Generalized eruptive histiocytosis
Synonyms: Generalized eruptive histiocytoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood, Elderly
- Status
- active
A rare non-Langerhans cell histiocytosis characterized by rapid onset of crops of asymptomatic small red to brown papules, typically distributed symmetrically over the face, trunk, and proximal extremities, occasionally with mucous membrane involvement. The lesions resolve spontaneously without scarring after a variable time span and do not recur in most cases. Histopathology reveals diffuse, uniform dermal infiltration with non-xanthomatous histiocytes staining positive for CD68 and Ki-M1p. Multinucleate giant cells may occasionally be found.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Generalized eruptive histiocytosis | Generalized eruptive histiocytoma |
| fr | Histiocytose éruptive généralisée | Histiocytome éruptif généralisé |
| de | Histiozytose, eruptive generalisierte | Histiozytom, eruptives generalisiertes |
| es | Histiocitosis eruptiva generalizada | Histiocitoma eruptivo generalizado |
| it | Istiocitosi generalizzata eruttiva | Istiocitoma generalizzato eruttivo |
| nl | Gegeneraliseerde eruptieve histiocytose | Gegeneraliseerd eruptief histiocytoom |
| pt | Histiocitose generalizada eruptiva | |
| pl | Histiocytoza uogólniona | = en |
| cs | Generalizovaná eruptivní histiocytóza | Generalizovaný eruptivní histiocytom |
| uk | Генералізований еруптивний гістіоцитоз | Генералізована еруптивна гістіоцитома |
| zh | 泛发性发疹性组织细胞增生病 | 泛发性发疹性组织细胞瘤 |
| tr | jeneralize erüptif histiyositoz | Jeneralize eruptiv histiyositoma |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D76.3 | NTBT | attributed | validated |
| ICD-11 | EE81 | NTBT | index | validated |
| MONDO | 0015532 | E | validated | |
| UMLS | C0347404 | 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 (1):
- ORPHA:157987Non-Langerhans cell histiocytosis
rare skin diseases; rare systemic and rheumatological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | <1 / 1 000 000 | — | Europe | Class only | Not yet 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.
HPO phenotypes
12 annotations · validated 2019-07-15| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0025475 | Erythematous macule | |
| HP:0030350 | Erythematous papule | |
| HP:0100727 | Histiocytosis | |
| Frequent (79-30%) | ||
| HP:0040186 | Maculopapular exanthema | |
| Occasional (29-5%) | ||
| HP:0040126 | Abnormal vitamin B12 level | |
| HP:0032061 | Hypereosinophilia | |
| HP:0031901 | Increased serum mast cell beta-tryptase concentration | |
| HP:0000989 | Pruritus | |
| Very rare (<4-1%) | ||
| HP:0001909 | Leukemia | |
| HP:0005585 | Spotty hyperpigmentation | |
| Excluded (0%) | ||
| HP:0031871 | Abnormal Langerhans cell morphology | |
| HP:0002716 | Lymphadenopathy | |
Run the differential on the 3 obligate / very frequent terms
Annotation source: 14060074[PMID]_25923837[PMID]_14699379[PMID]_14746649[PMID]_17989894[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/157991
- This record as JSON: /data/d/157991.json