Wissler's syndrome
| Wissler's syndrome | |
|---|---|
| Synonyms | Wissler-Fanconi syndrome |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Fever, rash, arthralgia, myalgia, leukocytosis, elevated erythrocyte sedimentation rate |
| Complications | Pericarditis, pleuritis |
| Onset | Typically in childhood or adolescence |
| Duration | Variable |
| Types | N/A |
| Causes | Unknown, possibly autoimmune |
| Risks | Unknown |
| Diagnosis | Clinical evaluation, exclusion of other conditions |
| Differential diagnosis | Juvenile idiopathic arthritis, systemic lupus erythematosus, rheumatic fever |
| Prevention | N/A |
| Treatment | Nonsteroidal anti-inflammatory drugs, corticosteroids |
| Medication | N/A |
| Prognosis | Generally good with treatment |
| Frequency | Rare |
| Deaths | N/A |
Wissler's syndrome is a rare rheumatic disease.
Clinical presentation
Wissler's syndrome has a presentation similar to sepsis.
Related to Still's disease
It is sometimes considered closely related to Still's disease.
Naming
It is named for Guido Fanconi and Hans Wissler.
History
It was first described by Wissler in 1944 and Fanconi in 1946. Single observations by E. Uhse in 1943 («Febris maculosa intermittens»), Fykow in 1929 and Nowak in 1942.
Signs and symptoms
A symptom complex characterised by:
- high intermittent fever of septic type,
- constantly recurring exanthema,
- transient arthralgia,
- carditis,
- pleurisy,
- neutrophil leukocytosis, and
- increased erythrocyte sedimentation rate.
Causes
Etiology uncertain. Wissler suggested an allergic reaction to bacteraemia as the pathogenic factor.
Diagnosis
History, physical examination and studies to rule out other common causes
Epidemiology
Children and adolescents are most frequently affected
Ages affected
The age in the reported cases varied from 5 to 17 years.
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Contributors: Prab R. Tumpati, MD