ORPHA:280062
Calciphylaxis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare vascular calcification disorder typically characterized by occlusion of microvessels in the cutaneous tissue resulting in painful cutaneous lesions. The disorder is often life-limiting.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Calciphylaxis | |
| fr | Calciphylaxie | |
| de | Kalziphylaxie | CUA · Calciphylaxie · Kalzifizierende urämische Arteriolopathie |
| es | Calcifilaxis | |
| it | Calcifilassi | |
| nl | Calciphylaxis | Calcificerende arteriolopathie door uremie · Calcificerende uremische arteriolopathie · Calcifylaxie |
| pt | Calcifilaxia | |
| pl | Kalcyfilaksja | |
| cs | Kalcifylaxe | |
| uk | Кальцифілаксія | |
| zh | 钙过敏症 | |
| tr | Kalsifilaksi |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | E83.5 | NTBT | attributed | validated |
| ICD-11 | EB90.42 | E | specific | validated |
| MONDO | 0017215 | E | validated | |
| MeSH | D002115 | E | validated | |
| UMLS | C0006666 | E | validated | |
| GARD | 5980 | E | validated | |
| MedDRA | 10051714 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:496924Non-inflammatory vasculopathy
rare circulatory system diseases - ORPHA:182222Rare systemic disease
rare systemic and rheumatological diseases
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
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HPO phenotypes
13 annotations · validated 2017-02-22| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0001939 | Abnormality of metabolism/homeostasis | |
| HP:0011122 | Abnormality of skin physiology | |
| HP:0003207 | Arterial calcification | |
| HP:0100658 | Cellulitis | |
| HP:0000965 | Cutis marmorata | |
| HP:0011986 | Ectopic ossification | |
| HP:0100758 | Gangrene | |
| HP:0002905 | Hyperphosphatemia | |
| HP:0000867 | Secondary hyperparathyroidism | |
| HP:0100806 | Sepsis | |
| HP:0200042 | Skin ulcer | |
| HP:0003774 | Stage 5 chronic kidney disease | |
| Frequent (79-30%) | ||
| HP:0033260 | Livedo racemosa | |
Run the differential on the 12 obligate / very frequent terms
Annotation source: 9426423[PMID]_26394755[PMID]
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/280062
- This record as JSON: /data/d/280062.json