Sweet syndrome

General Information (adopted from Orphanet):

Synonyms, Signs: GOMM-BUTTON DISEASE
SWEET SYNDROME
SS
AFND
Acute febrile neutrophilic dermatosis
Number of Symptoms 33
OrphanetNr: 3243
OMIM Id: 608068
ICD-10: L98.2
UMLs: C0085077
MeSH: D016463
MedDRA: 10000748
Snomed: 84625002

Prevalence, inheritance and age of onset:

Prevalence: > 100 cases [Orphanet]
Inheritance: Unknown
[Orphanet]
Age of onset: Adult
[Orphanet]

Disease classification (adopted from Orphanet):

Parent Diseases: Other acquired skin disease
 -Rare skin disease

Symptom Information: Sort by abundance 

1
 (HPO:0000083) Renal insufficiency Occasional [Orphanet] 232 / 7739
2
 (HPO:0100820) Glomerulopathy Occasional [Orphanet] 46 / 7739
3
 (HPO:0000790) Hematuria Occasional [Orphanet] 106 / 7739
4
 (HPO:0000093) Proteinuria Occasional [Orphanet] 169 / 7739
5
 (HPO:0011830) Abnormality of oral mucosa Occasional [Orphanet] 47 / 7739
6
 (HPO:0100533) Inflammatory abnormality of the eye Occasional [Orphanet] 70 / 7739
7
 (HPO:0002315) Headache Very frequent [Orphanet] 175 / 7739
8
 (HPO:0000924) Abnormality of the skeletal system Frequent [Orphanet] 114 / 7739
9
 (HPO:0005059) Arthralgia/arthritis Very frequent [Orphanet] 141 / 7739
10
 (HPO:0002024) Malabsorption Occasional [Orphanet] 142 / 7739
11
 (HPO:0006568) Increased hepatic glycogen content Very frequent [Orphanet] 34 / 7739
12
 (HPO:0001744) Splenomegaly Very frequent [Orphanet] 337 / 7739
13
 (HPO:0200042) Skin ulcer Very frequent [Orphanet] 138 / 7739
14
 (HPO:0010783) Erythema Very frequent [Orphanet] 138 / 7739
15
 (HPO:0200037) Skin vesicle Occasional [Orphanet] 102 / 7739
16
 (HPO:0200039) Pustule Occasional [Orphanet] 20 / 7739
17
 (HPO:0000962) Hyperkeratosis Frequent [Orphanet] 216 / 7739
18
 (HPO:0001031) Subcutaneous lipoma Very frequent [Orphanet] 112 / 7739
19
 (HPO:0000988) Skin rash Very frequent [Orphanet] 98 / 7739
20
 (HPO:0001873) Thrombocytopenia Occasional [Orphanet] 224 / 7739
21
 (HPO:0001903) Anemia Occasional [Orphanet] 289 / 7739
22
 (HPO:0001974) Leukocytosis Very frequent [Orphanet] 33 / 7739
23
 (HPO:0004447) Poikilocytosis Frequent [Orphanet] 16 / 7739
24
 (HPO:0011227) Elevated C-reactive protein level Very frequent [Orphanet] 55 / 7739
25
 (HPO:0001945) Fever Very frequent [Orphanet] 218 / 7739
26
 (HPO:0002113) Pulmonary infiltrates Occasional [Orphanet] 36 / 7739
27
 (HPO:0002205) Recurrent respiratory infections Occasional [Orphanet] 254 / 7739
28
 (HPO:0001324) Muscle weakness Very frequent [Orphanet] 859 / 7739
29
 (HPO:0003326) Myalgia Very frequent [Orphanet] 143 / 7739
30
 (HPO:0030350) Erythematous papule Very frequent [Orphanet] 123 / 7739
31
 (OMIM) Stiff skin Frequent [Orphanet] 31 / 7739
32
 ([DEL]MedDRA:10011224) Cough Occasional [Orphanet] 70 / 7739
33
 (MedDRA:10025421) Macule Very frequent [Orphanet] 55 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information:

Diagnosis OMIM Su and Liu (1986) proposed 2 major and 4 minor criteria for diagnosis of Sweet syndrome. They suggested fulfillment of both of the major criteria (abrupt onset of tender or painful erythematous or violaceous plaques or nodules; predominantly ...
Clinical Description OMIM Sweet (1964) described 8 patients, all women, with an acute dermatosis characterized by fever, neutrophilia, painful erythematous plaques, a dense dermal infiltrate of mature neutrophils, and rapid response to corticosteroids. In 7 of the patients the condition was ...
Molecular genetics OMIM Mizoguchi et al. (1988) reported a Japanese patient with Sweet syndrome with symptoms similar to those of Behcet disease and performed HLA typing on 28 patients with Sweet syndrome and 49 patients with Behcet disease. They found that ...