DefinitionThis section has been translated automatically.
Particular course of primary chronic polyarthritis in adulthood, an autoinflammatory syndrome with recurrent urticarial exanthema, lymphadenopathy, re- and intermittent fever > 39 °C, carditis, enlargement of liver and spleen, pericarditis, more rarely myocarditis.
ManifestationThis section has been translated automatically.
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Clinical featuresThis section has been translated automatically.
- Main criteria (n. Yamaguchi et al.):
- Integument: Volatile, urticarial, morbilliform or scarlatiniform exanthema of the extremities, trunk and face with high recurrence rate. Reinforcement often in the evening, in the morning often absent (90%)
- Intermittent fever attacks > 39 °C with 1-2 peaks/day, persisting for at least 1 week (> 95%)
- Arthralgia for at least 2 weeks
- Arthritides > 2 weeks (90%)
- Leukocytosis (> 10,000/μl) with at least 80% neutrophils.
- Secondary criteria:
- Sore throat
- Visceral manifestations: Generalized, non-painful lymphadenopathy with or without splenomegaly (45%)
- Transaminases and/or LDH increased
- pericarditis (25%)
- Pleurisy (25%)
- Rheumatoid factor negative
- ANA negative.
The diagnosis "adult breastfeeding syndrome" requires at least 5 criteria, 2 of which are main 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.
Differential diagnosisThis section has been translated automatically.
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 (6)Angioimmunoblastic T cell lymphoma; Dermatitis-arthritis syndromes; Felty syndrome; Interleukin-1; Platelet; Schnitzler syndrome;
Outgoing links (4)Autoinflammatory syndromes (overview); Rheumatoid arthritis (ra); Schnitzler syndrome; Urticaria (overview);
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.