Sweet syndrome
Synonyms: Acute febrile neutrophilic dermatosis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Multigenic/multifactorial
- Age of onset
- Adult
- Status
- active
A rare inflammatory disease characterized by abrupt appearance of painful, edematous and erythematous papules, plaques and nodules on the skin, and frequently accompanied by fever and neutrophilia with a dense infiltration of mature neutrophils that are typically located in the upper dermis. The disease is classically associated with inflammatory disease, pregnancy, infection (mostly of the upper respiratory tract), or vaccination but may be idiopathic, associated with a hematological or visceral malignancy, or drug-induced.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Sweet syndrome | Acute febrile neutrophilic dermatosis |
| fr | Syndrome de Sweet | Dermatose neutrophile aiguë fébrile |
| de | Sweet-Syndrom | Dermatose, akute febrile neutrophile |
| es | Síndrome de Sweet | |
| it | Sindrome di Sweet | Dermatosi neutrofila acuta febbrile |
| nl | Syndroom van Sweet | Acute febriele neutrofiele dermatose |
| pt | Síndrome Sweet | Dermatose neutrofílica febril aguda |
| pl | Zespół Sweeta | Ostra neutrofilowa dermatoza gorączkowa |
| cs | Sweetův syndrom | Akutní febrilní neutrofilní dermatóza |
| uk | Синдром Світа | Гострий гарячковий нейтрофільний дерматоз |
| zh | Sweet综合征 | 急性发热性嗜中性皮肤病 |
| tr | Sweet sendromu | Akut ateşli nötrofilik dermatoz |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 608068 | E | validated | |
| ICD-10 | L98.2 | E | specific | validated |
| ICD-11 | EB20 | E | specific | validated |
| MONDO | 0011959 | E | validated | |
| MeSH | D016463 | E | validated | |
| UMLS | C0085077 | E | validated | |
| GARD | 521 | E | validated | |
| MedDRA | 10000748 | 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 (5):
- ORPHA:319719Autoinflammatory syndrome of childhood
rare systemic or rheumatologic diseases of childhood - ORPHA:290842Autoinflammatory syndrome with skin involvement
rare skin diseases - ORPHA:622720Genetic autoinflammatory syndrome with skin involvement
rare genetic diseases - ORPHA:324927Pyogenic autoinflammatory syndrome
rare systemic and rheumatological diseases - ORPHA:619238Rare hereditary autoinflammatory disease
rare genetic diseases
No children.
Genes
2 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| MEFV | Disease-causing germline mutation(s) in | Assessed | HGNC:6998 | ENSG00000103313 | 608107 | 16p13.3 | MEFV innate immunity regulator, pyrin | 28835462[PMID] |
| PTPN6 | Major susceptibility factor in | Assessed | HGNC:9658 | ENSG00000111679 | 176883 | 12p13.31 | protein tyrosine phosphatase non-receptor type 6 | 21406173[PMID] |
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET_17655751[PMID] |
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
39 annotations · validated 2020-12-10| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0011117 | Abnormal circulating interleukin concentration | |
| HP:0030350 | Erythematous papule | |
| HP:0025474 | Erythematous plaque | |
| HP:0012531 | Pain | |
| HP:0031236 | Predominantly dermal neutrophilic infiltrate | |
| HP:0200036 | Skin nodule | |
| Frequent (79-30%) | ||
| HP:0000271 | Abnormality of the face | |
| HP:0001155 | Abnormality of the hand | |
| HP:0000464 | Abnormality of the neck | |
| HP:0011118 | Abnormality of tumor necrosis factor secretion | |
| HP:0001061 | Acne | |
| HP:0002829 | Arthralgia | |
| HP:0011227 | Elevated circulating C-reactive protein concentration | |
| HP:0003565 | Elevated erythrocyte sedimentation rate | |
| HP:0030783 | Increased circulating interleukin 6 concentration | |
| HP:0001974 | Increased total leukocyte count | |
| HP:0003326 | Myalgia | |
| HP:0100614 | Myositis | |
| HP:0011897 | Neutrophilia | |
| HP:0032324 | Non-periodic recurrent fever | |
| HP:0011944 | Small vessel vasculitis | |
| HP:0025616 | Sterile abscess | |
| Occasional (29-5%) | ||
| HP:0008066 | Abnormal blistering of the skin | |
| HP:0020169 | Abnormal drug response | |
| HP:0040154 | Acne inversa | |
| HP:0004808 | Acute myeloid leukemia | |
| HP:0001903 | Anemia | |
| HP:0004377 | Hematological neoplasm | |
| HP:0002037 | Inflammation of the large intestine | |
| HP:0002664 | Neoplasm | |
| HP:0040313 | Oligoarthritis | |
| HP:0012490 | Panniculitis | |
| HP:0200039 | Pustule | |
| HP:0025452 | Pyoderma gangrenosum | |
| HP:0002719 | Recurrent infections | |
| HP:0200037 | Skin vesicle | |
| Very rare (<4-1%) | ||
| HP:0003002 | Breast carcinoma | |
| HP:0005550 | Chronic lymphatic leukemia | |
| HP:0001644 | Dilated cardiomyopathy | |
Run the differential on the 6 obligate / very frequent terms
Annotation source: 27030597[PMID]_28835462[PMID]_28981179[PMID]_30734344[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/3243
- This record as JSON: /data/d/3243.json