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Sweet-Syndrom

Zuletzt geändert von Thomas Brinkmeier am 2025/08/26 19:50

Sweet-Syndrom

Histr: R.D. Sweet, 1964

Syn: akute febrile neutrophile Dermatose

Engl: sweet's syndrome, acute febrile neutrophilic dermatosis

Ät: unbekannt

Pg: unbekannt

TF: - Infekte

Amn: Prodrome (1-3 Wochen vorher)

KL: Diarrhoe, Tonsillitis, grippeähnliches Krankheitsbild

Bsp: - Hepatitis C

Lit: J Cutan Med Surg. 2014 Nov;18(6):436-8

- Burkholderia pseudomallei

Lit: BMC Dermatol. 2019 Dec 2;19(1):16. doi: 10.1186/s12895-019-0096-2 (Erstbeschreibung)  

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- Sporotrichose

Lit: An Bras Dermatol. 2024 Jun 7:S0365-0596(24)00094-1. http://doi.org/10.1016/j.abd.2024.03.001

- Malignome

- Impfungen

Bsp: - BCG

- Influenza

- COVID-19

Lit: JAAD Case Rep. 2022 Jan;19:64-67. http://doi.org/10.1016/j.jdcr.2021.11.010

- Medikamente

Stoff: Azathioprin, Bortezomib, Celecoxib, Chloroquin, Clindamycin (auch topisch), GCSF, Hydralazin, Imatinib, Interferon beta-1b, Ipilimumab, Ketoconazol, orale Kontrazeptiva, Minocyclin, Pegfilgrastim, Piperacillin/Tazobactam, Trimethoprim Sulfamethoxazol, Vemurafenib, Tocilizumab, Erlotinib, Lamotrigin

- Trauma der Haut

Lit: Australas J Dermatol. 2014 Mar 11. http://doi.org/10.1111/ajd.12142 (Australien)

PT: CR

- Tätowierung

Vork: extrem selten

Lit: Ann Dermatol Venereol. 2022 Jun 22:S0151-9638(22)00033-3. http://doi.org/10.1016/j.annder.2022.03.002

Bed: Erstbeschreibung

KL: - Fieber mit Leukozytose (febril, neutrophil)

- Arthralgien

- typische Hautveränderungen

Bef: - herdförmig konfluierende sukkulente Papeln

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Note: dermales Ödem bedingt Pseudovesikelaspekt

- dunkelrot, schmerzhaft, asymmetrisch

Lok: Gesicht und Hals, Oberarmstreckseiten, Stamm

CV: - auch untere Extremitäten möglich, wo die Läsionen tief im Panniculus adiposus liegen und so eine Pannikulitis oder Erythema nodosum imitieren.

- keine Schleimhautbeteiligung

So: palmoplantares Sweet-Syndrom

Vork: sehr selten

Lit:  

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Vork: - selten

- Frauen überwiegen Männer = 5/1; meist Frauen mittleren Alters

Hi: 

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So: - Neutrophile Dermatose der Hände

Engl: neutrophilic dermatosis of the hands

Lit: -  

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- Dermatol Online J. 2023 Dec 15;29(6). http://doi.org/10.5070/D329662999

Etlg: - neutrophilic dermatosis of the palms (NDP)

- neutrophilic dermatosis of the dorsal hands (NDDH)

Lit: -  

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- J Am Acad Dermatol. 2019 Sep 6. pii: S0190-9622(19)32678-7. http://doi.org/10.1016/j.jaad.2019.08.070

Def: lokalisierte, auf die Hände beschränkte Variante des Sweet-Syndroms

- xanthomatisiertes Sweet-Syndrom

Engl: xanthomatized Sweet's syndrome

Lit: J Dermatol. 2021 May;48(5):695-698. Fehler! Linkreferenz ungültig.

- herpetiformes Sweet-Syndrom

Lit: Am J Dermatopathol. 2024 May 7. http://doi.org/10.1097/DAD.0000000000002733

- subkutanes Sweet-Syndrom

Lit: Am J Dermatopathol. 2024 Oct 15. http://doi.org/10.1097/DAD.0000000000002845

Ass: - bakterielle Infektionen

Bsp: - Yersinia-Infektionen/Yersiniose

Inf: Infektion meist durch Tierkontakte oder kontaminierte Fleischprodukte, ferner durch Bluttransfusionen (anamnestisch abklären)

Err: meist Yersinia enterocolitica, seltener Yersinia pseudotuberculosis

Bed: nachweisbar bei ca. 1% aller Diarrhoen

KL: - Gastroenteritis

Vork: insbes. Kleinkinder

- Lymphadenitis mesenterica

KL: Pseudoappendizitis

- Enterokolitis

KL: Diarrhoe

Kopl: gehäuft bei HLA-B27-positiven Pat.:

- reaktive Arthritis

- Erythema nodosum

Di: Stuhl, mesenteriale LK (nach Op), Darmbiopsie, Blut (bei Sepsis), Serum (Ak-Titer beider Erregerformen)

CV: Bei erhöhtem Ak-Titer gegen Yersinia enterocolitica aber negativer Stuhlprobe muss differentialdiagnostisch eine Bruzellose ausgeschlossen werden wegen Kreuzantigenität zwischen Bruzellen und Yersinien (Y. enterocolitica, Serotyp 09)

Th: Primär wichtig ist der orale Flüssigkeits- und Elektrolytausgleich

- Helicobacter-pylori-Infektion

- Staph. aureus

- maligne Grunderkrankungen

Bsp: - akute myeloische Leukämie (AML)

Vork: - in 10% d. F. von Sweet-Syndrom

Verl: Sweet-Syndrom geht akuter myeloischer Leukämie oft Monate voraus

CV: hämatologische Verlaufskontrollen sind indiziert

KL: klinisch dann häufig mit bullösen Läsionen, die ein Pyoderma gangraenosum imitieren können

Lit:  

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- meist Erwachsene (85%); selten Kinder (15% d. F., dann meist im 1.-3. Lj.)

Risk: - Trisomie 21

- Polycythaemia vera

Pg: Unfähigkeit der Leukämiezellen, über das Stadium der Myeloblasten oder Promyelozyten hinauszureifen und ungehemmte Proliferation der Blasten

Di: - Blutbild: nur Myeloblasten erhöht

- Hiatus leucaemicus

Vork: nur bei akuten Anämien

Def: gleichzeitiges Auftreten völlig unreifer Zellformen neben reifen Zellen bei Fehlen der Zwischenformen

- Auer-Stäbchen (25% d. F.)

Def: Zellorganellen (Reifungszeichen) im Zytoplasma von Myeloblasten

Di: färben sich rot in der Giemsa-Färbung

CV: finden sich seltener auch bei CML

- i. d. R. deutliche Infiltration des Knochenmarks mit Blasten (> 30%)

- Infiltration der Periportalfelder der Leber

So: Promyelozytenleukämie mit charakteristischer Verbrauchskoagulopathie

DD: myelodysplastisches Syndrom

Hi: - positive Lysozymfärbung bei spezifischen kutanen Infiltraten

- nierenförmig gebuchtete Zellkerne

Th: - Chemotherapie

- autologe Knochenmark-/Stammzelltransplantation

- allogene Knochenmarktransplantation

Prog: 20% Heilungsrate mit Chemotherapie (komplette Remission bei 60-80%)

- multiples Myelom

- Lupus erythematodes

Lit: J Dermatol. 2013 May 20. http://doi.org/10.1111/1346-8138.12184 (Japan)

Di: - Hautbiopsie

Hi: - Epidermis meist normal, gel. leichte Spongiose oder Pusteln unter dem Stratum corneum

- Ödem des Stratum papillare (Papillarkörper) mit insgesamt bandförmig verlaufendem entzündlichem Infiltrat mit zahlreichen Neutrophilen, perivaskulär orientiert und bis in die tiefe Dermis reichend (Stratum reticulare)

Note: In späteren Stadien nimmt der Anteil an Lymphozyten zu.

- ggf. Leukozytoklasie

- Vasodilatation und Endothelschwellung sowie leichtgradige Erythrozytenextravasation möglich (ausgeprägte Vaskulitiszeichen fehlen aber)

So: atypisches Sweet-Syndrom / histiozytoides Sweet-Syndrom

Engl: histiocytoid Sweet's syndrome

Histr: Erstbeschreibung im Jahre 2005

Gen: ggf. Mutationen der Isocitratdehydrogenase

Lit: J Cutan Pathol. 2019 Jan 10. http://doi.org/10.1111/cup.13418

Bef: histiozytoide monozytäre Zellen dominieren

Hyp: unreife neutrophile Granulozyten

Bed: häufig Malignom-assoziiert, insbes. myelodysplastisches Syndrom in fast 50% der Fälle (dann auch als Myelodysplasia cutis bezeichnet)

Lit: Dermatol Clin. 2024 Apr;42(2):209-217. http://doi.org/10.1016/j.det.2023.08.004

DD: RAS-Associated Autoimmune Leukoproliferative Disease (RALD Cutis)

Gen: somatische Mutationen von entweder NRAS- oder KRAS-Genen in einer Untergruppe hämatopoetischer Zellen

KL: Autoimmunphänomene, Splenomegalie, Lymphadenopathie, Monozytose

Lit: Am J Dermatopathol. 2018 Dec 26. http://doi.org/10.1097/DAD.0000000000001332

Lit: - Case Rep Dermatol Med. 2014;2014:954254.  

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- JAAD Case Rep. 2021 Oct 5;17:96-99. http://doi.org/10.1016/j.jdcr.2021.09.025

- Cutis. 2022 Jan;109(1):E36-E38. http://doi.org/10.12788/cutis.0457

- Pediatr Dermatol. 2025 Apr 25. http://doi.org/10.1111/pde.15932

DD: - Erythema elevatum et diutinum

- spezifische Infiltrate bei Leukaemia cutis (z. B. bei CML)

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Syn: Leukämia cutis, Leucaemia cutis, Leucemia cutis, Leukemia cutis

Lit: -  

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- JAAD Case Rep. 2020 Aug 28;6(11):1138-1140

- Pegfilgastrim-induzierte neutrophile Dermatose

Lit: Acta Derm Venereol. 2019 Mar 8. http://doi.org/10.2340/00015555-3170

- VEXAS-Syndrom

Engl: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic)

Histr: Erstbeschreibung durch Beck et al. im Jahre 2020

Gen: Mutation im UBA1-Gen

KL: vorwiegend Beteiligung von Haut (90% d.F.), Knorpel und Knochen

Lit: -  

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- JAMA Dermatol. 2021 Sep 8:e213344. http://doi.org/10.1001/jamadermatol.2021.3344

- Arch Dermatol Res. 2022 Feb 24. http://doi.org/10.1007/s00403-022-02340-4

- JEADV Clin Pract. 2024;3:1035-1048. https://doi.org/10.1002/jvc2.395

- Labor

- BSG erhöht

- Leukozytose

Bef: meist > 15.000/Mikroliter

- Diffentialblutbild

- Immunelektrophorese

Bef: rel. häufig monoklonale Gammopathie

- Ausschluss einer Sepsis

Meth: Blutkulturen

Prog: - Unbehandelt vergrößern sich die Läsionen, um sich schließlich nach Wochen ohne Narbenbildung zurückzubilden.

- Rezidive in 50% d. F.

Lit: -  

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- JAAD Case Rep. 2020 Sep 16;6(12):1231-1233. http://doi.org/10.1016/j.jdcr.2020.08.039

- JAAD Case Rep. 2020 Oct 15;6(12):1196-1200. http://doi.org/10.1016/j.jdcr.2020.10.006

- JAAD Case Rep. 2024 Feb 4;45:91-93. http://doi.org/10.1016/j.jdcr.2024.01.025

- JEADV Clin Pract. 2024;3:1267-1269. https://doi.org/10.1002/jvc2.445

Th: - Glukokortikoide

Appl: - systemisch

Bed: GS

- topisch

- Dapson

Ind: Therapieresistenz

Dos: 100 mg/Tag

- Cyclosporin A

Ind: Therapieresistenz

Lit: Clin Exp Dermatol 1994; 19: 274-7

- Azathioprin

Note: Azathioprin ist anekdotisch auch als Auslöser eines Sweet-Syndroms beschrieben.

Lit: Dermatol Online J. 2015 Jul 15;21(7)

Ind: histiozytoides Sweet-Syndrom

- Indometacin

Ind: insbes. bei Arthralgien

Dos: 25-50 mg/Tag

Lit: J Am Acad Dermatol 1997; 36: 436-9

PT: CS (18 Pat.)

- Antibiotika

Ind: z. B. entzündliche Darmerkrankungen, Yersinia-assoziierte Fälle

Stoff: Cotrimoxazol, Tetrazykline, Cephalosporine der 3. Generation, Metronidazol, Doxycyclin

- Kaliumiodid

Dos: 600-900 mg/Tag

Lit: Br J Dermatol 1998; 139: 555-6

- Pentoxyfillin

Lit: J Am Acad Dermatol 1995; 32: 533-5

- Interferon-alpha

Lit: Clin Exp Dermatol 1999; 24: 443-5

- Colchicin

Dos: 2x/Tag 0,6 mg

Lit: - J Am Acad Dermatol 2002; 47: 323-4

PT: CR

- Br J Dermatol 1999; 140: 565-6

PT: CS (20 Pat.)

- Int J Dermatol. 2016 Sep;55(9):1033-9 (Tunesien)

PT: CS

- Infliximab

Lit: Ann Rheum Dis 2003; 62: 81-2

CV: Pat. verstarb an Sepsis

Altn: Adalimumab

Lit: - JAMA Dermatol. 2016 Jul 1;152(7):842-4 (USA)

PT: CR

- J Dtsch Dermatol Ges. 2020 Aug 24. http://doi.org/10.1111/ddg.14223

- Brodalumab

Lit: Int J Dermatol. 2024 May 10. http://doi.org/10.1111/ijd.17197

- Spesolimab

Lit: JAMA Dermatol. 2025 Jan 1;161(1):110-112. http://doi.org/10.1001/jamadermatol.2024.4342

- Anakinra

Def: Anti-IL1-Rezeptor-Antagonist

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Lit: Dermatology. 2011;222(2):123-7

PT: CR

  
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