Benign cephalic histiocytosis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Infancy
- Status
- active
A rare non-Langerhans cell histiocytosis characterized by multiple small yellowish-red or brown papules initially erupting predominantly in the head and neck region. The histopathological hallmark of these eventually self-healing lesions is a dermal proliferation of histiocytes with intracytoplasmic comma-shaped bodies, coated vesicles, and desmosome-like structures. Birbeck granules are absent. The disease typically occurs in young children.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Benign cephalic histiocytosis | |
| fr | Histiocytose céphalique bénigne | |
| de | Histiozytose, benigne zephale | |
| es | Histiocitosis cefálica benigna | |
| it | Istiocitosi cefalica benigna | |
| nl | Benigne cefale histiocytose | |
| pt | Histiocitoma cefálico benigno | |
| pl | Łagodna histiocytoza głowy | |
| cs | Benigní cefalická histiocytóza | |
| uk | Доброякісний гістіоцитоз головного мозку | |
| zh | 良性头部组织细胞增多症 | |
| tr | Selim sefalik histiyositoz |
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 | 0015533 | E | validated | |
| UMLS | C0347403 | 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
4 annotations · validated 2019-06-26| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0100727 | Histiocytosis | |
| HP:0200034 | Papule | |
| HP:0000988 | Skin rash | |
| Frequent (79-30%) | ||
| HP:0011123 | Inflammatory abnormality of the skin | |
Run the differential on the 3 obligate / very frequent terms
Annotation source: 7741553[PMID]_6721528[PMID]_3740882[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/157997
- This record as JSON: /data/d/157997.json