[Wissler's allergic subsepsis]. / Subsepsis allergica Wissler.

A 20-year-old female patient with the typical signs of Wissler's subsepsis allergica (Wissler-Fanconi syndrome) is described. This rare disease is characterized by four typical symptoms: polymorphous exanthemas, recurrent high fever, leucocytosis and arthralgia. In the early stages it is difficult to differentiate from septicaemia. This syndrome has sometimes been considered equivalent to or an initial stage of Still's disease (juvenile rheumatoid arthritis) progressing to degenerative arthritis in many patients, whereas other authors have classified it as a separate entity with good prognosis. The present case demonstrates that Wissler's subsepsis allergica should be considered when ever transient polymorphous exanthema is accompanied by high recurrent fever, leucocytosis and arthralgia.

[Subsepsis allergica in a patient with type I polyglandular autoimmune syndrome]. / Subsepsis allergica bei einer Patientin mit polyglandulärem Autoimmunsyndrom Typ I.

A 10.6 year old Turkish girl developed + the signs of a polyglandular autoimmune syndrome (PGA) type I since her first year of age. Apart from the endocrine and non-endocrine symptoms of PGA, she suffered from an acute state of illness with therapy-resistant fever and multiform exanthemas in the early course of disease. All included the criteria of Wissler-Fanconi syndrome became clear which has not yet been reported in association with PGA. Although this syndrome generally is considered an equivalent of Still's syndrome, rheumatoid symptoms could not be ascertained during the following 9-year-course of PGA.

Síndrome de Wissler-Fanconi: uma forma incomun de artrite reumatóide / Wissler-Fanconi Syndrome: an unusual form of rheumatoid arthritis

A artrite reumatóide pode apresentar-se raramente sem sintomas articulares, apenas com manifestaçöes sistêmicas, quadro esse que autores de língua alemä chamam de síndrome de Wissler-Fanconi. Relata-se o caso de uma criança masculina de 11 anos, que iniciou com febre, cefaléia, rigidez de nuca, esplenomegalia e prostraçäo. Com o diagnóstico de meningite inespecífica, recebeu ampicilina, com melhora do meningismo e das alteraçöes liquóricas, mas näo da febre e prostraçäo. Posteriormente surgiu eritema. VHS de 115mm, leucocitose de 17.000, culturas de sangue e urina negativas, FAN e LE negativos, látex 50UI/ml. Tratado com prednisona, apresentou considerável e rápida melhora clínica, persistindo apenas eritema facial episódico

Hematuria as presenting sign in Wissler-Fanconi syndrome.

We describe a case of persistent microscopic hematuria as initial finding in incomplete Still's disease or Wissler-Fanconi syndrome. Renal biopsy findings were compatible with intravascular coagulation. Wissler-Fanconi syndrome and the associated renal abnormalities are briefly reviewed.

[Fatal form of pericardo-myocarditis in Wissler-Fanconi syndrome]. / Forme mortelle de péricardo-myocardite au cours d'un syndrome de Wissler-Fanconi.

The Wissler-Fanconi syndrome is an inflammatory disease of unknown origin, similar to Still's disease, a systemic form of juvenile arthritis. The long-term evolution is marked by recurrent febrile exacerbations, sometimes complicated by pericardo-myocarditis which usually resolves without sequellae. The authors report a case with chronic pericardo-myocarditis progressing over a 7 year period to refractory congestive cardiac failure.

[Still disease in adults]. / Maladie de Still de l'adulte.

In 1971, less than one-hundred years after Still's publication (1897) concerning children, Bywaters described the adult-onset form of Still disease. Over one-hundred cases of this disorder have been recorded since. In adults, Still disease affects mainly women, and the average age at onset is twenty-seven. The well-known features demonstrated by clinical examination and complementary investigations are recalled. They include fever, arthritis, rash, lymphadenopathy, enlarged spleen, and serositis. Attention is drawn to hepatic and muscular involvement as well as to articular prognosis. Additional information is provided on questions which remain open to debate, particularly concerning management and therapeutic results.

[Familial subsepsis allergica- a systemic form of chronic juvenile arthritis]. / Zur familiaritt der subsepsis allergica-systemische form der juvenilen chonischen arthritis.

Case report of a baby and his 26-year old mother suffering from Subsepsis allergica. Her disease was discovered when she was 2 years old. Both patients showed common symptoms such as regular bouts of high fever with brief exanthemas resistant to antibiotic treatment. The results of their hematological tests were typical of this illness. The mother has also suffered from sporadic attacks of arthritis for the past ten years, but these have not handicapped her in any way. In spite of extensive diagnostic tests we were unable to shed new light on the etiopathogenesis of this systemic form of chronic juvenile arthritis. Since the baby's illness has so far proved to be of benign nature, long-term medication was found to be unnecessary. The mother finds relief through symptomatic treatment.