DefinitionThis section has been translated automatically.
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Clinical featuresThis section has been translated automatically.
- Major criteria (n. Yamaguchi et al.):
- Integument: Volatile, urticarial, morbilliform, or scarlatiniform exanthema on extremities, trunk, and face with high recurrence. Intensification often in the evening, often absent in the morning (90%).
- Intermittent episodes of fever > 39 °C with 1-2 spikes/day, persistent for at least 1 week (> 95%)
- Arthralgias for at least 2 weeks
- Arthritides > 2 weeks (90%)
- Leukocytosis (> 10,000/μl) with at least 80% neutrophils.
- Minor criteria:
- Sore throat
- Visceral manifestations: Generalized non-painful lymphadenopathy with or without splenomegaly (45%).
- Transaminases and/or LDH elevated
- Pericarditis (25%)
- pleurisy (25%)
- Rheumatoid factor negative
- ANA negative
The diagnosis of "adult Still syndrome" requires at least 5 criteria, 2 of which are major criteria.
LaboratoryThis section has been translated automatically.
Nonspecific signs of inflammation with leukocytosis and neutrophilia, BSG and CRP significantly elevated, often high ferritin level, thrombocytosis possible (Höllweger N et al. 2018).
HistologyThis section has been translated automatically.
Dermal oedema with dilated blood and lymphatic vessels, thin perivascular infiltrate of neutrophil granulocytes, lymphocytes and macrophages.
DiagnosisThis section has been translated automatically.
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TherapyThis section has been translated automatically.
Note(s)This section has been translated automatically.
The adult breastfeeding syndrome is counted among the extended circle of autoinflammatory syndromes.
In rare cases, overlaps are described with Kikuchi-Fujimoto Syndrome, a cervically emphasized self-limited lymphadenopathy.
LiteratureThis section has been translated automatically.
- Herzer P (1986) Still syndrome in adults. German Med Weekly 111: 865-867
- Höllweger N et al (2018) Adult onset Still`s syndrome. J Dtsch Dermatol 16: 491-492
- Kaufmann S et al (1981) Still syndrome in adults. A contribution to the differential diagnosis of "fever of unknown origin". Med World 32: 1692-1697
- Prendiville JS et al (2004) A Pruritic Linear Urticarial Rash, Fever, and Systemic Inflammatory Disease in Five Adolescents: Adult-Onset Still Disease or Systemic Juvenile Idiopathic Arthritis sine Arthritis? Pediatric Dermatol 21: 580-588
- Zollner RC et al (1997) Hyperferritinemia in Still syndrome in the adult and reactive hemophagocytic syndrome. Med Klin (Munich) 92: 494-498
Incoming links (7)Angioimmunoblastic T cell lymphoma; Breastfeeding syndrome; Dermatitis-arthritis syndromes; Felty syndrome; Interleukin-1; Platelet; Schnitzler syndrome;
Outgoing links (5)Autoinflammatory syndromes (overview); Breastfeeding syndrome; Rheumatoid arthritis and skin manifestations; Schnitzler syndrome; Urticaria (overview);
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.