episode later in life. As individuals with erythromelalgia get older and the disease progresses, the hands and feet may be constantly red, and the affected areas can extend from the hands to the arms, shoulders, and face, and from the feet to the entire legs.
  • #32 Erythromelalgia (Erythermalgia) – Dermatology Advisor
    https://www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/erythromelalgia-erythermalgia/
    Erythromelalgia is a rare disorder characterized by recurrent burning pain, erythema and increased skin temperature in the distal extremities, especially the feet and hands. This condition is induced or aggravated by exercise or warmth, and relieved by cooling the affected sites. […] The pain and redness are symmetrical and episodic, mainly involving feet at the beginning. Symptoms can extend to the lower legs, hands, and even the ears or face. Severity may progress with age and become constant. […] The symptoms are often milder than the primary form, and relief is obtained after the underlying diseases are properly treated or the offending drug is discontinued.
  • #33 Erythromelalgia Clinical Presentation: History, Physical Examination, Complications
    https://emedicine.medscape.com/article/200071-clinical
    In cases associated with a myeloproliferative disorder, erythromelalgia usually precedes the diagnosis of the myeloproliferative disorder by a median of 2.5 years. Rapid relief with aspirin is typical of this type of erythromelalgia and can be supportive diagnostically. […] Complications appear to be more frequent in patients who clearly have platelet-mediated disease. […] Ulceration, necrosis, and gangrene of affected extremities are possible. Digital necrosis or skin ulceration with secondary infection can lead to amputation. At least one patient had near-fatal hypothermia related to constant cooling required to control symptoms.
  • #34 Erythromelalgia – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/sites/books/NBK557787/
    Most patients with primary and secondary erythromelalgia are difficult to manage and are often resistant to treatment. […] Over time, ulceration, necrosis, and gangrene can develop in response to repeated ice water immersion or other forms of chronic cooling of the extremities. Quality of life has been shown to be dramatically affected and can lead to chronic depression due to fear of precipitating episodes.
  • #35 Erythromelalgia – symptoms, causes and treatment | healthdirect
    https://www.healthdirect.gov.au/erythromelalgia
    Erythromelalgia is a rare skin condition that causes red, painful, warm skin. […] The symptoms of erythromelalgia are having episodes of: redness this may look different on dark skin, pain which may start as itching, warm or hot skin, swelling, sweating more than normal. […] The symptoms most often affect your feet and sometimes your hands. Symptom episodes can last anywhere from a few minutes to days. Symptoms are more common in the evening and night. […] Erythromelalgia can lead to skin ulcers or gangrene (tissue death). […] Erythromelalgia can cause ongoing pain and affect your quality of life. If the pain is affecting your mental health, you may want to see a counsellor or psychologist.
  • #36 Erythromelalgia (EM) – Rare Awareness Rare Education
    https://rareportal.org.au/rare-disease/erythromelalgia-em/
    Erythromelalgia (EM) is a condition characterised by redness of the skin, feeling of warmth, and burning pain sensations in the feet and sometimes in the hands (extremities). There may also be itching and swelling in the affected areas. EM typically affects both sides of the body (bilateral) but may be observed in just one side (unilateral). The symptoms may come and go (intermittent), with the onset of symptoms described as a flare or episode. The flares often occur later in the day and at night. EM symptoms can vary from mild to severe, with some individuals experiencing on-going (chronic) pain. […] Symptoms of erythromelalgia (EM) include burning pain sensations in the feet and/or hands (extremities), accompanied by redness and increased temperature of the skin in the affected areas. These areas may also feel itchy, swollen, sore and sensitive to touch. Symptoms can vary from mild to severe and may come and go (intermittent). The flare of symptoms may be triggered by heat, alcohol and spicy food. The pain may become on-going (chronic) and affect daily activities. There may also be other complications such as skin ulcers, infection and tissue death (necrosis).
  • #37 Erythromelalgia
    https://dermnetnz.org/topics/erythromelalgia
    Erythromelalgia is characterised by intense burning pain, erythema (marked redness), and increased skin temperature, primarily of the feet and hands. […] The classic description of erythromelalgia is red, painful, warm hands or feet, brought on by warming or hanging the limb downward, and relieved with cooling and elevation. […] Symptoms often become so bad that normal functioning and quality of life are greatly affected. Patients avoid warm weather and may even relocate to cooler climates. Many cannot wear socks or closed shoes even in winter. Some patients become virtually housebound by continuous flare-ups and pain. […] In most patients with primary erythromelalgia, their symptoms remain refractory to sodium channel blockers and other treatment, and symptoms tend to progress over time. Quality of life can be greatly affected due pain and disability due to pain, secondary tissue damage (ulcers, necrosis, gangrene) or self-harming behaviours can be an issue.
  • #38 Pediatric erythromelalgia from multidisciplinary perspectives: a scoping review | Pediatric Research
    https://www.nature.com/articles/s41390-025-03817-4
    Impaired quality of life was reported in 107 studies (C=211), which included difficulty sleeping, limited physical activity, limited social activity, lifestyle change (e.g. wheelchair-bound, loss of autonomy, moving environment), and preference to walk barefoot. […] The scoping review revealed variability in the clinical presentation of pediatric erythromelalgia regarding diagnostic criteria, clinical examination findings and treatments offered.
  • #39 Erythromelalgia
    https://www.pcds.org.uk/clinical-guidance/erythromelalgia
    Erythromelalgia presents before the appearance of the myeloproliferative disorder in 85% of cases and by a median time of 2.5 years. […] The classic description of erythromelalgia is painful, red, warm hands or feet, precipitated by exercise, heat or hanging the limb downward, and relieved with cooling and elevation. […] Symptoms often become so bad that normal functioning and quality of life are greatly affected. Patients avoid warm weather and may even relocate to cooler climates. Many cannot wear socks or closed shoes even in winter. Some patients become virtually housebound by frequent flare-ups. […] The prognosis varies. Patients who respond to aspirin have limited morbidity. Spontaneous remissions may occur. Early-onset disease is relatively unresponsive to treatment and generally unremitting.
  • #40 The Erythromelalgia Association | Information, Research, Support
    https://burningfeet.org/
    The three hallmark symptoms of EM are: […] Redness, caused by excessive blood flow to the area […] Heat, meaning that the skin will feel warm or hot to the touch, also due to increased blood flow […] Pain, which may range from mild tingling to severe burning […] Additional symptoms may include swelling caused by the buildup of fluid in the affected body part, changes in perspiration, or purple discoloration when not actively flaring. EM flares are most commonly triggered by warmth, physical activity, or stress.
  • #41 Erythromelalgia – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/sites/books/NBK557787/
    Most patients with primary and secondary erythromelalgia are difficult to manage and are often resistant to treatment. […] Over time, ulceration, necrosis, and gangrene can develop in response to repeated ice water immersion or other forms of chronic cooling of the extremities. Quality of life has been shown to be dramatically affected and can lead to chronic depression due to fear of precipitating episodes.
  • #42 Pediatric erythromelalgia from multidisciplinary perspectives: a scoping review | Pediatric Research
    https://www.nature.com/articles/s41390-025-03817-4
    Erythromelalgia is a rare, chronic pain disorder characterized by the triad of intense burning sensation, warmth, and redness, primarily involving the hands and feet, and usually alleviated by cold and worsened by heat. […] Patients with these findings commonly experience prolonged delay in diagnosis, suffer from missed diagnosis or remained undiagnosed. […] The studies reported cases mainly affected in their hands (C=236), feet (C=358), and ears (C=45), or hands and feet (C=230). […] Most of the studies did not report any inciting event (S=130) or reported the spontaneous onset of erythromelalgia symptoms (C=10). […] Psychosocial factors were assessed in 23 studies, but findings were reported in only 21 studies, which primarily included anxiety, behavioral problems, depression, and suicidal ideation.
  • #43 Erythromelalgia – BAD Patient Hub
    https://www.skinhealthinfo.org.uk/condition/erythromelalgia/
    Erythromelalgia is a rare condition. It can cause episodes of intense burning pain, painful swelling and redness of the feet or hands. […] The symptoms of erythromelalgia are redness or increased darkening of the skin, increased skin temperature and a burning sensation at the affected area. The condition may produce symptoms intermittently but occasionally they may be continuous. It can last for a few minutes to several days. Symptoms can begin gradually or suddenly. […] A flare-up of erythromelalgia can be triggered by exposure to warmth, for example coming into a warm room, or by tight-fitting clothing, or by exercise. […] During a flare-up, the affected area may look red, blue, darker or mottled and swollen, and feels warm to the touch. […] There is no cure for erythromelalgia. The underlying cause for secondary erythromelalgia should be treated where possible. […] Unfortunately, treatment can be difficult and is mainly aimed at trying to prevent flare-ups. Responses to treatment vary. […] If you experience any psychological distress associated with the condition, you should talk about this with your doctor.
  • #44 Erythromelalgia – symptoms, causes and treatment | healthdirect
    https://www.healthdirect.gov.au/erythromelalgia
    Erythromelalgia is a rare skin condition that causes red, painful, warm skin. […] The symptoms of erythromelalgia are having episodes of: redness this may look different on dark skin, pain which may start as itching, warm or hot skin, swelling, sweating more than normal. […] The symptoms most often affect your feet and sometimes your hands. Symptom episodes can last anywhere from a few minutes to days. Symptoms are more common in the evening and night. […] Erythromelalgia can lead to skin ulcers or gangrene (tissue death). […] Erythromelalgia can cause ongoing pain and affect your quality of life. If the pain is affecting your mental health, you may want to see a counsellor or psychologist.
  • #45 Erythromelalgia (EM) – Rare Awareness Rare Education
    https://rareportal.org.au/rare-disease/erythromelalgia-em/
    Primary EM may occur spontaneously without a known cause (idiopathic) or may have a genetic component (inherited). Secondary EM is associated with other medical conditions, such as but not limited to, blood (haematological), metabolic, connective tissue and neurological disorders, cancers, autoimmune conditions, infections as well as side effects of certain medications.
  • #46 Erythromelalgia: A Very Rare Condition
    https://www.verywellhealth.com/erythromelalgia-overview-4582735
    Erythromelalgia (EM) is a rare condition affecting the feet and sometimes the hands. It causes episodic or continuous intense burning pain, severe redness, and skin warmth. The most common symptoms of EM are swelling, tenderness, deep painful aching, radiating or shooting sensations, and painful burning sensations in the hands and feet. The feet are more often affected, but symptoms can also affect the hands, face, and ears. […] In primary EM, burning pain, redness, and warmth are the most common symptoms, and the symptoms can be debilitating. The pain usually affects both sides of the body—both hands, for instance—is sporadic and severe, and will often affect the feet more than the hands. […] Primary EM pain attacks start with itchiness and will progress to severe burning pain. These pain attacks can last for minutes, hours, or even days. […] The symptoms of secondary EM occur when a person is experiencing symptoms of the underlying associated condition. Symptoms of secondary EM will present gradually and will worsen in a short time. Burning pain, redness, and warmth are the most common symptoms.
  • #47 Erythromelalgia – Wikipedia
    https://en.wikipedia.org/wiki/Erythromelalgia
    Symptoms may present gradually and incrementally, sometimes taking years to become intense enough for patients to seek medical care. In other cases symptoms emerge full blown with onset. […] Epidemic erythromelalgia is characterized by burning pain in the toes and soles of the feet, accompanied by foot redness, congestion, and edema; a few patients may have fever, palpitations, headache, and joint pain. […] The neuropathological symptoms of primary erythromelalgia arise from hyperexcitability of C-fibers in the dorsal root ganglion. Specifically, nociceptors appear to be the primarily affected neurons in these fibers. This hyperexcitability results in the severe burning pain experienced by patients. […] Many of those with primary erythromelalgia avoid wearing shoes or socks as the heat this generates is known to produce erythromelalgia attacks.
  • #48 Erythromelalgia: A Very Rare Condition
    https://www.verywellhealth.com/erythromelalgia-overview-4582735
    Erythromelalgia (EM) is a rare condition affecting the feet and sometimes the hands. It causes episodic or continuous intense burning pain, severe redness, and skin warmth. The most common symptoms of EM are swelling, tenderness, deep painful aching, radiating or shooting sensations, and painful burning sensations in the hands and feet. The feet are more often affected, but symptoms can also affect the hands, face, and ears. […] In primary EM, burning pain, redness, and warmth are the most common symptoms, and the symptoms can be debilitating. The pain usually affects both sides of the body—both hands, for instance—is sporadic and severe, and will often affect the feet more than the hands. […] Primary EM pain attacks start with itchiness and will progress to severe burning pain. These pain attacks can last for minutes, hours, or even days. […] The symptoms of secondary EM occur when a person is experiencing symptoms of the underlying associated condition. Symptoms of secondary EM will present gradually and will worsen in a short time. Burning pain, redness, and warmth are the most common symptoms.
  • #49 Erythromelalgia: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/200071-overview
    Erythromelalgia is a rare disorder characterized by burning pain, warmth, and redness, predominantly involving the extremities. The feet are more frequently involved than the hands. Rarely, non-extremity involvement is seen in areas such as the face and genitals. […] A distinction is generally made between primary (idiopathic or genetic) and secondary erythromelalgia (most commonly associated with myeloproliferative disorders), as well as between early- and late-onset disease. […] There is no known cure for erythromelalgia and thus symptomatic treatment forms the cornerstone of management. Therapies that are effective for many patients include aspirin, topical agents, and behavioral modifications. […] Patients who respond to aspirin have little morbidity. Spontaneous remissions may occur. Early-onset disease is relatively unresponsive to treatment and generally is unremitting.
  • #50 Erythromelalgia
    https://www.pcds.org.uk/clinical-guidance/erythromelalgia
    Erythromelalgia presents before the appearance of the myeloproliferative disorder in 85% of cases and by a median time of 2.5 years. […] The classic description of erythromelalgia is painful, red, warm hands or feet, precipitated by exercise, heat or hanging the limb downward, and relieved with cooling and elevation. […] Symptoms often become so bad that normal functioning and quality of life are greatly affected. Patients avoid warm weather and may even relocate to cooler climates. Many cannot wear socks or closed shoes even in winter. Some patients become virtually housebound by frequent flare-ups. […] The prognosis varies. Patients who respond to aspirin have limited morbidity. Spontaneous remissions may occur. Early-onset disease is relatively unresponsive to treatment and generally unremitting.
  • #51 Erythromelalgia (EM) – Rare Awareness Rare Education
    https://rareportal.org.au/rare-disease/erythromelalgia-em/
    Primary EM may occur spontaneously without a known cause (idiopathic) or may have a genetic component (inherited). Secondary EM is associated with other medical conditions, such as but not limited to, blood (haematological), metabolic, connective tissue and neurological disorders, cancers, autoimmune conditions, infections as well as side effects of certain medications.
  • #52 Erythromelalgia | NICS Well
    https://www.nicswell.co.uk/conditions-and-treatments/erythromelalgia
    Symptoms are usually triggered by an increase in body temperature. […] Cooling or elevating the affected part of the body may help relieve symptoms. […] In most cases of erythromelalgia, the cause is unknown. […] But it’s sometimes caused by another underlying medical condition or a faulty gene inherited from a parent. […] Erythromelalgia sometimes results from an underlying condition, such as a blood disorder such as polycythaemia, nerve damage for example, caused by peripheral neuropathy, multiple sclerosis (MS), or an autoimmune problem such as lupus or rheumatoid arthritis, where the immune system attacks the body’s own tissues. […] In some people with erythromelalgia, the disease is caused by a faulty gene. […] The faulty gene causes changes in the way pain signals are delivered to the brain, increasing or strengthening them.
  • #53 Erythromelalgia – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK557787/
    Most patients with primary and secondary erythromelalgia are difficult to manage and are often resistant to treatment. One exception is secondary disease due to polycythemia vera and essential thrombocythemia, as these patient populations often have a favorable response to aspirin therapy. […] Over time, ulceration, necrosis, and gangrene can develop in response to repeated ice water immersion or other forms of chronic cooling of the extremities. Quality of life has been shown to be dramatically affected and can lead to chronic depression due to fear of precipitating episodes.
  • #54
    https://journals.lww.com/dmms/fulltext/2023/18040/rare_case_of_systemic_lupus_erythematosus.35.aspx
    Erythromelalgia (EM) is a characteristic triad of symptoms, including erythema, burning pain, and local rise of temperature. […] Secondary EM in patients with systemic lupus erythematosus (SLE) is a unique presentation; EM can be preceding or only clinical manifestation of systemic lupus for as long as 4 years or it may develop in a patient with well-established SLE. […] EM is a rare clinical syndrome characterized by a triad of symptoms of redness, warmth, and burning pain, most notably affecting the extremities. […] The episodic attacks can last from minutes to days or may become chronic persistent and tend to be precipitated by heat, exercise, and physical dependency. […] It is thought that EM in SLE may be on the same spectrum as Raynaud phenomenon (RP), with RP having a more prominent vasoconstrictive phase and EM having a more prominent reactive hyperemia. […] Although well established, occurrence of EM in SLE patients is a rare entity with exact incidence is not being known.
  • #55 Erythromelalgia: A Very Rare Condition
    https://www.verywellhealth.com/erythromelalgia-overview-4582735
    Erythromelalgia (EM) is a rare condition affecting the feet and sometimes the hands. It causes episodic or continuous intense burning pain, severe redness, and skin warmth. The most common symptoms of EM are swelling, tenderness, deep painful aching, radiating or shooting sensations, and painful burning sensations in the hands and feet. The feet are more often affected, but symptoms can also affect the hands, face, and ears. […] In primary EM, burning pain, redness, and warmth are the most common symptoms, and the symptoms can be debilitating. The pain usually affects both sides of the body—both hands, for instance—is sporadic and severe, and will often affect the feet more than the hands. […] Primary EM pain attacks start with itchiness and will progress to severe burning pain. These pain attacks can last for minutes, hours, or even days. […] The symptoms of secondary EM occur when a person is experiencing symptoms of the underlying associated condition. Symptoms of secondary EM will present gradually and will worsen in a short time. Burning pain, redness, and warmth are the most common symptoms.
  • #56 Erythromelalgia (Erythermalgia) – Dermatology Advisor
    https://www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/erythromelalgia-erythermalgia/
    Erythromelalgia is a rare disorder characterized by recurrent burning pain, erythema and increased skin temperature in the distal extremities, especially the feet and hands. This condition is induced or aggravated by exercise or warmth, and relieved by cooling the affected sites. […] The pain and redness are symmetrical and episodic, mainly involving feet at the beginning. Symptoms can extend to the lower legs, hands, and even the ears or face. Severity may progress with age and become constant. […] The symptoms are often milder than the primary form, and relief is obtained after the underlying diseases are properly treated or the offending drug is discontinued.
  • #57 Erythromelalgia – MD Searchlight
    https://mdsearchlight.com/blood-disorders/erythromelalgia/
    Symptoms may last from minutes to days. Relief often comes from cooling or elevating the affected area. Episodes often occur at night due to increased temperature. Initially presents as an itching sensation, later progresses to severe burning pain. Affected areas are usually normal between episodes. […] Patients suffering from primary and secondary erythromelalgia, a rare condition characterized by episodes of burning pain and redness in parts of the body, can be challenging to treat. A type of erythromelalgia, secondary to conditions known as polycythemia vera and essential thrombocythemia, has been seen to respond favorably to aspirin therapy. Currently, there are differing views on the severity and outlook of primary versus secondary erythromelalgia. […] Moreover, in response to long-term immersion in ice water or other consistent cooling practices, patients might develop ulceration, necrosis (the death of body tissue), or gangrene (a condition where body tissue dies due to a lack of blood flow or a bacterial infection). This can significantly hinder the quality of life and could lead to chronic depression because of the fear of episode triggers.
  • #58 Erythomelalgia | SRUK
    https://www.sruk.co.uk/scleroderma/erythomelalgia/
    Symptoms of Erythromelalgia (EM) become apparent during an attack- or as it’s known with Erythromelalgia- a 'flare-up’: Redness – caused by excessive blood flow to the affected area. The medical term for this is erythema. Hot to the touch. Pain – discomfort may range from mild tingling to severe burning. Others may feel sensations such as pins and needles or itching. […] Other symptoms may include: Swelling – the increased blood flow may cause a build-up of fluid in the affected body part. The medical term is edema. Change in perspiration – some people with erythromelalgia can sweat less (hidrosis) in the affected body part while others may sweat more (hyperhidrosis). Purple discoloration and/or cold to the touch – some may experience cold and/or bluish skin when they are not experiencing a flare-up. This may be related to the narrowing of the blood vessels, known as vasoconstriction. Some people may find that their symptoms appear on the face, ears, knees or other parts of the body. […] In some cases, when the underlying condition is treated, EM symptoms will improve or resolve completely.
  • #59 Pediatric erythromelalgia from multidisciplinary perspectives: a scoping review | Pediatric Research
    https://www.nature.com/articles/s41390-025-03817-4
    Erythromelalgia is a rare, chronic pain disorder characterized by the triad of intense burning sensation, warmth, and redness, primarily involving the hands and feet, and usually alleviated by cold and worsened by heat. […] Patients with these findings commonly experience prolonged delay in diagnosis, suffer from missed diagnosis or remained undiagnosed. […] The studies reported cases mainly affected in their hands (C=236), feet (C=358), and ears (C=45), or hands and feet (C=230). […] Most of the studies did not report any inciting event (S=130) or reported the spontaneous onset of erythromelalgia symptoms (C=10). […] Psychosocial factors were assessed in 23 studies, but findings were reported in only 21 studies, which primarily included anxiety, behavioral problems, depression, and suicidal ideation.
  • #60 Erythromelalgia in the Feet | Podiatrist In Knoxville, TN | Family Foot & Ankle Clinic
    https://www.gardnerfootclinic.com/articles/general/514358-erythromelalgia-in-the-feet/
    Genetic factors play a pivotal role in foot Erythromelalgia. […] Foot Erythromelalgia introduces a layer of complexity in diagnosis due to the unique variability of symptoms in the feet. […] Distinguishing foot Erythromelalgia from other foot conditions is crucial. […] A tailored approach to diagnosis involves foot-specific criteria, considering the unique aspects of foot Erythromelalgia. […] Managing foot Erythromelalgia often necessitates footwear modifications to alleviate pain and discomfort. […] Medications play a pivotal role in controlling foot Erythromelalgia symptoms. […] Cool therapies can be particularly beneficial for foot Erythromelalgia. […] Living with foot Erythromelalgia demands tailored psychological support. […] Engaging in foot-specific patient advocacy and support groups can provide a unique platform for individuals with foot Erythromelalgia.
  • #61 Clinical Characterization of Pediatric Erythromelalgia: A Single-Center Case Series
    https://www.mdpi.com/2227-9067/10/8/1282
    Erythromelalgia is a descriptive term for severe burning pain and erythema in the distal extremities relieved by cold and exacerbated by heat. […] The median onset age was 12 years (IQR = 3–14). Patients saw a median of three specialists (IQR = 2–3) for a diagnosis. The majority (90%) reported bilateral symptoms. Cooling methods usually provided partial relief, while heat and exercise exacerbated pain. […] Symptoms are worsened by ambient or local heat exposure and generally partially relieved by the application of local cooling, such as immersing extremities in ice water. […] Symptoms were most frequently exacerbated by exercise/strenuous activity, heat, and ambient temperature changes. […] An inciting event was reported by 36% of patients. The most commonly reported type of inciting event was infection.
  • #62 Pediatric erythromelalgia from multidisciplinary perspectives: a scoping review | Pediatric Research
    https://www.nature.com/articles/s41390-025-03817-4
    Erythromelalgia is a rare, chronic pain disorder characterized by the triad of intense burning sensation, warmth, and redness, primarily involving the hands and feet, and usually alleviated by cold and worsened by heat. […] Patients with these findings commonly experience prolonged delay in diagnosis, suffer from missed diagnosis or remained undiagnosed. […] The studies reported cases mainly affected in their hands (C=236), feet (C=358), and ears (C=45), or hands and feet (C=230). […] Most of the studies did not report any inciting event (S=130) or reported the spontaneous onset of erythromelalgia symptoms (C=10). […] Psychosocial factors were assessed in 23 studies, but findings were reported in only 21 studies, which primarily included anxiety, behavioral problems, depression, and suicidal ideation.
  • #63 Erythromelalgia: Symptoms, Causes, Treatment | Qwark
    https://qwarkhealth.com/conditions/erythromelalgia
    Erythromelalgia is a chronic condition, meaning that it typically persists over time. While the severity and frequency of episodes may vary, the condition may worsen over time if left unmanaged. […] The prognosis for Erythromelalgia can be influenced by various factors, including the frequency and severity of symptoms, any underlying comorbid conditions, age at diagnosis, and response to treatment. […] While Erythromelalgia is a chronic condition, it is not always a progressive condition. In some cases, symptoms may remain stable over time with proper management strategies. However, in some individuals, the condition may worsen with time, leading to more frequent and severe episodes.
  • #64 Erythromelalgia
    https://www.pcds.org.uk/clinical-guidance/erythromelalgia
    Erythromelalgia presents before the appearance of the myeloproliferative disorder in 85% of cases and by a median time of 2.5 years. […] The classic description of erythromelalgia is painful, red, warm hands or feet, precipitated by exercise, heat or hanging the limb downward, and relieved with cooling and elevation. […] Symptoms often become so bad that normal functioning and quality of life are greatly affected. Patients avoid warm weather and may even relocate to cooler climates. Many cannot wear socks or closed shoes even in winter. Some patients become virtually housebound by frequent flare-ups. […] The prognosis varies. Patients who respond to aspirin have limited morbidity. Spontaneous remissions may occur. Early-onset disease is relatively unresponsive to treatment and generally unremitting.
  • #65 Erythromelalgia: Symptoms, Causes, Treatment | Qwark
    https://qwarkhealth.com/conditions/erythromelalgia
    Erythromelalgia is a chronic condition, meaning that it typically persists over time. While the severity and frequency of episodes may vary, the condition may worsen over time if left unmanaged. […] The prognosis for Erythromelalgia can be influenced by various factors, including the frequency and severity of symptoms, any underlying comorbid conditions, age at diagnosis, and response to treatment. […] While Erythromelalgia is a chronic condition, it is not always a progressive condition. In some cases, symptoms may remain stable over time with proper management strategies. However, in some individuals, the condition may worsen with time, leading to more frequent and severe episodes.
  • #66 Erythromelalgia: Practice Essentials, Background, Pathophysiology
    https://emedicine.medscape.com/article/200071-overview
    Erythromelalgia is a rare disorder characterized by burning pain, warmth, and redness, predominantly involving the extremities. The feet are more frequently involved than the hands. Rarely, non-extremity involvement is seen in areas such as the face and genitals. […] A distinction is generally made between primary (idiopathic or genetic) and secondary erythromelalgia (most commonly associated with myeloproliferative disorders), as well as between early- and late-onset disease. […] There is no known cure for erythromelalgia and thus symptomatic treatment forms the cornerstone of management. Therapies that are effective for many patients include aspirin, topical agents, and behavioral modifications. […] Patients who respond to aspirin have little morbidity. Spontaneous remissions may occur. Early-onset disease is relatively unresponsive to treatment and generally is unremitting.
  • #67 Erythromelalgia
    https://www.pcds.org.uk/clinical-guidance/erythromelalgia
    Erythromelalgia presents before the appearance of the myeloproliferative disorder in 85% of cases and by a median time of 2.5 years. […] The classic description of erythromelalgia is painful, red, warm hands or feet, precipitated by exercise, heat or hanging the limb downward, and relieved with cooling and elevation. […] Symptoms often become so bad that normal functioning and quality of life are greatly affected. Patients avoid warm weather and may even relocate to cooler climates. Many cannot wear socks or closed shoes even in winter. Some patients become virtually housebound by frequent flare-ups. […] The prognosis varies. Patients who respond to aspirin have limited morbidity. Spontaneous remissions may occur. Early-onset disease is relatively unresponsive to treatment and generally unremitting.
  • #68 Erythromelalgia: Symptoms, Causes, Treatment & More
    https://www.healthline.com/health/erythromelalgia
    Erythromelalgia is a rare skin condition that affects the feet, hands, arms, and legs. Symptoms include episodes of redness, burning, and pain in the areas affected. […] Some people with erythromelalgia experience mild tingling pain, while others experience severe burning pain. Erythromelalgia episodes are called flare-ups. They occur when your body is exposed to certain triggers. […] Treatment for erythromelalgia depends on the severity, cause, and progression of the condition. […] Symptoms might be mild or severe. When symptoms are mild, you may be able to manage them without significant disruption to your daily life. When erythromelalgia symptoms are severe, the pain can make it difficult to walk or even sleep normally. […] Even with treatment, erythromelalgia can sometimes be progressive and get worse over time. Your outlook will depend on your specific case.
  • #69 Erythromelalgia: Symptoms, Causes, Treatment | Qwark
    https://qwarkhealth.com/conditions/erythromelalgia
    Erythromelalgia symptoms usually last for a few minutes to a few hours or sometimes several days, and can happen intermittently for several years. […] Common symptoms of erythromelalgia include a severe burning sensation, redness and warmth, swelling, and increased skin temperature in affected areas of the body especially in the hands and feet. […] Physical sensations that are usually indicative of erythromelalgia include redness, warmth and burning. Patients also experience swelling and increased skin temperature in the affected areas. […] The long-term outcomes for patients with Erythromelalgia vary significantly based on individual cases. While some individuals may experience mild and manageable symptoms, others may experience severe pain and disabling symptoms that may impact their daily activities and quality of life.
  • #70 Erythromelalgia: Symptoms, Causes, Treatment
    https://my.clevelandclinic.org/health/diseases/22752-erythromelalgia
    Erythromelalgia symptoms, which often include burning pain, worsen over time. […] The three primary erythromelalgia symptoms are: Increase in skin temperature, Pain that ranges from mild tingling to severe burning, Skin redness. […] Symptoms may occur on one or both sides of your body. They can range from mild to intense. Some people experience their first flare in childhood, while others aren’t affected until adulthood. […] Flares usually occur when you experience a sudden increase in body temperature. […] Symptom flares can affect your quality of life, but they are rarely fatal. Most people live well into older adulthood. […] People who have the disease say that symptom flares can feel like: A binding sensation, as if your skin is too tight, Being on fire or scalded by hot water, Razor blades cutting your skin, Severe sunburn.
  • #71 Erythromelalgia | Symptoms, Diagnosis & Causes | Conditions | Healthera
    https://healthera.co.uk/conditions-a-z/erythromelalgia/
    Erythromelalgia is a rare condition that causes burning pain, redness, and warmth in the hands and feet. […] People with this condition experience episodes where their skin becomes red, hot, and painful. These episodes can last from minutes to hours and may be triggered by heat or exercise. […] The symptoms of erythromelalgia can vary, but common signs include: Burning pain in the feet, hands, or legs, Redness of the affected skin, Increased warmth in the affected areas, Episodes that can last for a long time, Worsening pain with heat or when standing. […] Erythromelalgia can cause significant discomfort, but there are ways to manage the symptoms and find support. If you are concerned about your health or symptoms, please speak to your GP or healthcare provider.