Infantile digital fibromatosis
Synonyms: Inclusion body fibromatosis · Recurring digital fibrous tumor of childhood · Reye tumor
- Type
- Disease
- Group
- Disorder
- Age of onset
- Childhood, Infancy, Neonatal
- Status
- active
A rare, benign, superficial fibromatosis characterized by firm, pinkish to flesh-colored, solitary or multiple nodular growths, typically less than 2 cm in size. They occur on the dorsal or lateral aspect of fingers and toes and have a tendency to recur. Histology reveals bland intradermal spindle cells with spherical perinuclear inclusion bodies.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Infantile digital fibromatosis | Inclusion body fibromatosis · Recurring digital fibrous tumor of childhood · Reye tumor |
| fr | Fibromatose digitale infantile | Fibromatose à corps d'inclusion · Tumeur de Reye · Tumeur fibreuse digitale récurrente de l'enfance |
| de | Fibromatose, digitale infantile | Fibroblastentumor mit Zytoplasmaeinschlüssen · Reye-Tumor · Rezidivierender digitaler fibröser Tumor der Kindheit |
| es | Fibromatosis digital infantil | Fibromatosis con cuerpos de inclusión · Tumor de Reye · Tumor fibroso digital recurrente de la infancia |
| it | Fibromatosi digitale infantile | Fibromatosi con corpi inclusi · Tumore di Reye · Tumore fibroso digitale ricorrente dell'infanzia |
| nl | Infantiele digitale fibromatose | Inclusielichaamfibromatose · Recidiverende digitale fibreuze tumor in de kindertijd · Reye-tumor |
| pt | Fibromatose digital da infância | |
| pl | Dziecięca włókniakowatość palców | Guz Reye'a · Nawracający dziecięcy guz włóknisty palców · Włókniakowatość z ciałkami wtrętowymi |
| cs | Infantilní digitální fibromatóza | Fibromatóza s inkluzními tělísky · Recidivující digitální fibrózní nádor dětského věku · Reyeův nádor |
| uk | Інфантильний дигітальний фіброматоз | Інклюзивний фіброматоз тіла · Рецидивуюча фіброзна пухлина пальця дитячого віку · Пухлина Рейє |
| zh | 婴儿指趾纤维瘤病 | 儿童期复发指趾纤维瘤病 |
| tr | İnfantil dijital fibromatoz | Reye tümörü · İnklüzyon vücut fibromatozu · Çocukluk çağı nükseden dijital fibröz tümörü |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | M72.8 | NTBT | attributed | validated |
| ICD-11 | EE6Y | NTBT | index | validated |
| MONDO | 0016039 | E | validated | |
| UMLS | C1318562 | E | validated | |
| GARD | 8487 | 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:199257Superficial fibromatosis
rare skin diseases; rare neoplastic 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 |
|---|---|---|---|---|---|---|
| Cases/families | 200 cases | Worldwide | Case(s) | Validated | 23157779[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | 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.
HPO phenotypes
5 annotations · validated 2016-11-02| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0200036 | Skin nodule | |
| Frequent (79-30%) | ||
| HP:0025092 | Epidermal acanthosis | |
| HP:0000962 | Hyperkeratosis | |
| HP:0001036 | Parakeratosis | |
| Very rare (<4-1%) | ||
| HP:0012531 | Pain | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 27684985[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/199267
- This record as JSON: /data/d/199267.json