Sweet syndrome (acute febrile neutrophilic dermatosis)

Overview
Clinical Features & Triggers
- Triggers: Classically, infection + medications + neoplasms plus pregnancy + IBD.
- Mucosal Involvement: Sweet's Syndrome (like Behçet's disease) typically causes aphthae on the oral mucosa — from this follows the therapeutic logic Colchicine (analogous to Behçet's, inhibits neutrophil migration).
- Bullous Variant: A bullous Sweet's variant with tense blisters is typically associated with myeloproliferative disease — obligatory hematological screening.
- Associated underlying disorders: besides the para-/post-infectious forms (above all upper respiratory streptococcal infection, yersiniosis) and the paraneoplastic forms, Sweet's syndrome is associated with autoimmune diseases — first and foremost rheumatoid arthritis and inflammatory bowel disease (ulcerative colitis, Crohn's disease, often in parallel with flares); further with thyroid disease, Behçet's disease and pregnancy. Consequence: every Sweet's syndrome warrants a targeted history for joint and bowel symptoms plus haematological screening.
- Pathogenesis: Activation of neutrophilic granulocytes by dermal immune complex deposition + IL-1 + IL-17.
- Clinical Presentation: Typical are painful succulent plaques on the upper back / dorsum of hands, pseudo-vesicular appearance due to dermal edema.
- General: Typical are high fever + arthralgias plus Neutrophilia + leukocytosis.
Diagnostic Criteria (Su / Liu, Walker / Cohen)
- 2 Major + 2 Minor ⇒ Diagnosis.
- Major: Abrupt eruption + histology with dense neutrophilic infiltrate without vasculitis.
- Minor: Fever, trigger (infection / tumor / medication), excellent response to systemic corticosteroids, ESR ↑ / leukocytosis / neutrophilia.
Differential diagnoses
- Erythema nodosum
- Polyarteritis nodosa
- Abscess
- Granuloma annulare
- Urticaria
- Psoriasis vulgaris (plaque psoriasis)
- Pyoderma gangrenosum
- Leukocytoclastic vasculitis
Practise Sweet syndrome (acute febrile neutrophilic dermatosis) in the app
Flashcards with spaced repetition, exam questions and spot-the-diagnosis on this topic – in DermaFuchs, free of charge.
In the DermaFuchs app: 5 flashcards · 2 clinical images
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More inflammatory skin diseases
- Cheilitis granulomatosa (Miescher)
- Erythema annulare centrifugum (EAC)
- Erythema nodosum
- Graft-versus-host disease (GvHD)
- Granuloma annulare
- Cutaneous sarcoidosis
- Lichen planus
- Lichen sclerosus
Note: This page is intended for medical education and does not replace diagnosis or treatment decisions in individual cases. Treatment and follow-up content is available in the app.