Secondary erythromelalgia
Synonyms: Secondary erythermalgia
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare neurologic disease characterized by intermittent pain, erythema, swelling, and heat of the extremities which is aggravated by warming and improved by cooling. Attacks can last between minutes and days and tend to be precipitated by heat, exercise, or physical dependence. The disease may be linked to a variety of underlying conditions including hematological, metabolic, connective tissue or neurological disorders, neoplasia, infections, and certain drug side effects, among others.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Secondary erythromelalgia | Secondary erythermalgia |
| fr | Erythromélalgie secondaire | Erythermalgie secondaire |
| de | Sekundäre Erythromelalgie | |
| es | Eritromelalgia secundaria | Eritermalgia secundaria |
| it | Eritromelalgia secondaria | |
| nl | Secundaire erytromelalgie | Secundaire erythermalgie |
| pt | Eritromelalgia secundária | |
| pl | Wtórna erytromelalgia | Wtórny bolesny rumień |
| cs | Sekundární erytromelalgie | = en |
| uk | Вторинна еритромелалгія | Вторинна еритема |
| zh | 继发性红斑性肢痛病 | = en |
| tr | Sekonder eritromelalji | Sekonder eritmalji |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | I73.8 | NTBT | attributed | validated |
| ICD-11 | EG00 | NTBT | index | validated |
| MONDO | 0035149 | E | validated | |
| UMLS | C0543820 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:496924Non-inflammatory vasculopathy
rare circulatory system diseases - ORPHA:79379Skin vascular disease
rare skin diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
| Annual incidence | 1-9 / 1 000 000 | 0.2 / 100 000 | United States | Value and class | Validated | 18713229[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
22 annotations · validated 2026-05-19| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0010783 | Erythema | |
| HP:0032147 | Erythromelalgia | |
| HP:0012531 | Pain | |
| HP:0009830 | Peripheral neuropathy | |
| Frequent (79-30%) | ||
| HP:0000970 | Anhidrosis | |
| HP:0033665 | Diminished health-related quality of life | |
| HP:0000989 | Pruritus | |
| Occasional (29-5%) | ||
| HP:0001872 | Abnormality of thrombocytes | |
| HP:0001063 | Acrocyanosis | |
| HP:0012533 | Allodynia | |
| HP:0002960 | Autoimmunity | |
| HP:0000819 | Diabetes mellitus | |
| HP:0002936 | Distal sensory impairment | |
| HP:0012534 | Dysesthesia | |
| HP:0002045 | Hypothermia | |
| HP:0001909 | Leukemia | |
| HP:0005547 | Myeloproliferative disorder | |
| HP:0002664 | Neoplasm | |
| HP:0010741 | Pedal edema | |
| HP:0001901 | Polycythemia | |
| HP:0200042 | Skin ulcer | |
| HP:0002633 | Vasculitis | |
Run the differential on the 4 obligate / very frequent terms
Annotation source: 26419464[PMID]_10724194[PMID]_23762561[PMID]_29641704[PMID]_30609105[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/529864
- This record as JSON: /data/d/529864.json