Interstitial granulomatous dermatitis with arthritis
Synonyms: Ackerman dermatitis syndrome · Ackerman syndrome · IGDA
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
Interstitial granulomatous dermatitis with arthritis is a rare rheumatologic disease characterized by the occurrence of inflammatory arthritis in association with large, erythematous, symmetrical cutaneous lesions (ranging from typical, but infrequent, cord-like lesions on the flanks to more common violaceous plaques on the trunk and limbs) featuring a typical histologic infiltrate mainly constituted of histiocytes.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Interstitial granulomatous dermatitis with arthritis | Ackerman dermatitis syndrome · Ackerman syndrome · IGDA |
| fr | Syndrome de dermatite granulomateuse interstitielle-arthrite | Dermatite d'Ackerman · Syndrome d'Ackerman |
| de | Dermatitis, granulomatöse interstitielle mit Arthritis | Ackerman-Dermatitis-Syndrom · Ackerman-Syndrom · IGDA |
| es | Dermatitis granulomatosa intersticial con artritis | IGDA · Síndrome de Ackerman · Síndrome de dermatitis de Ackerman |
| it | Dermatite granulomatosa interstiziale con artrite | Dermatite di Ackerman · IGDA |
| nl | Interstitiële granulomateuze dermatitis met artritis | Dermatitissyndroom van Ackerman · IGDA · Syndroom van Ackerman |
| pt | Dermatite granulomatosa intersticial com artrite | IGDA · Síndrome de dermatite de Ackerman |
| pl | Śródmiąższowe ziarniniakowe zapalenie skóry z zapaleniem stawów | IGDA · Zespół zapalenia skóry Ackermana |
| cs | Intersticiální granulomatózní dermatitida s artritidou | Ackermanuv dermatitický syndrom · IGDA · Ackermanův dermatitický syndrom |
| uk | Інтерстиціальний гранулематозний дерматит з артритом | Синдром акерманського дерматиту · Синдром Аккермана · IGDA |
| zh | 间质肉芽肿性皮炎伴关节炎 | Ackerman皮炎综合征 |
| tr | Artrit ile seyreden interstisyel granülomatöz dermatit | IGDA · Ackerman sendromu · Ackerman dermatit sendromu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L30.8 | NTBT | attributed | validated |
| ICD-11 | LD27.0Y | NTBT | index | validated |
| MONDO | 0019199 | E | validated | |
| UMLS | C4751206 | 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:90077Other acquired skin disease
rare skin diseases - ORPHA:182231Rare rheumatologic disease
rare systemic and rheumatological 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 | 53 cases | Worldwide | Case(s) | Validated | 22059717[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
8 annotations · validated 2016-09-23| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0010783 | Erythema | Diagnostic criterion |
| HP:0001370 | Rheumatoid arthritis | Diagnostic criterion |
| Very frequent (99-80%) | ||
| HP:0011227 | Elevated circulating C-reactive protein concentration | |
| HP:0003565 | Elevated erythrocyte sedimentation rate | |
| HP:0011123 | Inflammatory abnormality of the skin | |
| HP:0200034 | Papule | |
| Frequent (79-30%) | ||
| HP:0002923 | Rheumatoid factor positive | |
| Occasional (29-5%) | ||
| HP:0000989 | Pruritus | |
Run the differential on the 6 obligate / very frequent terms
Annotation source: 27274645[PMID]_26131871[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/79099
- This record as JSON: /data/d/79099.json