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DOI: 10.1055/s-0028-1109423
© Georg Thieme Verlag KG Stuttgart · New York
Pregnancy-associated Sweet’s Syndrome in an Acute Episode of Ulcerative Colitis
Sweet-Syndrom in der SchwangerschaftPublikationsverlauf
manuscript received: 28.9.2008
manuscript accepted: 3.4.2009
Publikationsdatum:
06. August 2009 (online)

Zusammenfassung
Eine im 2. Trimenon schwangere 33-jährige Patientin (15. SSW) mit einer seit Geburt des 1. Kindes bekannten Colitis ulcerosa stellte sich in unserer Klinik aufgrund von neu aufgetretenem, progredientem Gewichtsverlust, Fieber, Leukozytose und blutig/schleimig tingierten Diarrhöen vor. Endoskopisch konnte ein akuter Schub der bekannten Colitis ulcerosa nachgewiesen werden. Wenige Tage später manifestierten sich zusätzlich kutane erythematöse Knötchen und Plaques, anhand von Hautbiopsien wurde die klinische Verdachtsdiagnose einer neutrophilen Dermatitis (Sweet-Syndrom) histologisch gesichert. Eine unverzüglich initiierte, hoch dosierte intravenöse Steroidstoßtherapie erzielte eine unmittelbare Remission, sowohl der kutanen als auch der intestinalen Manifestationen. Beide Krankheitsentitäten weisen eng miteinander assoziierte immunologische Alterationen auf. Pathogenetisch bestehen sowohl bei den Aktivierungsmustern auf Zytokin- und Chemokinebene als auch bei der Aktivierung inflammatorischer Zellreihen signifikante Parallelen. Aktuelle Daten belegen, dass es im Rahmen der Gravidität zu einer Elevation proinflammatorischer Zytokinspiegel kommen kann. Dieses Phänomen spielt möglicherweise eine Schlüsselrolle, sowohl bei der Manifestation intestinaler und extraintestinaler Symptome als auch für den Schweregrad des Krankheitsverlaufs. Im Folgenden werden ein Diagnostikalgorithmus und ein pathogenetischer Erklärungsansatz für das Sweet-Syndrom diskutiert.
Abstract
A 33-year old pregnant patient (pregnancy week 15) with a past medical history of ulcerative colitis with onset of the disease following the birth of her first child was admitted to the hospital with symptoms of weight loss, pyrexia, leukocytosis and bloody and mucous diarrhoea. Total ileocolonoscopy revealed an acute flare of ulcerative colitis. Within a few days, tender erythematous skin lesions occurred and were histologically proven to be neutrophilic dermatosis. Treatment with highly-dosed prednisone led to a complete remission of both cutaneous and intestinal manifestations. Both pathogenic entities are associated with similar immunological alterations, such as comparable cytokine and chemokine release patterns and recruitment of inflammatory cells. Recent data also indicates that proinflammatory cytokine levels are elevated in pregnancy, which might be pivotal in the pathogenesis and the severity of intestinal and extraintestinal symptoms. We present and discuss a diagnostic algorithm and an overall therapeutic rationale for Sweet’s syndrome.
Schlüsselwörter
Colitis ulcerosa - neutrophile Dermatose - Schwangerschaft - Zytokine - IL-6 - G-CSF - AFP - CRP - Inflammation
Key words
inflammatory bowel disease - neutrophilic dermatosis - pregnancy - cytokines - IL-6 - G-CSF - AFP - CRP - inflammation
References
- 1
Cohen P R.
Sweet’s syndrome – a comprehensive review of an acute febrile neutrophilic dermatosis.
Orphanet J Rare Dis.
2007;
2
34
MissingFormLabel
- 2
Canbay A, Runzi M, Gieseler R. et al .
Generalized pustulous psoriasis: A novel extraintestinal manifestation of Crohn’s
disease?.
Z Gastroenterol.
2001;
39
801-805
MissingFormLabel
- 3
Yates V M, Watkinson G, Kelman A.
Further evidence for an association between psoriasis, Crohn’s disease and ulcerative
colitis.
Br J Dermatol.
1982;
106
323-330
MissingFormLabel
- 4
Cohen P R, Kurzrock R.
The pathogenesis of Sweet’s syndrome.
J Am Acad Dermatol.
1991;
25
734
MissingFormLabel
- 5
Su W P, Liu H N.
Diagnostic criteria for Sweet’s syndrome.
Cutis.
1986;
37
167-174
MissingFormLabel
- 6
Ramsey-Goldman R, Schilling E.
Immunosuppressive drug use during pregnancy.
Rheum Dis Clin North Am.
1997;
23
149-167
MissingFormLabel
- 7
Langagergaard V, Pedersen L, Gislum M. et al .
Birth outcome in women treated with azathioprine or mercaptopurine during pregnancy:
A Danish nationwide cohort study.
Aliment Pharmacol Ther.
2007;
25
73-81
MissingFormLabel
- 8
Johnson P J, McFarlane I G, Williams R.
Azathioprine for long-term maintenance of remission in autoimmune hepatitis.
N Engl J Med.
1995;
333
958-963
MissingFormLabel
- 9
Vaccaro M, Guarneri F, Guarneri C. et al .
Sweet’s syndrome and erythema nodosum after Klebsiella pneumoniae cystitis.
Acta Derm Venereol.
2003;
83
290-291
MissingFormLabel
- 10
Travis S, Innes N, Davies M G. The South West Gastroenterology Group. .
Sweet’s syndrome: an unusual cutaneous feature of Crohn’s disease or ulcerative colitis.
Eur J Gastroenterol Hepatol.
1997;
9
715-720
MissingFormLabel
- 11
Arai H, Rino Y, Yamanaka S. et al .
Lung cancer associated with Sweet’s syndrome: report of a case.
Surg Today.
2008;
38
639-643
MissingFormLabel
- 12
Cohen P R, Talpaz M, Kurzrock R.
Malignancy-associated Sweet’s syndrome: review of the world literature.
J Clin Oncol.
1988;
6
1887-1897
MissingFormLabel
- 13
Buck T, Gonzalez L M, Lambert W C. et al .
Sweet’s syndrome with hematologic disorders: a review and reappraisal.
Int J Dermatol.
2008;
47
775-782
MissingFormLabel
- 14
Thompson D F, Montarella K E.
Drug-induced Sweet’s syndrome.
Ann Pharmacother.
2007;
41
802-811
MissingFormLabel
- 15
Becuwe C, Delaporte E, Colombel J F. et al .
Sweet’s syndrome associated with Crohn’s disease.
Acta Derm Venereol.
1989;
69
444-445
MissingFormLabel
- 16
Malheiros A P, Teixeira M G, Takahashi M D. et al .
Sweet syndrome associated with ulcerative colitis.
Inflamm Bowel Dis.
2007;
13
1583-1584
MissingFormLabel
- 17
Cohen P R.
Pregnancy-associated Sweet’s syndrome: world literature review.
Obstet Gynecol Surv.
1993;
48
584-587
MissingFormLabel
- 18
Metcalf D.
The molecular control of cell division, differentiation commitment and maturation
in haemopoietic cells.
Nature.
1989;
339
27-30
MissingFormLabel
- 19
Wegmann T G, Athanassakis I, Guilbert L. et al .
The role of M-CSF and GM-CSF in fostering placental growth, fetal growth, and fetal
survival.
Transplant Proc.
1989;
21
566-568
MissingFormLabel
- 20
Paoluzi O A, Crispino P, Amantea A. et al .
Diffuse febrile dermatosis in a patient with active ulcerative colitis under treatment
with steroids and azathioprine: a case of Sweet’s syndrome. Case report and review
of literature.
Dig Liver Dis.
2004;
36
361-366
MissingFormLabel
- 21
Inglot A D, Gelder F, Georgiades J A.
Tumor-associated antigens are cytokine inducers and hyporeactivity factors to the
immune system.
Biotherapy.
1998;
11
27-37
MissingFormLabel
- 22
Filella X, Molina R, Alcover J. et al .
Influence of AFP, CEA and PSA on the in vitro production of cytokines.
Tumour Biol.
2001;
22
67-71
MissingFormLabel
- 23
Weimann E, Reisbach G, Reinsberg J. et al .
IL-6 and G-CSF levels in amniotic fluid during the second trimester in normal and
abnormal pregnancies.
Arch Gynecol Obstet.
1995;
256
125-130
MissingFormLabel
- 24
Reinecker H C, Steffen M, Witthoeft T. et al .
Enhanced secretion of tumour necrosis factor-alpha, IL-6, and IL-1 beta by isolated
lamina propria mononuclear cells from patients with ulcerative colitis and Crohn’s
disease.
Clin Exp Immunol.
1993;
94
174-181
MissingFormLabel
- 25
Reuss-Borst M A, Muller C A, Waller H D.
The possible role of G-CSF in the pathogenesis of Sweet’s syndrome.
Leuk Lymphoma.
1994;
15
261-264
MissingFormLabel
- 26
Reuss-Borst M A, Pawelec G, Saal J G. et al .
Sweet’s syndrome associated with myelodysplasia: possible role of cytokines in the
pathogenesis of the disease.
Br J Haematol.
1993;
84
356-358
MissingFormLabel
- 27
Shochat E, Rom-Kedar V, Segel L A.
G-CSF control of neutrophils dynamics in the blood.
Bull Math Biol.
2007;
69
2299-2338
MissingFormLabel
- 28
Le J M, Vilcek J.
Interleukin 6: a multifunctional cytokine regulating immune reactions and the acute
phase protein response.
Lab Invest.
1989;
61
588-602
MissingFormLabel
- 29
Ohzato H, Yoshizaki K, Nishimoto N. et al .
Interleukin-6 as a new indicator of inflammatory status: detection of serum levels
of interleukin-6 and C-reactive protein after surgery.
Surgery.
1992;
111
201-209
MissingFormLabel
- 30
Castellheim A, Thorgersen E B, Hellerud B C. et al .
New biomarkers in an acute model of live Escherichia coli-induced sepsis in pigs.
Scand J Immunol.
2008;
68
75-84
MissingFormLabel
- 31
Prat C, Sancho J M, Dominguez J. et al .
Evaluation of procalcitonin, neopterin, C-reactive protein, IL-6 and IL-8 as a diagnostic
marker of infection in patients with febrile neutropenia.
Leuk Lymphoma.
2008;
49
1752-1761
MissingFormLabel
- 32
Henriksen M, Jahnsen J, Lygren I. et al .
C-reactive protein: a predictive factor and marker of inflammation in inflammatory
bowel disease. Results from a prospective population-based study.
Gut.
2008;
57
1518-1523
MissingFormLabel
- 33
Kawakami T, Ohashi S, Kawa Y. et al .
Elevated serum granulocyte colony-stimulating factor levels in patients with active
phase of sweet syndrome and patients with active behcet disease: implication in neutrophil
apoptosis dysfunction.
Arch Dermatol.
2004;
140
570-574
MissingFormLabel
PD Dr. Ali Canbay
Gastroenterology and Hepatology, University Hospital Essen
Hufelandstr. 55
45122 Essen
Telefon: + + 49/2 01/7 23 36 11
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eMail: ali.canbay@uni-essen.de