Breastfeeding syndrome adultes M06.1

Particular course of primary chronic polyarthritis in adulthood, an autoinflammatory syndrome with recurrent urticarial exanthema, lymphadenopathy, re- and intermittent fever > 39 °C, carditis, liver and spleen enlargement, pericarditis, rarely myocarditis. See also Still's syndrome in childhood.

• 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.

Nonspecific signs of inflammation with leukocytosis and neutrophilia, BSG and CRP significantly elevated, often high ferritin level, thrombocytosis possible (Höllweger N et al. 2018).

Dermal oedema with dilated blood and lymphatic vessels, thin perivascular infiltrate of neutrophil granulocytes, lymphocytes and macrophages.

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.

1. Herzer P (1986) Still syndrome in adults. German Med Weekly 111: 865-867
2. Höllweger N et al (2018) Adult onset Still`s syndrome. J Dtsch Dermatol 16: 491-492
3. 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
4. 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
5. Zollner RC et al (1997) Hyperferritinemia in Still syndrome in the adult and reactive hemophagocytic syndrome. Med Klin (Munich) 92: 494-498