350:) can produce rapid improvement and are the “gold standard” for treatment. The temperature, white blood cell count, and eruption improve within 72 hours. The skin lesions clear within 3 to 9 days. Abnormal laboratory values rapidly return to normal. There are, however, frequent recurrences. Corticosteroids are tapered within 2 to 6 weeks to zero.Resolution of the eruption is occasionally followed by milia and scarring. The disease clears spontaneously in some patients. Topical and/or intralesional corticosteroids may be effective as either monotherapy or adjuvant therapy. Oral
362:, 150 mg per day, was given for the first week, and 100 mg per day was given for 2 additional weeks. Seventeen of 18 patients had a good initial response; fever and arthralgias were markedly attenuated within 48 hours, and eruptions cleared between 7 and 14 days. Patients whose cutaneous lesions continued to develop were successfully treated with prednisone (1 mg/kg per day). No patient had a relapse after discontinuation of indomethacin. Other alternatives to corticosteroid treatment include
64:
132:
144:
40:
196:—can cause SS. Lesions typically occur when the patient has leukocytosis and neutrophilia but not when the patient is neutropenic. However, G-CSF may cause SS in neutropenic patients because of the induction of stem cell proliferation, the differentiation of neutrophils, and the prolongation of neutrophil survival.
334:. This led to the name acute febrile neutrophilic dermatosis. Larger series of patients showed that fever and neutrophilia are not consistently present. The diagnosis is based on the two constant features, a typical eruption and the characteristic histologic features; thus the eponym "Sweet's syndrome" is used.
314:
within the vessel walls; red blood cell extravasation;inflammatory infiltration of vascular walls) are absent in early lesions.Perivasculitis occurs secondarily, because of cytokines released by the lesional neutrophils. True transmural vasculitis is not an expected finding histopathologically in SS.
151:
Acute, tender, erythematous plaques, nodes, pseudovesicles and, occasionally, blisters with an annular or arciform pattern occur on the head, neck, legs, and arms, particularly the back of the hands and fingers. The trunk is rarely involved. Fever (50%); arthralgia or arthritis (62%); eye
168:
SS is a reactive phenomenon and should be considered a cutaneous marker of systemic disease. Careful systemic evaluation is indicated, especially when cutaneous lesions are severe or hematologic values are abnormal. Approximately 20% of cases are associated with malignancy, predominantly
262:(HLA) types B54 and Cw1 are notably high, indicating their association with the condition. However, it is suggested that multiple risk factors, including these, are involved in the onset of the disease. While the more frequent HLA type associated with a related disorder,
302:(83%). Skin biopsy shows a papillary and mid-dermal mixed infiltrate of polymorphonuclear leukocytes with nuclear fragmentation and histiocytic cells. The infiltrate is predominantly perivascular with endothelial-cell swelling in some vessels, but vasculitic changes (
691:
676:
253:
In SS, inflammatory lesions are known to occur not only on the skin but also in various organs. When complications such as encephalitis or meningitis occur, it is referred to as neuro-Sweet disease. It is known that treatment with
185:, solid tumors, pregnancy) is found in up to 50% of cases. Attacks of SS may precede the hematologic diagnosis by 3 months to 6 years, so that close evaluation of patients in the “idiopathic” group is required.
1228:
1595:
358:
may induce rapid resolution.Patients who have a potential systemic infection or in whom corticosteroids are contraindicated can use these agents as a first-line therapy. In one study,
1221:
638:
Hisanaga K, Iwasaki Y, Itoyama Y, the neuro-Sweet disease study group (2005). “Neuro-Sweet disease": Clinical manifestations and criteria for diagnosis. Neurology. 64: 1756-1761.
1846:
1457:
1565:
1214:
247:
Pregnancy associated Sweet syndrome typically present in first or second trimester. It may recur with subsequent pregnancy, but there seems to be no risk to the fetus.
193:
767:
1266:
604:
Parsapour K, Reep MD, Gohar K, Shah V, Church A, Shwayder TA (July 2003). "Familial Sweet's syndrome in 2 brothers, both seen in the first 2 weeks of life".
1473:
1323:
1382:
160:
SS can be classified based upon the clinical setting in which it occurs: classical or idiopathic SS, malignancy-associated SS, and drug-induced SS.
147:
Punch biopsy of a skin lesion showing neutrophilic infiltration in the dermis, with no evidence of vasculitis (same patient with Crohn's disease).
1765:
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1000:
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152:
involvement, most frequently conjunctivitis or iridocyclitis (38%); and oral aphthae (13%) are associated features.
123:. It was also known as Gomm–Button disease in honour of the first two patients Sweet diagnosed with the condition.
1377:
1292:
947:
902:
237:
213:
178:
170:
1528:
1307:
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647:
Hisanaga K (2022). “Neuro-Behçet disease, neuro-Sweet disease, and spectrum disorders.” Intern Med. 61: 447-450.
1816:
1367:
1047:
717:
266:, is different (B51), it is believed to form a spectrum of disorders with other shared predisposing factors.
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135:
Pustular lesions with central necrosis on the left leg of a patient with Sweet's syndrome associated with
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120:
109:
105:
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323:
Sweet described a disease with four features: fever; leukocytosis; acute, tender, red plaques; and a
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63:
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351:
250:
A genetic association has been suggested, but no specific genetic link has been identified.
221:
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1005:
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343:
255:
204:
Although it may occur in the absence of other known disease, SS is often associated with
499:
1590:
1327:
1136:
1076:
1020:
700:
589:
563:"Sweet's syndrome – a comprehensive review of an acute febrile neutrophilic dermatosis"
562:
524:
446:
419:
311:
188:
There is now good evidence that treatment with hematopoietic growth factors—including
1835:
1508:
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225:
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540:
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685:
355:
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229:
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17:
378:. All of these drugs influence migration and other functions of neutrophils.
1638:
1405:
1105:
436:
283:
113:
1543:
625:
579:
532:
455:
324:
1724:
1030:
978:
617:
387:
209:
745:
668:
1039:
400:, also known as autoinflammatory diseases or autoinflammatory syndromes
363:
101:
1428:
680:
327:
307:
142:
130:
89:
286:. Recurrences are common and affect up to one third of patients.
47:
Sweet syndrome lesions with the classical form of the dermatosis.
1034:
711:
1547:
1210:
749:
420:"Sweet syndrome in a patient with Crohn disease: a case report"
294:
Studies show a moderate neutrophilia (less than 50%), elevated
511:
Sweet RD (1964). "An acute febrile neutrophilic dermatosis".
471:
Andrews
Diseases of the Skin: Clinical Dermatology (10th ed.)
298:(greater than 30 mm/h) (90%), and a slight increase in
258:
often leads to favorable outcomes, and the frequencies of
1509:
Pyogenic arthritis–pyoderma gangrenosum–acne syndrome
658:
1799:
1746:
1722:
1713:
1690:
1637:
1614:
1581:
1486:
1466:
1436:
1427:
1404:
1395:
1316:
1275:
1251:
1242:
1165:
1029:
911:
830:
807:
784:
662:
51:
32:
192:(G-CSF), which is used to treat neutropenia, and
194:granulocyte-macrophage colony-stimulating factor
1458:Bowel-associated dermatosis–arthritis syndrome
1559:
1267:Eosinophilic granulomatosis with polyangiitis
1222:
761:
274:The clinical differential diagnosis includes
8:
556:
554:
552:
550:
119:The syndrome was first described in 1964 by
1474:Neutrophilic dermatosis of the dorsal hands
1324:Angiolymphoid hyperplasia with eosinophilia
1847:Reactive neutrophilic cutaneous conditions
1719:
1566:
1552:
1544:
1433:
1401:
1248:
1229:
1215:
1207:
768:
754:
746:
659:
62:
38:
29:
588:
578:
445:
435:
1383:Pruritic papular eruption of HIV disease
1237:Neutrophilic and eosinophilic dermatoses
410:
1766:Acrokeratosis paraneoplastica of Bazex
1647:Paraneoplastic cerebellar degeneration
1373:Pachydermatous eosinophilic dermatitis
469:James, W; Berger, T; Elston D (2005).
1348:Eosinophilic ulcer of the oral mucosa
190:granulocyte colony-stimulating factor
88:characterized by the sudden onset of
82:acute febrile neutrophilic dermatosis
7:
1629:Nonbacterial thrombotic endocarditis
1089:Hydroxyethyl starch-induced pruritus
181:, nonlymphocytic leukemia and other
853:Urushiol-induced contact dermatitis
1624:Multicentric reticulohistiocytosis
1519:Superficial granulomatous pyoderma
1514:Rheumatoid neutrophilic dermatitis
926:Autoimmune progesterone dermatitis
525:10.1111/j.1365-2133.1964.tb14541.x
25:
1677:Lambert–Eaton myasthenic syndrome
1504:Neutrophilic eccrine hidradenitis
567:Orphanet Journal of Rare Diseases
561:Cohen, Philip R (December 2007).
1524:Sweet's syndrome-like dermatosis
1397:Reactive neutrophilic dermatoses
173:(AML). An underlying condition (
1807:Febrile neutrophilic dermatosis
1776:Florid cutaneous papillomatosis
1682:Anti-NMDA receptor encephalitis
94:elevated white blood cell count
1419:Subcorneal pustular dermatosis
963:Hyperkeratotic hand dermatitis
921:Autoimmune estrogen dermatitis
418:Mustafa NM, Lavizzo M (2008).
282:, adverse drug reactions, and
1:
1738:Necrolytic migratory erythema
1172:substances taken internally:
1011:Nutritional deficiency eczema
817:Pityriasis simplex capillitii
216:), solid cancers (especially
1771:Extramammary Paget's disease
970:Autosensitization dermatitis
858:African blackwood dermatitis
393:List of cutaneous conditions
104:and plaques that show dense
1414:Keratoderma blennorrhagicum
1353:Erythema toxicum neonatorum
1333:Annular erythema of infancy
1298:Papuloerythroderma of Ofuji
1196:Papuloerythroderma of Ofuji
1001:Juvenile plantar dermatosis
1873:
1601:Zollinger–Ellison syndrome
1378:Papular eruption of blacks
1293:Hypereosinophilic syndrome
948:Topical steroid withdrawal
903:Protein contact dermatitis
238:inflammatory bowel disease
214:acute myelogenous leukemia
179:inflammatory bowel disease
171:acute myelogenous leukemia
169:hematological, especially
1529:Vesicopustular dermatosis
1308:Eosinophilic folliculitis
1016:Sulzberger–Garbe syndrome
473:. Saunders. p. 145.
46:
37:
1817:Paraneoplastic pemphigus
1575:Paraneoplastic syndromes
1368:Juvenile xanthogranuloma
1048:Lichen simplex chronicus
183:hematologic malignancies
1733:Erythema gyratum repens
1705:Hypertrophic osteopathy
1288:Eosinophilic cellulitis
1262:Eosinophilic vasculitis
1244:Eosinophilic dermatosis
873:Abietic acid dermatitis
437:10.1186/1752-1947-2-221
398:Periodic fever syndrome
260:human leukocyte antigen
175:streptococcal infection
110:neutrophil granulocytes
1494:Acute erythema nodosum
1363:Itchy red bump disease
1358:Incontinentia pigmenti
1343:Eosinophilic granuloma
1338:Eosinophilic fasciitis
580:10.1186/1750-1172-2-34
264:neuro-Behçet's disease
148:
140:
1084:Drug-induced pruritus
809:Seborrheic dermatitis
606:J. Am. Acad. Dermatol
146:
134:
1812:Pyoderma gangrenosum
1761:Ichthyosis acquisita
1756:Acanthosis nigricans
1453:Pyoderma gangrenosum
1130:Cholestatic pruritus
1120:due to liver disease
989:Molluscum dermatitis
618:10.1067/mjd.2003.328
300:alkaline phosphatase
276:pyoderma gangrenosum
234:rheumatoid arthritis
121:Robert Douglas Sweet
1672:Transverse myelitis
1657:Limbic encephalitis
1186:Nummular dermatitis
1179:Fixed drug reaction
996:Circumostomy eczema
794:Prurigo gestationis
280:erythema multiforme
232:diseases including
208:disease (including
1786:Pityriasis rotunda
1606:Cushing's syndrome
1276:Without vasculitis
1142:Prurigo pigmentosa
1101:Aquagenic pruritus
893:Contact stomatitis
832:Contact dermatitis
290:Laboratory studies
149:
141:
127:Signs and symptoms
100:, well-demarcated
1829:
1828:
1825:
1824:
1781:Leser-Trélat sign
1652:Encephalomyelitis
1541:
1540:
1537:
1536:
1499:Marshall syndrome
1482:
1481:
1391:
1390:
1303:Granuloma faciale
1283:Arthropod assault
1204:
1203:
1115:Adult blaschkitis
1052:Prurigo nodularis
943:Eyelid dermatitis
883:Airbag dermatitis
786:Atopic dermatitis
743:
742:
480:978-0-7216-2921-6
310:, complement, or
242:Behçet's syndrome
164:Systemic diseases
71:
70:
27:Medical condition
16:(Redirected from
1864:
1720:
1568:
1561:
1554:
1545:
1467:with vasculitis:
1448:Sweet's syndrome
1434:
1402:
1328:Kimura's disease
1249:
1231:
1224:
1217:
1208:
1152:Puncta pruritica
1125:Biliary pruritus
770:
763:
756:
747:
660:
648:
645:
639:
636:
630:
629:
601:
595:
594:
592:
582:
558:
545:
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491:
485:
484:
466:
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459:
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439:
415:
352:potassium iodide
306:; deposition of
222:gastrointestinal
212:, most commonly
67:
66:
42:
30:
21:
1872:
1871:
1867:
1866:
1865:
1863:
1862:
1861:
1832:
1831:
1830:
1821:
1795:
1742:
1709:
1700:Dermatomyositis
1692:Musculoskeletal
1686:
1633:
1610:
1577:
1572:
1542:
1533:
1478:
1462:
1423:
1387:
1312:
1271:
1238:
1235:
1205:
1200:
1191:Pityriasis alba
1161:
1157:Uremic pruritus
1147:Prurigo simplex
1096:Senile pruritus
1072:Pruritus vulvae
1067:Pruritus scroti
1038:
1025:
1006:Nummular eczema
907:
888:Baboon syndrome
834:
826:
803:
780:
774:
744:
739:
738:
671:
657:
652:
651:
646:
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637:
633:
603:
602:
598:
560:
559:
548:
519:(8–9): 349–56.
513:Br. J. Dermatol
510:
509:
505:
492:
488:
481:
468:
467:
463:
417:
416:
412:
407:
384:
344:corticosteroids
340:
321:
312:immunoglobulins
292:
272:
256:corticosteroids
202:
166:
158:
137:Crohn's disease
129:
61:
28:
23:
22:
15:
12:
11:
5:
1870:
1868:
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1809:
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1793:
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1748:Papulosquamous
1744:
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1591:Hypercalcaemia
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1176:
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1167:
1163:
1162:
1160:
1159:
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1149:
1144:
1139:
1137:Prion pruritus
1134:
1133:
1132:
1127:
1117:
1111:
1110:
1109:
1108:
1098:
1093:
1092:
1091:
1080:
1079:
1077:Scalp pruritus
1074:
1069:
1064:
1055:
1054:
1044:
1042:
1027:
1026:
1024:
1023:
1021:Xerotic eczema
1018:
1013:
1008:
1003:
998:
993:
992:
991:
986:
981:
967:
966:
965:
960:
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929:
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828:
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819:
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758:
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688:
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664:
663:Classification
656:
655:External links
653:
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486:
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424:J Med Case Rep
409:
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339:
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330:infiltrate of
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165:
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128:
125:
96:, and tender,
74:Sweet syndrome
69:
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49:
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44:
43:
35:
34:
33:Sweet syndrome
26:
24:
18:Sweet syndrome
14:
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3:
2:
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1857:Rare diseases
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1715:Mucocutaneous
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1616:Hematological
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984:Dermatophytid
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933:Breast eczema
931:
930:
927:
924:
922:
919:
918:
916:
914:
910:
904:
901:
899:
898:Metal allergy
896:
894:
891:
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886:
884:
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871:
868:
867:
864:
863:Tulip fingers
861:
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842:
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829:
823:
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799:Sweat allergy
797:
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500:Who Named It?
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277:
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226:breast cancer
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19:
1806:
1667:Polymyositis
1639:Neurological
1447:
1119:
1062:Pruritus ani
1059:by location:
1058:
869:
849:
727:
716:
705:
690:
675:
643:
634:
612:(1): 132–8.
609:
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570:
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489:
470:
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427:
423:
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376:cyclosporine
360:indomethacin
341:
322:
293:
273:
252:
249:
246:
203:
200:Associations
187:
167:
159:
150:
118:
86:skin disease
81:
77:
73:
72:
1791:Tripe palms
974:Id reaction
958:Dyshidrosis
953:Hand eczema
878:Diaper rash
372:clofazimine
368:doxycycline
332:neutrophils
304:blood clots
230:immunologic
206:hematologic
106:infiltrates
58:Dermatology
1836:Categories
1662:Opsoclonus
1439:vasculitis
1254:vasculitis
1174:Bromoderma
938:Ear eczema
822:Cradle cap
779:and eczema
777:Dermatitis
729:DiseasesDB
405:References
356:colchicine
348:prednisone
319:Definition
114:histologic
1852:Syndromes
1842:Erythemas
1723:Reactive
1583:Endocrine
1487:Ungrouped
1406:Epidermis
1317:Ungrouped
1106:Aquadynia
495:synd/3019
346:such as (
342:Systemic
338:Treatment
325:papillary
284:urticaria
270:Diagnosis
53:Specialty
1725:erythema
1437:without
1031:Pruritus
979:Candidid
841:irritant
837:allergic
626:12833027
541:53772268
533:14201182
456:18588703
388:Chloroma
382:See also
210:leukemia
1040:Prurigo
850:plants:
723:D016463
590:1963326
447:2503996
430:: 221.
364:dapsone
102:papules
84:, is a
1429:Dermis
913:Eczema
870:other:
712:608068
701:695.89
624:
587:
539:
531:
477:
454:
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374:, and
328:dermal
308:fibrin
228:) and
80:), or
60:
1800:Other
1596:SIADH
1252:With
1166:Other
734:12727
686:L98.2
573:(1).
537:S2CID
156:Cause
92:, an
90:fever
1035:Itch
718:MeSH
707:OMIM
696:9-CM
622:PMID
529:PMID
475:ISBN
452:PMID
224:and
692:ICD
677:ICD
614:doi
585:PMC
575:doi
521:doi
498:at
442:PMC
432:doi
354:or
296:ESR
112:on
108:by
98:red
1838::
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684::
681:10
620:.
610:49
608:.
583:.
569:.
565:.
549:^
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527:.
517:76
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440:.
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220:,
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78:SS
1567:e
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679:-
669:D
628:.
616::
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577::
571:2
543:.
523::
483:.
458:.
434::
428:2
139:.
76:(
20:)
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