4 free views remaining today
erythromelalgia
ICD-10 Codes
Related ICD-10:
Description
Erythromelalgia is a rare disorder characterized by burning pain, warmth, and redness in the extremities, particularly the feet and hands.
Symptoms:
- Intense burning pain [2]
- Erythema (marked redness) [4]
- Increased skin temperature [4]
- Episodes of pain, redness, and swelling [8]
Classic Description:
The classic description of erythromelalgia is a triad of redness, pain, and warmth in the extremities, brought on by warming or dependency [7].
Other Characteristics:
- Redness or increased darkening of the skin [9]
- Increased skin temperature [9]
- Burning sensation at the affected area [9]
Erythromelalgia is a rare condition characterized by paroxysmal intense burning pain, erythema, and warmth of the skin [5]. It primarily occurs on the feet [3] and can be caused by mutations of the SCN9a gene or as a secondary disorder due to hematologic disorders or medication side effects [11].
References: [1] Not applicable [2] Jul 25, 2024 — Erythromelalgia is a rare disorder characterized by burning pain, warmth, and redness, predominantly involving the extremities. [3] Aug 5, 2024 — Erythromelalgia is a rare disorder that causes intermittent sensations of burning and redness. It primarily occurs on the feet. [4] Erythromelalgia is characterised by intense burning pain, erythema (marked redness), and increased skin temperature, primarily of the feet and hands. [5] Erythromelalgia (also known as erythermalgia) is a rare condition characterized by paroxysmal intense burning pain, erythema, and warmth of the skin. [6] Not applicable [7] Jul 25, 2024 — The classic description of erythromelalgia is a triad of redness, pain, and warmth in the extremities, brought on by warming or dependency ... [8] Feb 1, 2016 — Erythromelalgia is a condition characterized by episodes of pain, redness, and swelling in various parts of the body, particularly the hands ... [9] The symptoms of erythromelalgia are redness or increased darkening of the skin, increased skin temperature and a burning sensation at the affected area. The ... [10] Not applicable [11] Result: a rare disorder characterized by periodic inflammation and blockage of the vessels of the extremities, resulting in skin redness, swelling, and burning pain in the affected sites; it may manifest as a primary disorder caused by mutations of the SCN9a gene or as a secondary disorder due to hematologic disorders or medication side effects
Additional Characteristics
- Intense burning pain
- Erythema (marked redness)
- Increased skin temperature
- Episodes of pain, redness, and swelling
- Redness or increased darkening of the skin
- Burning sensation at the affected area
Signs and Symptoms
Erythromelalgia is a rare condition that affects the skin, causing burning pain, redness, and increased skin temperature. The symptoms can vary from person to person, but common signs include:
- Burning pain in the feet or hands, which can be severe and debilitating [4]
- Redness of the affected skin area, which can be intense and warm to the touch [1][5]
- Increased skin temperature, which can feel hot to the touch [7]
- Episodes of pain, redness, and swelling in various parts of the body, particularly the hands and feet [6]
In most cases (approximately 90%), erythromelalgia affects the feet, but it can also occur on the hands. The symptoms can be constant or come and go, and treatment is available to manage the condition.
It's worth noting that the exact cause of erythromelalgia is still not fully understood, but research suggests that it may be related to genetic mutations, autoimmune disorders, or other underlying medical conditions [2][3]. If you're experiencing symptoms of erythromelalgia, it's essential to consult with a healthcare professional for proper diagnosis and treatment.
References: [1] Apr 18, 2022 — Erythromelalgia causes episodes of burning pain in the feet and other extremities. Symptom flares can slow you down, but you can still live ... [2] Erythromelalgia is a rare condition that causes burning pain, redness and hot skin, mainly in the feet. It can be constant or come and go, but treatment and ... [3] Erythromelalgia is a rare skin condition which causes a burning pain and red skin usually on your hands or feet. Read more about erythromelalgia here. [4] Nov 28, 2022 — The signs and symptoms of erythromelalgia include heat, pain, and redness in the affected skin area. In approximately 90 percent of individuals, ... [5] Aug 5, 2024 — Erythromelalgia is a rare disorder that causes intermittent sensations of burning and redness. It primarily occurs on the feet. [6] Feb 1, 2016 — Erythromelalgia is a condition characterized by episodes of pain, redness, and swelling in various parts of the body, particularly the hands and feet. [7] 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 ( ...
Additional Symptoms
- Increased skin temperature
- Burning pain in the feet or hands
- Redness of the affected skin area
- Episodes of pain, redness, and swelling in various parts of the body
Diagnostic Tests
Erythromelalgia diagnosis involves a combination of clinical evaluation and various diagnostic tests to confirm the condition.
Blood Tests: A complete blood count (CBC) with differential is often performed to rule out other conditions that may cause similar symptoms. This test helps identify any abnormalities in blood cell counts, which can be indicative of myeloproliferative disorders [1].
Genetic Testing: Gene sequencing has become the gold standard for diagnosing erythromelalgia, as it can identify specific genetic mutations associated with the condition [5]. Genetic and genomic testing may also be performed to look for changes in genes that may be causing symptoms [7].
Thermography: This test measures skin temperature, which is often elevated in individuals with erythromelalgia. Thermography can help confirm the diagnosis by showing increased skin temperature in affected areas.
Imaging Tests: Imaging tests such as X-rays and thermography may also be used to rule out other conditions that may cause similar symptoms [3]. However, these tests are not typically used for diagnosing erythromelalgia.
It's worth noting that a diagnosis of erythromelalgia is often based on clinical evaluation and the presence of characteristic symptoms, such as burning pain and increased skin temperature. Diagnostic tests are used to support this diagnosis and rule out other conditions.
References: [1] - Search result 2 [3] - Search result 4 [5] - Search result 6 [7] - Search result 8
Additional Diagnostic Tests
- Blood Tests
- Thermography
- Genetic Testing
Treatment
Erythromelalgia treatment often involves drug therapy to alleviate symptoms such as burning pain, redness, and warmth in the extremities.
Oral Medications
Several oral medications have been used to treat erythromelalgia, including:
- Anticonvulsants: Gabapentin or carbamazepine may be prescribed to relieve pain and reduce the frequency of episodes [4].
- Antidepressants: Amitriptyline or venlafaxine can also be effective in managing symptoms [4].
- Selective serotonin and norepinephrine reuptake inhibitors (SSNRIs): Fluoxetine, sertraline, and venlafaxine have been used with reported remission [5].
Topical Medications
In some cases, topical medications may be used as a first-line treatment to alleviate erythema and pain. These include:
- Aspirin: May provide relief of short duration [6].
- Capsaicin: Can help reduce pain and inflammation [7].
- Diclofenac gel 1%: May be effective in managing symptoms [7].
- Brimonidine: Has been used to alleviate erythema and pain [7].
Other Options
In some cases, other medications may be considered, such as:
- Anagrelide: May be an alternative for patients who do not respond to other treatments [14].
- Platelet-inhibiting agents: Such as ticlopidine and dipyridamole, have been tried but are not effective in treating erythromelalgia [14].
It's essential to note that each patient's response to treatment may vary, and a healthcare provider should be consulted for personalized guidance on managing erythromelalgia symptoms.
References:
[4] - Context result 4 [5] - Context result 5 [6] - Context result 6 [7] - Context result 7
Recommended Medications
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Erythromelalgia is a rare disorder characterized by burning pain, redness, and heat in the hands and feet. When diagnosing erythromelalgia, it's essential to consider several conditions that may mimic its symptoms. Here are some of the key differential diagnoses:
- Complex Regional Pain Syndrome (CRPS): Also known as Reflex Sympathetic Dystrophy, CRPS is a condition that causes chronic pain, redness, and swelling in one or more limbs. It's often associated with trauma or injury to the affected area.
- Fabry disease: A genetic disorder that affects the production of an enzyme called alpha-Galactosidase A, leading to the accumulation of fatty substances in the body's cells. Symptoms can include burning pain, redness, and heat in the hands and feet.
- Peripheral neuropathy: Damage to the peripheral nerves can cause numbness, tingling, and pain in the affected areas. In some cases, it may be mistaken for erythromelalgia.
- Raynaud phenomenon: A condition that affects blood flow to the fingers and toes, causing them to turn white or blue in response to cold temperatures or stress.
- Paroxysmal extreme pain disorder (PEPD): A rare genetic disorder characterized by recurring episodes of severe pain, often accompanied by redness and heat in the affected areas.
These conditions can be challenging to distinguish from erythromelalgia, as they share similar symptoms. However, a thorough evaluation, including medical history, physical examination, and laboratory tests, is essential for accurate diagnosis.
According to [1], the differential diagnoses of erythromelalgia include post-traumatic reflex dystrophies, shoulder-hand syndrome, peripheral neuropathy, causalgia, Fabry disease, and bacterial infections. A detailed evaluation of these conditions can help healthcare providers make an informed decision about the underlying cause of symptoms.
In some cases, erythromelalgia may be an early sign of polycythaemia (raised red cells) or thrombocythaemia (raised platelets), where symptoms may precede diagnosis of the underlying condition [9].
References:
[1] Caldito, E. G. (2024). Paroxysmal extreme pain disorder, complex regional pain syndrome (CRPS), peripheral neuropathy, Fabry's disease, and Raynaud Phenomenon are conditions that can mimic erythromelalgia.
[2] Caldito, E. G. (2023). Key points: Several conditions characterized by erythema and/or pain can mimic erythromelalgia (EM).
[9] Apr 18, 2022 — There's no specific test for diagnosing erythromelalgia. Your healthcare provider reviews your symptoms and examines any active flare-ups or...
Additional Differential Diagnoses
Additional Information
- rdf-schema#domain
- https://w3id.org/def/predibionto#has_symptom_13603
- owl#annotatedSource
- t456083
- oboInOwl#hasOBONamespace
- disease_ontology
- oboInOwl#id
- DOID:9240
- core#notation
- DOID:9240
- oboInOwl#hasDbXref
- UMLS_CUI:C0014804
- IAO_0000115
- A neuropathy that is characterized by intense, burning pain of affected extremities, severe redness and increased skin temperature.
- oboInOwl#hasExactSynonym
- erythermalgia
- rdf-schema#label
- erythromelalgia
- oboInOwl#inSubset
- http://purl.obolibrary.org/obo/doid#NCIthesaurus
- rdf-schema#subClassOf
- http://purl.obolibrary.org/obo/DOID_870
- relatedICD
- http://example.org/icd10/I73.81
- 22-rdf-syntax-ns#type
- http://www.w3.org/2002/07/owl#Class
Medical Disclaimer: The information provided on this website is for general informational and educational purposes only.
It is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified healthcare provider with questions about your medical condition.