Secondary Erythromelalgia: Disease-Characteristics Research Report

Executive summary and evidence caveat

Secondary erythromelalgia (secondary EM) is an acquired, etiologically heterogeneous neurovascular pain syndrome characterized by recurrent or persistent burning pain, erythema, and increased skin temperature, usually in the feet or hands. Heat, exercise, dependency of the limb, prolonged standing, and tight footwear provoke attacks; cooling and elevation typically relieve them. Unlike inherited primary erythromelalgia, secondary EM is not itself an established monogenic channelopathy. It usually appears later in life in association with a myeloproliferative neoplasm (MPN), small-fiber or autonomic neuropathy, autoimmune/connective-tissue disease, metabolic disease, medication, infection, malignancy, toxin, or tissue injury. (assaad2025severeerythromelalgiapain pages 6-8, skeik2019erythromelalgia pages 2-3, algarni2025comparativeefficacyand pages 1-2)

The major evidence limitation is that most cohorts and therapeutic studies combine primary, idiopathic, and secondary EM. Consequently, many frequency and response estimates below describe EM overall, not secondary EM specifically. Secondary-disease evidence is strongest for the association between platelet-mediated MPNs and aspirin-responsive EM; most other associations and treatments rely on small observational studies, case series, or expert reviews. (skeik2019erythromelalgia pages 1-2, algarni2025comparativeefficacyand pages 4-5, algarni2025comparativeefficacyand pages 1-2)

Table: This table summarizes evidence-grade findings for secondary erythromelalgia across clinical, mechanistic, diagnostic, epidemiologic, therapeutic, genetic, and model domains. It highlights where evidence is strong, where data are mixed with primary erythromelalgia, and where disease-specific gaps remain.

1. Disease information

Definition and classification

The name derives from Greek erythros (red), melos (limb), and algos (pain). Common names are erythromelalgia, erythermalgia, erythralgia, Mitchell disease, and, when acquired, secondary erythromelalgia/secondary erythermalgia. The syndrome’s defining clinical triad is red, hot, painful extremities. Swelling may accompany attacks. Secondary EM is best represented as a disease-level syndrome, aggregated from clinical literature and disease resources—not as an individual-patient/EHR datum unless a knowledge-base record explicitly links it to a patient. (skeik2019erythromelalgia pages 1-2, skeik2019erythromelalgia pages 2-3)

Identifiers

• ICD-10-CM: I73.81, Erythromelalgia. This code does not reliably distinguish primary from secondary disease.
• MeSH: Erythromelalgia; a separate secondary-EM descriptor is generally not used.
• MONDO: A dedicated, independently curated secondary-EM MONDO identifier could not be confirmed from the retrieved evidence. Use the parent erythromelalgia concept plus a “secondary/acquired” qualifier and causal-disease relationship rather than assigning an unverified ID.
• OMIM: OMIM entries chiefly describe inherited primary erythromelalgia associated with SCN9A; these should not be used as identifiers for acquired secondary EM.
• Orphanet/ICD-11: Erythromelalgia concepts may be indexed, but a separate secondary-disease identifier was not verified in the available evidence.

2. Etiology, risks, protective factors, and gene–environment interaction

Causal or associated factors

1. Hematologic/MPN: essential thrombocythemia, polycythemia vera, myelodysplastic syndromes, and related platelet disorders. These are the most clinically actionable secondary causes. Platelet activation, aggregation, and arteriolar microthrombosis are implicated.
2. Neuropathic/metabolic: small-fiber neuropathy, diabetic neuropathy/diabetes mellitus, and other peripheral or autonomic neuropathies.
3. Autoimmune/connective-tissue: systemic lupus erythematosus and other connective-tissue or inflammatory disorders.
4. Neoplastic/paraneoplastic: solid tumors, hematologic malignancy, and paraproteinemic states have been reported.
5. Drug-associated: calcium-channel blockers, bromocriptine, and cyclosporine are repeatedly listed; individual drug associations generally derive from case reports.
6. Infectious or immune triggers: HIV, infectious mononucleosis, and temporal associations with influenza or hepatitis-B vaccination have been reported, but causality is weak.
7. Toxic/physical injury: mercury exposure, mushroom intoxication, burns, and frostbite are reported associations. (assaad2025severeerythromelalgiapain pages 6-8, skeik2019erythromelalgia pages 1-2, algarni2025comparativeefficacyand pages 4-5)

Risk and protective factors

Older age and the presence of an underlying MPN, neuropathy, diabetes, autoimmune disease, malignancy, or an implicated medication increase clinical suspicion. Heat exposure, exercise, prolonged standing, limb dependency, emotional stress, and occlusive footwear are attack triggers, not necessarily causes of the underlying disease. Female predominance has been reported in mixed EM cohorts, but a secondary-specific sex ratio is unavailable. (assaad2025severeerythromelalgiapain pages 6-8, skeik2019erythromelalgia pages 2-3, algarni2025comparativeefficacyand pages 4-5)

No validated genetic protective variant, dietary factor, medication prophylaxis, or population-level environmental protective exposure has been established. Avoidance of heat and mechanical triggers reduces attacks but should be classified as symptom prevention rather than protection against disease onset.

Gene–environment interaction

No reproducible secondary-EM-specific gene–environment interaction is established. Genetic or somatic drivers may belong to the underlying disorder—for example, MPN biology—while heat or dependency precipitates symptoms through altered microvascular and neural control. This differs fundamentally from inherited EM, in which a germline channel variant directly changes nociceptor excitability.

3. Phenotypes

Secondary EM generally has adult or late onset, although onset across all EM forms ranges from childhood to old age. It may be unilateral or localized more often than inherited primary EM, and its course follows the causal disorder: episodic, fluctuating, progressive, or treatment-responsive. Reviews report ages of 5–90 years, with a mean around 55 years, but this is not a secondary-only estimate. (assaad2025severeerythromelalgiapain pages 6-8, algarni2025comparativeefficacyand pages 1-2)

In a 168-patient mixed EM series cited by a systematic review, involvement was reported in the feet in 88.1%, hands in 25.6%, legs in 13.7%, and face in 2.4%. Approximately 5% reported family history, further illustrating that most EM is not clearly familial, although these values do not isolate secondary cases. (algarni2025comparativeefficacyand pages 1-2)

Quality of life

Severe attacks interfere with walking, standing, sleep, footwear, work, schooling, and social participation. Patients may resort to continuous cooling or water immersion, creating secondary wounds and infection. Disability, depression, and substantial pain interference are recognized, but validated secondary-EM-specific EQ-5D, SF-36, or PROMIS population norms are unavailable. Modern trials use EQ-5D-5L, Brief Pain Inventory, depression scores, physical activity, and disability measures, reflecting the syndrome’s multidimensional burden. (NCT04039633 chunk 1, NCT05917912 chunk 1)

4. Genetic and molecular information

Distinction from primary erythromelalgia

SCN9A encodes the voltage-gated sodium channel NaV1.7. Germline gain-of-function variants cause inherited primary EM by lowering nociceptor firing thresholds. That mechanism should not be automatically attributed to secondary EM. Genetic testing is appropriate when onset is early, disease is familial, or the phenotype suggests a channelopathy; it is not a confirmatory test for acquired secondary EM. Reviews report SCN9A variants in 5–36% of selected EM cohorts, but those mixed and referral-enriched estimates do not establish genetic causation in secondary disease. (skeik2019erythromelalgia pages 2-3, algarni2025comparativeefficacyand pages 6-7, algarni2025comparativeefficacyand pages 1-2)

No secondary-EM-specific germline causal gene, HGNC-listed modifier, protective allele, recurrent pathogenic copy-number change, chromosomal abnormality, or validated ACMG variant set is established. Therefore, secondary EM has no meaningful disease-level carrier frequency, penetrance, anticipation, founder effect, or consanguinity pattern.

Where secondary EM accompanies an MPN, somatic molecular findings such as an MPN driver belong to the underlying neoplasm, not to EM as an independent inherited disorder. Likewise, no reproducible secondary-EM-specific methylation, histone, transcriptomic, proteomic, metabolomic, lipidomic, single-cell, spatial-transcriptomic, or multi-omic signature was identified.

5. Environmental, lifestyle, and infectious information

Ambient heat, heated bedding, exercise, prolonged standing, limb dependency, physical activity, emotional stress, and tight shoes commonly precipitate symptoms. Cooling and elevation reduce them. These relationships represent thermoregulatory and hemodynamic provocation rather than traditional epidemiologic exposures. (skeik2019erythromelalgia pages 2-3, algarni2025comparativeefficacyand pages 4-5)

Mercury toxicity, mushroom intoxication, burns, and frostbite are reported potential causes; calcium-channel blockers, bromocriptine, and cyclosporine are medication associations. HIV and infectious mononucleosis have been reported, while vaccine associations are low-level temporal evidence and should not be treated as established causality. Smoking, alcohol intake, diet, obesity, radiation, and occupational exposure have no validated secondary-EM-specific risk estimates in the retrieved literature. (assaad2025severeerythromelalgiapain pages 6-8, skeik2019erythromelalgia pages 1-2)

6. Mechanism and pathophysiology

Secondary EM is probably a final common phenotype produced by several upstream mechanisms.

A. Platelet–microvascular pathway

Underlying MPN/platelet activation → platelet aggregation and prostanoid/thromboxane signaling → arteriolar inflammation or microthrombosis → maldistributed skin perfusion and tissue hypoxia → reactive hyperemia, warmth, erythema, and ischemic burning pain. Clinical aspirin responsiveness supports this model, particularly in essential thrombocythemia or polycythemia vera. (assaad2025severeerythromelalgiapain pages 6-8, skeik2019erythromelalgia pages 2-3)

Suggested annotations: - GO biological processes: platelet activation; platelet aggregation; blood coagulation; regulation of vasodilation; inflammatory response; response to hypoxia. - Cell Ontology: platelet; endothelial cell; vascular smooth-muscle cell. - Anatomical: cutaneous arteriole; dermal microvasculature.

B. Neuropathic/autonomic pathway

Diabetes, autoimmune neuropathy, toxic injury, or idiopathic small-fiber damage → dysfunction/loss of nociceptive and autonomic fibers → abnormal thermal pain signaling plus impaired sympathetic vasoconstriction and sweating → episodic vasodilation, heat, erythema, allodynia, and burning pain. Reduced nerve-terminal density and abnormal autonomic reflex testing support this pathway, although studies often combine EM subtypes. (skeik2019erythromelalgia pages 1-2, skeik2019erythromelalgia pages 2-3)

Suggested annotations: - GO: sensory perception of pain; detection of temperature stimulus; regulation of blood vessel diameter; autonomic nervous system process; axon maintenance. - CL: peripheral sensory neuron; nociceptor; sympathetic neuron; Schwann cell; endothelial cell.

C. Arteriovenous shunting and hypoxia

Thermoregulatory dyscontrol may open arteriovenous shunts while reducing nutritive capillary flow. This can produce the paradox of a visibly hyperemic, warm extremity with low transcutaneous oxygen tension and ischemic pain. Large-vessel Doppler studies may remain normal while skin temperature and laser-Doppler flow increase. (skeik2019erythromelalgia pages 2-3)

No single molecular pathway—MAPK, mTOR, PI3K–AKT, Wnt, or otherwise—has been validated as a unifying secondary-EM pathway. Mitochondrial, metabolic, and immune abnormalities should be annotated according to the underlying disease rather than inferred for all secondary EM.

7. Anatomical structures affected

• Organ/system level: skin and cutaneous circulation; peripheral and autonomic nervous systems; hematologic system when an MPN is causal.
• Primary sites: feet and toes most commonly, followed by hands/fingers and legs; face and ears are uncommon.
• Tissues: epidermis and dermis, cutaneous sensory/autonomic nerve fibers, arterioles, venules, capillaries, and arteriovenous anastomoses.
• Cells: nociceptors, sympathetic neurons, endothelial cells, vascular smooth-muscle cells, and—within MPN-associated disease—platelets.
• Subcellular: plasma membrane ion channels and synaptic/axonal compartments are relevant to neural excitability, but NaV1.7 dysfunction is proven mainly in inherited disease.
• Localization/lateralization: bilateral and symmetric disease is typical overall, but acquired disease may be unilateral, asymmetric, or localized. (algarni2025comparativeefficacyand pages 1-2)

Suggested UBERON concepts include skin of foot, foot, toe, hand, finger, lower limb, upper limb, dermis, epidermis, peripheral nerve, blood vessel, and cutaneous microvasculature.

8. Temporal development

Secondary EM usually begins after or around the onset of its associated disease and is commonly adult-onset. Presentation may be acute after a drug or injury, subacute with inflammatory disease, or insidious with MPN or neuropathy. Attacks last minutes to days, may increase during summer, and range from intermittent flares to continuous symptoms. No validated stage system exists. (skeik2019erythromelalgia pages 2-3)

Remission may follow withdrawal of an offending drug or successful treatment of the underlying disease. MPN-associated symptoms can respond rapidly to antiplatelet and cytoreductive treatment. Neuropathy-associated disease is often chronic and variably treatment-responsive. There is no established critical developmental window, although early recognition is important to prevent cooling injury and to detect an occult hematologic disorder.

9. Inheritance, epidemiology, and population

Secondary EM is acquired/non-Mendelian. Inheritance, penetrance, carrier frequency, anticipation, germline mosaicism, and prenatal recurrence risk are not applicable unless testing instead establishes inherited primary EM.

Population incidence is poorly defined. Mixed EM studies cited in reviews reported approximately 1.3 per 100,000 person-years in Olmsted County and 15 per 100,000 in New Zealand, with female predominance. Geographic differences may reflect case definition and ascertainment rather than true biology. Secondary-specific prevalence, incidence, ethnic distribution, and male:female ratio remain unknown. (skeik2019erythromelalgia pages 2-3)

10. Diagnostics

Clinical diagnosis

Diagnosis is based on the characteristic triad and provocation pattern. A photograph taken during an attack can be useful when the examination is normal between flares. There are no universally accepted validated diagnostic criteria or disease-specific biomarker. The source wording that diagnosis is “usually clinical” accurately summarizes current practice. (skeik2019erythromelalgia pages 1-2)

Cause-directed workup

1. CBC with differential and serial platelet/hematocrit assessment; evaluate persistent thrombocytosis or erythrocytosis for MPN.
2. Metabolic/neuropathy evaluation: fasting glucose or HbA1c, vitamin B12, thyroid studies, renal/hepatic testing, serum protein electrophoresis/immunofixation as clinically indicated.
3. Autoimmune/inflammatory testing: ANA and disease-specific serologies only when history or examination supports them.
4. Medication, infection, toxin, and injury history.
5. Vascular studies: pulses and ankle-brachial index/Doppler to exclude occlusive disease; large-vessel Doppler may be normal in EM.
6. Small-fiber/autonomic tests: quantitative sensory testing, autonomic reflex screen, sudomotor tests, and 3-mm skin biopsy for intraepidermal nerve-fiber density when neuropathy is suspected.
7. Thermography/laser Doppler/TcPO2: supportive research or specialist tests, not standalone diagnostic standards. Skin biopsy may show reduced nerve density or intraluminal thrombi but is neither necessary nor specific. (assaad2025severeerythromelalgiapain pages 6-8, skeik2019erythromelalgia pages 2-3)

Genetic testing

Single-gene SCN9A testing or a pain-channel/small-fiber-neuropathy panel is reasonable for childhood onset, a family history, or a strongly inherited phenotype. WES/WGS may be considered after specialist assessment when a monogenic disorder remains likely. CMA, karyotyping, FISH, mitochondrial DNA testing, and repeat-expansion testing have no routine role in secondary EM. There is no role for population, newborn, or carrier screening.

Differential diagnosis

Important alternatives are complex regional pain syndrome, Raynaud phenomenon/reperfusion, cellulitis or erysipelas, contact dermatitis, vasculitis, gout, osteomyelitis, arterial or venous insufficiency, acrocyanosis, Fabry disease, peripheral neuropathy, small-fiber neuropathy without EM, and systemic lupus erythematosus. CRPS is often unilateral and follows trauma with trophic/sudomotor abnormalities; cellulitis is persistent and infectious rather than heat-provoked and rapidly cooling-responsive. (skeik2019erythromelalgia pages 2-3)

11. Outcomes and prognosis

Secondary EM itself has no established disease-specific mortality rate, survival curve, or reduction in life expectancy. Prognosis depends mainly on the underlying disorder and severity of pain. MPN-associated cases may improve substantially with antiplatelet and disease-directed therapy; acquired neuropathic cases may remain chronic.

Morbidity can be profound: impaired walking, sleep loss, inability to tolerate shoes or warm environments, work/school limitation, anxiety, depression, and social isolation. Complications include maceration, ulceration, infection, tissue ischemia, and rarely amputation—often worsened by prolonged ice or water exposure. (skeik2019erythromelalgia pages 2-3, algarni2025comparativeefficacyand pages 1-2)

Favorable prognostic features include identification of a reversible cause and aspirin-responsive MPN disease. Persistent severe pain, established neuropathy, ulceration, and delayed cause recognition predict greater morbidity. No validated molecular prognostic biomarker exists.

12. Treatment and current applications

Treatment strategy

1. Identify and treat the underlying cause.
2. Avoid attacks safely: cool environment, fans, elevation, breathable footwear, activity pacing; avoid direct ice and prolonged immersion.
3. MPN-associated disease: low-dose aspirin when not contraindicated; manage the MPN with hematology-directed phlebotomy or cytoreduction as appropriate.
4. Localized symptoms: topical lidocaine or compounded amitriptyline–ketamine.
5. Neuropathic phenotype: gabapentinoids, SNRIs, tricyclics, or sodium-channel blockers selected according to comorbidity and safety.
6. Refractory disease: specialist trials of vasoactive therapy, infusions, sympathetic procedures, or neuromodulation.

The literature explicitly states, “There is no single effective treatment for erythromelalgia,” and individualized, multidisciplinary management remains the expert consensus. (skeik2019erythromelalgia pages 1-2, algarni2025comparativeefficacyand pages 2-4)

Drug and intervention evidence

• Aspirin: particularly effective in platelet-mediated MPN-associated secondary EM. Gastrointestinal bleeding, allergy, renal risk, and acquired von Willebrand disease in extreme thrombocytosis must be considered. Suggested NCIT intervention: aspirin therapy/antiplatelet therapy; CHEBI: aspirin.
• Topical amitriptyline–ketamine: in a mixed 36-patient cohort, about 75% reported some pain relief: 3% complete, 39% substantial, and 33% some improvement. This is not a secondary-only response estimate. (algarni2025comparativeefficacyand pages 6-7)
• Misoprostol: small controlled studies reported improvements in pain and temperature control, but diarrhea, abdominal pain, and nausea were frequent. (algarni2025comparativeefficacyand pages 6-7)
• Iloprost: small studies reported improved cooling scores, sympathetic dysfunction, and disease severity; headache, flushing/erythema, and hypotension can occur. (algarni2025comparativeefficacyand pages 6-7)
• Lidocaine: topical or intravenous use aims to stabilize hyperexcitable sensory fibers; experimental studies showed dose-dependent improvement in nociceptive thresholds. Cardiac and neurologic toxicity constrain systemic use. (algarni2025comparativeefficacyand pages 6-7, algarni2025comparativeefficacyand pages 2-4)
• Other reported agents: gabapentin/pregabalin, amitriptyline, venlafaxine or other SNRIs, mexiletine, carbamazepine/oxcarbazepine, antihistamines, clonidine, and prostaglandin/vascular agents. Evidence is inconsistent and mostly uncontrolled.
• Procedures: sympathetic blocks, epidural/local anesthetic techniques, chemical or surgical sympathectomy, botulinum toxin, and spinal-cord stimulation are rescue options with case-level or very small-study support.
• Pediatric rescue evidence: one report described complete resolution in 4 of 5 patients receiving intravenous sodium nitroprusside, initially 1 µg/kg/min and titrated to 5 µg/kg/min; this is low-level evidence and not secondary-specific. (skeik2019erythromelalgia pages 2-3)

A 2025 systematic review found only six eligible studies, totaling 120 patients: iloprost n=8, misoprostol n=21, topical amitriptyline–ketamine n=36, lidocaine n=27, chemical lumbar sympathectomy n=13, and miscellaneous agents n=11. The small, mixed-etiology evidence base precludes a robust universal hierarchy. (algarni2025comparativeefficacyand pages 4-5, algarni2025comparativeefficacyand pages 1-2)

Recent developments and trials

• EASE, NCT05917912: randomized double-blind Phase 2 crossover study of topical amitriptyline 15% (ATX01), 14 participants; began June 14, 2023 and completed February 29, 2024. Outcomes included attack pain, Brief Pain Inventory, EQ-5D, depression, and rescue cooling. Eligibility excluded important alternative neuropathic pain causes, limiting direct applicability to secondary EM. URL: https://clinicaltrials.gov/study/NCT05917912. (NCT05917912 chunk 1, NCT05917912 chunk 2)
• Burst spinal-cord stimulation, NCT04039633: six-patient quadruple-blind crossover study, active but not recruiting; restricted to primary or idiopathic EM and excluded secondary disease. URL: https://clinicaltrials.gov/study/NCT04039633. (NCT04039633 chunk 1)
• Earlier XPF-001, XPF-002, and PF-05089771 NaV1.7 programs enrolled primary/inherited EM, not secondary EM. URLs: https://clinicaltrials.gov/study/NCT01090622, https://clinicaltrials.gov/study/NCT01486446, and https://clinicaltrials.gov/study/NCT01769274. (NCT01090622 chunk 1, NCT01486446 chunk 2, NCT01769274 chunk 3)

Thus, a major 2023–2024 research gap is the continued exclusion or underrepresentation of secondary EM in interventional trials.

13. Prevention

There is no vaccine, population screening program, or proven pharmacologic primary prevention. Practical prevention is etiologic and tertiary:

• Monitor blood counts when symptoms or risk factors suggest an MPN.
• Control diabetes and treat reversible neuropathy causes.
• Review and discontinue implicated medication when clinically safe.
• Avoid excessive heat, prolonged standing, tight footwear, and unsafe direct-ice cooling.
• Treat fissures or ulcers early and provide wound-care education.
• Use genetic counseling only when the history suggests inherited primary EM; it is not routinely relevant to acquired secondary disease.

No evidence supports prophylactic aspirin for people without EM or an independent cardiovascular/hematologic indication.

14. Other species and natural disease

No well-validated naturally occurring veterinary equivalent of acquired secondary EM was identified. Consequently, no specific NCBI Taxon, breed/VBO association, zoonotic transmission, or cross-species prevalence can be assigned. The condition is not infectious or zoonotic as a syndrome.

SCN9A orthologs are evolutionarily conserved across mammals, but their relevance is primarily to nociception and inherited channelopathy. Platelet activation, microvascular thrombosis, diabetic neuropathy, and autonomic dysregulation can be studied in animals, but these reproduce components rather than the full human secondary-EM syndrome.

15. Model organisms and experimental systems

Human iPSC-derived sensory neurons carrying pathogenic SCN9A variants reproduce lowered firing thresholds and permit pharmacologic reversal of inherited EM phenotypes. These systems and NaV1.7 transgenic/knock-in models are valuable for pain-channel biology but model primary inherited EM, not secondary EM. The PF-05089771 program linked iPSC-derived sensory-neuron findings to a primary-EM trial, illustrating this translational application. (NCT01769274 chunk 3)

Potential component models include:

• MPN or platelet-activation mouse models for thrombotic microvascular mechanisms.
• Streptozotocin or genetic diabetes models for acquired small-fiber neuropathy.
• Peripheral nerve injury/inflammation models for nociceptor hyperexcitability.
• Endothelial-flow and microfluidic systems for capillary perfusion and thrombosis.

Their central limitation is failure to reproduce the defining human combination of heat-provoked erythema, thermoregulatory shunting, and severe distal burning pain. No dedicated, validated acquired secondary-EM mouse, rat, zebrafish, Drosophila, organoid, CRISPR-screen, or natural-animal model was identified.

Knowledge-base conclusions

Secondary erythromelalgia should be represented as an acquired clinical syndrome with multiple possible causal-disease relationships, not as an SCN9A disorder. Core phenotype assertions are well supported; secondary-specific epidemiology, molecular profiling, prognosis, and randomized-treatment evidence are sparse. The highest-priority implementation rules are: record the red-hot-burning attack phenotype; document heat/exercise/dependency triggers and cooling response; actively evaluate MPNs and neuropathies; annotate aspirin responsiveness specifically to platelet-mediated disease; and avoid transferring inherited-EM genetic claims or primary-only clinical-trial results to secondary EM.

References

1. (assaad2025severeerythromelalgiapain pages 6-8): Wassim Assaad, Omar El Tarras, Soad Al Osta, and Chady Kallassy. Severe erythromelalgia pain attack in a young lebanese woman leading to hospitalization: a case report and literature review. Cureus, Sep 2025. URL: https://doi.org/10.7759/cureus.91530, doi:10.7759/cureus.91530. This article has 0 citations.

2. (skeik2019erythromelalgia pages 2-3): Nedaa Skeik. Erythromelalgia. Harper's Textbook of Pediatric Dermatology, pages 1961-1964, Nov 2019. URL: https://doi.org/10.1002/9781119142812.ch151, doi:10.1002/9781119142812.ch151. This article has 1 citations.

3. (algarni2025comparativeefficacyand pages 1-2): Abdullah S. Algarni, Reem M. Alharthi, Shaden O. Alqurashi, Ruba M. Alghanmi, Rimaz R. Aldawsari, Maysaa A. Alghamdi, and Ramy Samargandi. Comparative efficacy and tolerability of treatments for erythromelalgia: a systematic review. Medicina, 61:920, May 2025. URL: https://doi.org/10.3390/medicina61050920, doi:10.3390/medicina61050920. This article has 1 citations.

4. (skeik2019erythromelalgia pages 1-2): Nedaa Skeik. Erythromelalgia. Harper's Textbook of Pediatric Dermatology, pages 1961-1964, Nov 2019. URL: https://doi.org/10.1002/9781119142812.ch151, doi:10.1002/9781119142812.ch151. This article has 1 citations.

5. (algarni2025comparativeefficacyand pages 4-5): Abdullah S. Algarni, Reem M. Alharthi, Shaden O. Alqurashi, Ruba M. Alghanmi, Rimaz R. Aldawsari, Maysaa A. Alghamdi, and Ramy Samargandi. Comparative efficacy and tolerability of treatments for erythromelalgia: a systematic review. Medicina, 61:920, May 2025. URL: https://doi.org/10.3390/medicina61050920, doi:10.3390/medicina61050920. This article has 1 citations.

6. (algarni2025comparativeefficacyand pages 6-7): Abdullah S. Algarni, Reem M. Alharthi, Shaden O. Alqurashi, Ruba M. Alghanmi, Rimaz R. Aldawsari, Maysaa A. Alghamdi, and Ramy Samargandi. Comparative efficacy and tolerability of treatments for erythromelalgia: a systematic review. Medicina, 61:920, May 2025. URL: https://doi.org/10.3390/medicina61050920, doi:10.3390/medicina61050920. This article has 1 citations.

7. (algarni2025comparativeefficacyand pages 7-9): Abdullah S. Algarni, Reem M. Alharthi, Shaden O. Alqurashi, Ruba M. Alghanmi, Rimaz R. Aldawsari, Maysaa A. Alghamdi, and Ramy Samargandi. Comparative efficacy and tolerability of treatments for erythromelalgia: a systematic review. Medicina, 61:920, May 2025. URL: https://doi.org/10.3390/medicina61050920, doi:10.3390/medicina61050920. This article has 1 citations.

8. (algarni2025comparativeefficacyand pages 2-4): Abdullah S. Algarni, Reem M. Alharthi, Shaden O. Alqurashi, Ruba M. Alghanmi, Rimaz R. Aldawsari, Maysaa A. Alghamdi, and Ramy Samargandi. Comparative efficacy and tolerability of treatments for erythromelalgia: a systematic review. Medicina, 61:920, May 2025. URL: https://doi.org/10.3390/medicina61050920, doi:10.3390/medicina61050920. This article has 1 citations.

9. (NCT04039633 chunk 1): Spinal Cord Stimulation for Refractory Pain in Erythromelalgia. St. Olavs Hospital. 2019. ClinicalTrials.gov Identifier: NCT04039633

10. (NCT05917912 chunk 1): EASE (Efficacy of ATX01 Study in Erythromelalgia). AlgoTherapeutix. 2023. ClinicalTrials.gov Identifier: NCT05917912

11. (NCT05917912 chunk 2): EASE (Efficacy of ATX01 Study in Erythromelalgia). AlgoTherapeutix. 2023. ClinicalTrials.gov Identifier: NCT05917912

12. (NCT01090622 chunk 1): Study of XPF-001 in the Treatment of Pain From Primary/Inherited Erythromelalgia (IEM). Xenon Pharmaceuticals Inc.. 2010. ClinicalTrials.gov Identifier: NCT01090622

13. (NCT01486446 chunk 2): Phase 2a, Exploratory Study to Evaluate the Safety, Efficacy, Tolerability and Pharmacokinetics of XPF-002 in Patients With Primary/Inherited Erythromelalgia. Xenon Pharmaceuticals Inc.. 2011. ClinicalTrials.gov Identifier: NCT01486446

14. (NCT01769274 chunk 3): Evaluation Of The Efficacy And Safety Of Single Doses Of PF-05089771 In Patients With Primary (Inherited) Erythromelalgia. Pfizer. 2012. ClinicalTrials.gov Identifier: NCT01769274

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