ORPHA:33577
Nodular non-suppurative panniculitis
Synonyms: Idiopathic lobular panniculitis · Idiopathic nodular panniculitis · Pfeiffer-Weber-Christian syndrome · Relapsing febrile nodular nonsuppurative panniculitis · Relapsing febrile nodular panniculitis · WCD · Weber-Christian disease · Weber-Christian panniculitis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare skin disorder characterized by recurring inflammation in the subcutaneous layer of fat.
1
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Nodular non-suppurative panniculitis | Idiopathic lobular panniculitis · Idiopathic nodular panniculitis · Pfeiffer-Weber-Christian syndrome · Relapsing febrile nodular nonsuppurative panniculitis · Relapsing febrile nodular panniculitis · WCD · Weber-Christian disease · Weber-Christian panniculitis |
| fr | Panniculite nodulaire non suppurative | Maladie de Weber-Christian · Panniculite de Weber-Christian · Panniculite lobulaire idiopathique · Panniculite nodulaire fébrile récurrente · Panniculite nodulaire fébrile récurrente non suppurative · Panniculite nodulaire idiopathique · Syndrome de Pfeiffer-Weber-Christian |
| de | Pannikulitis, noduläre nichteitrige | Noduläre nicht-suppurative Pannikulitis · Pannikulitis, febrile noduläre rekurrierende · Pannikulitis, idiopathische lobuläre · Pannikulitis, idiopathische noduläre · Pfeiffer-Weber-Christian-Syndrom · Weber-Christan-Krankheit · Weber-Christian Pannikulitis |
| es | Paniculitis nodular no supurativa | Enfermedad de Weber-Christian (WCD) · Paniculitis febril nodular recurrente · Paniculitis febril nodular recurrente no supurativa · Paniculitis idiopática lobular · Paniculitis idiopática nodular · Síndrome de Pfeiffer-Weber-Christian |
| it | Panniculite nodulare non suppurativa | Malattia di Weber-Christian (WCD) · Panniculite di Weber-Christian · Panniculite lobulare idiopatica · Panniculite nodulare borreliosica · Panniculite nodulare borreliosica non suppurativa · Panniculite nodulare idiopatica · Sindrome di Pfeiffer-Weber-Christian |
| nl | Nodulaire niet-suppuratieve panniculitis | Idiopathische lobulaire panniculitis · Idiopathische nodulaire panniculitis · Panniculitis van Weber-Christian · Syndroom van Pfeiffer-Weber-Christian · Terugkerende febriele nodulaire niet-suppuratieve panniculitis · Terugkerende febriele nodulaire panniculitis · WCD · Ziekte van Weber-Christian |
| pt | Paniculite não-supurativa nodular | Paniculite de Weber-Christian |
| pl | Nawracające nieropne zapalenie tkanki podskórnej | Choroba Webera i Christiana · Idiopatyczne guzkowe zapalenie tkanki podskórnej · Idiopatyczne płatowe zapalenie tkanki podskórnej · Nawracające gorączkowe guzkowe nieropne zapalenie tkanki podskórnej · Nawracające gorączkowe guzkowe zapalenie tkanki podskórnej · WCD · Zapalenie tkanki podskórnej Webera i Christiana · Zespół Pfeiffera, Webera i Christiana |
| cs | Nodulární nehnisavá panikulitida | Idiopatická lobulární panikulitida · Idiopatická nodulární panikulitida · Pfeifferova-Weberova-Christianova nemoc · Relabující febrilní nodulární nehnisavá panikulitida · Relabující febrilní nodulární panikulitida · WCD · Weberova-Christianova nemoc · Weberova-Christianova panikulitida · Pfeiffer-Weber-Christianova nemoc · Weber-Christianova nemoc · Weber-Christianova panikulitida |
| uk | Вузлуватий негнійний паннікуліт | Ідіопатичний лобулярний паннікуліт · Ідіопатичний вузликовий паннікуліт · Синдром Пфайфера-Вебера-Крістіана · Рецидивуючий гарячковий вузликовий ненагноительний паннікуліт · Рецидивуючий гарячковий вузликовий паннікуліт · WCD · Хвороба Вебера-Крістіана · Паннікуліт Вебера-Крістіана |
| zh | 非化脓性结节性脂膜炎 | 复发性发热性结节性非化脓性脂膜炎 |
| tr | Nodüler süpüratif olmayan pannikülit | WCD · Weber-Christian hastalığı · Weber-Christian pannikülit · İdiyopatik lobüler pannikülit · İdiyopatik nodüler pannikülit · Pfeiffer-Weber-Christian sendromu · Tekrarlayan ateşli nodüler pannikülit · Tekrarlayan ateşli nodüler, süpüratif olmayan pannikülit |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | M35.6 | E | specific | validated |
| ICD-11 | EF00.Y | NTBT | index | validated |
| MONDO | 0018063 | E | validated | |
| MeSH | D010201 | E | validated | |
| UMLS | C0030328 | E | validated | |
| GARD | 7879 | E | validated | |
| MedDRA | 10047883 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
3
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:79382Subcutaneous tissue disease
rare skin diseases
No children.
4
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 | 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.
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HPO phenotypes
15 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0008065 | Aplasia/Hypoplasia of the skin | |
| HP:0002829 | Arthralgia | |
| HP:0000969 | Edema | |
| HP:0010783 | Erythema | |
| HP:0001945 | Fever | |
| HP:0003326 | Myalgia | |
| HP:0002017 | Nausea and vomiting | |
| HP:0012490 | Panniculitis | |
| HP:0001482 | Subcutaneous nodule | |
| HP:0001824 | Weight loss | |
| Occasional (29-5%) | ||
| HP:0002960 | Autoimmunity | |
| HP:0002240 | Hepatomegaly | |
| HP:0100533 | Inflammatory abnormality of the eye | |
| HP:0001744 | Splenomegaly | |
Run the differential on the 11 obligate / very frequent terms
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/33577
- This record as JSON: /data/d/33577.json