This resource is in its pre-alpha development stage. Content is actively being curated and may be incomplete or subject to change.

Wissler syndrome

Immune MONDO:0006018 Autoinflammatory Disease Inflammatory Arthritis

A rheumatic syndrome of possibly allergic origin, usually affecting children and adolescents, and characterized by high fever, exanthema, arthralgia, leukocytosis, and increased sedimentation rate. Also called Wissler-Fanconi syndrome or subsepsis allergica.

0
Mappings
0
Definitions
0
Inheritance
0
Pathophysiology
0
Histopathology
9
Phenotypes
0
Genes
4
Treatments
0
Subtypes
5
Differentials
0
Datasets
0
Trials
●

Phenotypes

9
Blood(1) Cardiovascular(3) Genitourinary(1) Immune(1) Metabolism(1) Musculoskeletal(1) Constitutional(1)
Blood 1
Leukocytosis FREQUENT Increased total leukocyte count (HP:0001974)
Show evidence (1 reference)
PMID:8150635 SUPPORT
"This rare disease is characterized by four typical symptoms: polymorphous exanthemas, recurrent high fever, leucocytosis and arthralgia."
The abstract lists leukocytosis as a typical symptom of Wissler-Fanconi syndrome.
Cardiovascular 3
Pericarditis VERY_RARE Pericarditis (HP:0001701)
Show evidence (1 reference)
PMID:3092775 PARTIAL
"The long-term evolution is marked by recurrent febrile exacerbations, sometimes complicated by pericardo-myocarditis which usually resolves without sequellae."
The abstract notes pericardo-myocarditis as a complication during the course of disease.
Myocarditis VERY_RARE Myocarditis (HP:0012819)
Show evidence (1 reference)
PMID:3092775 PARTIAL
"The long-term evolution is marked by recurrent febrile exacerbations, sometimes complicated by pericardo-myocarditis which usually resolves without sequellae."
The abstract reports pericardo-myocarditis as a complication of Wissler-Fanconi syndrome.
Congestive heart failure VERY_RARE Congestive heart failure (HP:0001635)
Show evidence (1 reference)
PMID:3092775 SUPPORT
"The authors report a case with chronic pericardo-myocarditis progressing over a 7 year period to refractory congestive cardiac failure."
The case report documents progression to refractory congestive heart failure.
Genitourinary 1
Microscopic hematuria VERY_RARE Microscopic hematuria (HP:0002907)
Show evidence (1 reference)
PMID:3572917 PARTIAL
"We describe a case of persistent microscopic hematuria as initial finding in incomplete Still's disease or Wissler-Fanconi syndrome."
The case report documents microscopic hematuria as a presenting sign.
Immune 1
Exanthem FREQUENT Exanthem (HP:4000054)
Show evidence (1 reference)
PMID:8150635 SUPPORT
"This rare disease is characterized by four typical symptoms: polymorphous exanthemas, recurrent high fever, leucocytosis and arthralgia."
The abstract lists polymorphous exanthemas as a typical symptom of Wissler-Fanconi syndrome.
Metabolism 1
Recurrent high fever FREQUENT Recurrent fever (HP:0001954)
Show evidence (1 reference)
PMID:8150635 SUPPORT
"This rare disease is characterized by four typical symptoms: polymorphous exanthemas, recurrent high fever, leucocytosis and arthralgia."
The abstract lists recurrent high fever as a typical symptom of Wissler-Fanconi syndrome.
Musculoskeletal 1
Arthritis FREQUENT Arthritis (HP:0001369)
Migratory polyarthritis reported in case presentations.
Show evidence (1 reference)
PMID:27843372 PARTIAL
"A middle-aged female presented to the emergency department with shortness of breath and chest pain associated with fever, polyarthritis, and had a chronic polymorphic rash on the back and lower extremities."
The case report documents polyarthritis as part of the clinical presentation.
Constitutional 1
Arthralgia FREQUENT Arthralgia (HP:0002829)
Show evidence (1 reference)
PMID:8150635 SUPPORT
"This rare disease is characterized by four typical symptoms: polymorphous exanthemas, recurrent high fever, leucocytosis and arthralgia."
The abstract lists arthralgia as a typical symptom of Wissler-Fanconi syndrome.
💊

Treatments

4
Nonsteroidal anti-inflammatory drugs MAXO:0000221
Drug: naproxen ↗
NSAIDs can reduce fever and inflammatory symptoms.
Show evidence (1 reference)
PMID:27843372 PARTIAL
"A combination of nonsteroidal anti-inflammatory drugs and steroids achieved complete remission."
The case report documents remission with NSAID therapy.
Corticosteroids MAXO:0000640
Systemic corticosteroids (e.g., prednisolone) used as part of combination regimens to control inflammation.
Show evidence (2 references)
PMID:27843372 PARTIAL
"A combination of nonsteroidal anti-inflammatory drugs and steroids achieved complete remission."
The case report notes complete remission with steroids plus NSAIDs.
PMID:1210460 PARTIAL
"In the present case the disease could successfully be controlled using imuran in combination with corticosteroids."
The abstract indicates corticosteroids were part of successful therapy.
Dapsone MAXO:0000058
Dapsone was used alongside NSAIDs and corticosteroids in a reported case.
Show evidence (1 reference)
PMID:27843372 PARTIAL
"A combination of naproxen, dapsone, and prednisolone therapy resulted in significant improvement of the patient’s arthralgias and rash."
The case report documents improvement with dapsone as part of combination therapy.
Azathioprine MAXO:0000058
Immunosuppressive therapy used in combination with corticosteroids.
Show evidence (1 reference)
PMID:1210460 PARTIAL
"In the present case the disease could successfully be controlled using imuran in combination with corticosteroids."
The abstract reports successful control with azathioprine (imuran) plus steroids.
🔬

Biochemical Markers

2
Erythrocyte sedimentation rate (Elevated)
Show evidence (1 reference)
PMID:27843372 PARTIAL
"Blood sample analysis revealed high levels of CRP (42.9 mg/dL, normal: 0–0.3), erythrocyte sedimentation rate (113 mm/h normal: 2–20)"
The case report documents markedly elevated ESR, supporting a systemic inflammatory state.
C-reactive protein (Elevated)
Show evidence (1 reference)
PMID:27843372 PARTIAL
"Blood sample analysis revealed high levels of CRP (42.9 mg/dL, normal: 0–0.3), erythrocyte sedimentation rate (113 mm/h normal: 2–20)"
The case report documents markedly elevated CRP as part of the inflammatory syndrome.
🔀

Differential Diagnoses

5

Conditions with similar clinical presentations that must be differentiated from Wissler syndrome:

Sepsis Not Yet Curated MONDO:1040015
Overlapping Features Early Wissler-Fanconi presentations can be difficult to distinguish from septicemia.
Distinguishing Features
  • Repeated blood and urine cultures can be negative despite high fever and leukocytosis.
Show evidence (2 references)
PMID:8150635 SUPPORT
"In the early stages it is difficult to differentiate from septicaemia."
The abstract notes early overlap with septicemia.
PMID:27843372 SUPPORT
"Urine and multiple blood cultures were negative."
The case report documents negative cultures despite systemic inflammatory presentation.
Acute rheumatic fever Not Yet Curated MONDO:0017767
Overlapping Features Considered in the differential diagnosis because of overlapping febrile inflammatory presentations.
Show evidence (1 reference)
PMID:27843372 SUPPORT
"Features of Wissler-Fanconi syndrome can be found in a differential diagnosis that includes true sepsis, acute rheumatic fever, rheumatoid arthritis, and adult onset Still’s disease."
The case report explicitly lists acute rheumatic fever as part of the differential diagnosis.
Rheumatoid arthritis MONDO:0008383
Overlapping Features Inflammatory arthritis with systemic features can overlap with Wissler-Fanconi syndrome.
Show evidence (1 reference)
PMID:27843372 SUPPORT
"Features of Wissler-Fanconi syndrome can be found in a differential diagnosis that includes true sepsis, acute rheumatic fever, rheumatoid arthritis, and adult onset Still’s disease."
The case report explicitly lists rheumatoid arthritis as part of the differential diagnosis.
Adult-onset Still's disease Not Yet Curated MONDO:0019355
Overlapping Features Shares fever, rash, leukocytosis, and arthralgia; some authors consider Wissler-Fanconi syndrome related.
Show evidence (2 references)
PMID:27843372 SUPPORT
"Features of Wissler-Fanconi syndrome can be found in a differential diagnosis that includes true sepsis, acute rheumatic fever, rheumatoid arthritis, and adult onset Still’s disease."
The case report explicitly lists adult-onset Still's disease as part of the differential diagnosis.
PMID:8150635 SUPPORT
"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 abstract notes debated overlap with Still's disease.
Autoimmune polyendocrine syndrome type 1 Not Yet Curated MONDO:0009411
Overlapping Features Reported in a case with subsepsis allergica; included as a differential context.
Show evidence (1 reference)
PMID:2233764 PARTIAL
"A 10.6 year old Turkish girl developed + the signs of a polyglandular autoimmune syndrome (PGA) type I since her first year of age."
The report describes a patient with type I polyglandular autoimmune syndrome alongside subsepsis allergica.
{ }

Source YAML

click to show 
name: Wissler syndrome
creation_date: '2026-01-27T22:32:20Z'
updated_date: '2026-01-29T17:34:55Z'
category: Immune
description: >-
  A rheumatic syndrome of possibly allergic origin, usually affecting children
  and adolescents, and characterized by high fever, exanthema, arthralgia,
  leukocytosis, and increased sedimentation rate. Also called Wissler-Fanconi
  syndrome or subsepsis allergica.
disease_term:
  term:
    id: MONDO:0006018
    label: Wissler syndrome
  preferred_term: Wissler syndrome
parents:
- Autoinflammatory Disease
- Inflammatory Arthritis
synonyms:
- Wissler-Fanconi syndrome
- Subsepsis allergica
- Subsepsis hyperergica
phenotypes:
- name: Recurrent high fever
  category: Systemic
  frequency: FREQUENT
  severity: High
  phenotype_term:
    preferred_term: Recurrent fever
    term:
      id: HP:0001954
      label: Recurrent fever
  evidence:
  - reference: PMID:8150635
    supports: SUPPORT
    snippet: "This rare disease is characterized by four typical symptoms: polymorphous exanthemas, recurrent high fever, leucocytosis and arthralgia."
    explanation: The abstract lists recurrent high fever as a typical symptom of Wissler-Fanconi syndrome.
- name: Exanthem
  category: Dermatological
  frequency: FREQUENT
  phenotype_term:
    preferred_term: Exanthem
    term:
      id: HP:4000054
      label: Exanthem
  evidence:
  - reference: PMID:8150635
    supports: SUPPORT
    snippet: "This rare disease is characterized by four typical symptoms: polymorphous exanthemas, recurrent high fever, leucocytosis and arthralgia."
    explanation: The abstract lists polymorphous exanthemas as a typical symptom of Wissler-Fanconi syndrome.
- name: Arthralgia
  category: Musculoskeletal
  frequency: FREQUENT
  phenotype_term:
    preferred_term: Arthralgia
    term:
      id: HP:0002829
      label: Arthralgia
  evidence:
  - reference: PMID:8150635
    supports: SUPPORT
    snippet: "This rare disease is characterized by four typical symptoms: polymorphous exanthemas, recurrent high fever, leucocytosis and arthralgia."
    explanation: The abstract lists arthralgia as a typical symptom of Wissler-Fanconi syndrome.
- name: Leukocytosis
  category: Hematologic
  frequency: FREQUENT
  phenotype_term:
    preferred_term: Leukocytosis
    term:
      id: HP:0001974
      label: Increased total leukocyte count
  evidence:
  - reference: PMID:8150635
    supports: SUPPORT
    snippet: "This rare disease is characterized by four typical symptoms: polymorphous exanthemas, recurrent high fever, leucocytosis and arthralgia."
    explanation: The abstract lists leukocytosis as a typical symptom of Wissler-Fanconi syndrome.
- name: Microscopic hematuria
  category: Renal
  frequency: VERY_RARE
  phenotype_term:
    preferred_term: Microscopic hematuria
    term:
      id: HP:0002907
      label: Microscopic hematuria
  evidence:
  - reference: PMID:3572917
    supports: PARTIAL
    snippet: "We describe a case of persistent microscopic hematuria as initial finding in incomplete Still's disease or Wissler-Fanconi syndrome."
    explanation: The case report documents microscopic hematuria as a presenting sign.
- name: Arthritis
  category: Musculoskeletal
  frequency: FREQUENT
  notes: Migratory polyarthritis reported in case presentations.
  phenotype_term:
    preferred_term: Arthritis
    term:
      id: HP:0001369
      label: Arthritis
  evidence:
  - reference: PMID:27843372
    supports: PARTIAL
    snippet: "A middle-aged female presented to the emergency department with shortness of breath and chest pain associated with fever, polyarthritis, and had a chronic polymorphic rash on the back and lower extremities."
    explanation: The case report documents polyarthritis as part of the clinical presentation.
- name: Pericarditis
  category: Cardiovascular
  frequency: VERY_RARE
  phenotype_term:
    preferred_term: Pericarditis
    term:
      id: HP:0001701
      label: Pericarditis
  evidence:
  - reference: PMID:3092775
    supports: PARTIAL
    snippet: "The long-term evolution is marked by recurrent febrile exacerbations, sometimes complicated by pericardo-myocarditis which usually resolves without sequellae."
    explanation: The abstract notes pericardo-myocarditis as a complication during the course of disease.
- name: Myocarditis
  category: Cardiovascular
  frequency: VERY_RARE
  phenotype_term:
    preferred_term: Myocarditis
    term:
      id: HP:0012819
      label: Myocarditis
  evidence:
  - reference: PMID:3092775
    supports: PARTIAL
    snippet: "The long-term evolution is marked by recurrent febrile exacerbations, sometimes complicated by pericardo-myocarditis which usually resolves without sequellae."
    explanation: The abstract reports pericardo-myocarditis as a complication of Wissler-Fanconi syndrome.
- name: Congestive heart failure
  category: Cardiovascular
  frequency: VERY_RARE
  phenotype_term:
    preferred_term: Congestive heart failure
    term:
      id: HP:0001635
      label: Congestive heart failure
  evidence:
  - reference: PMID:3092775
    supports: SUPPORT
    snippet: "The authors report a case with chronic pericardo-myocarditis progressing over a 7 year period to refractory congestive cardiac failure."
    explanation: The case report documents progression to refractory congestive heart failure.
biochemical:
- name: Erythrocyte sedimentation rate
  presence: Elevated
  evidence:
  - reference: PMID:27843372
    supports: PARTIAL
    snippet: "Blood sample analysis revealed high levels of CRP (42.9 mg/dL, normal: 0–0.3), erythrocyte sedimentation rate (113 mm/h normal: 2–20)"
    explanation: The case report documents markedly elevated ESR, supporting a systemic inflammatory state.
- name: C-reactive protein
  presence: Elevated
  evidence:
  - reference: PMID:27843372
    supports: PARTIAL
    snippet: "Blood sample analysis revealed high levels of CRP (42.9 mg/dL, normal: 0–0.3), erythrocyte sedimentation rate (113 mm/h normal: 2–20)"
    explanation: The case report documents markedly elevated CRP as part of the inflammatory syndrome.
progression:
- phase: Onset (SNOMEDCT:77374008)
  age_range: Childhood to adulthood
  evidence:
  - reference: PMID:8150635
    supports: SUPPORT
    snippet: "A 20-year-old female patient with the typical signs of Wissler's subsepsis allergica (Wissler-Fanconi syndrome) is described."
    explanation: This case report documents onset in a young adult.
  - reference: PMID:1210460
    supports: SUPPORT
    snippet: "A further case of subsepsis allergica Wissler in a 35-year-old woman is reported."
    explanation: This report documents adult-onset disease.
  - reference: DOI:10.1007/978-3-642-94912-8_6
    supports: SUPPORT
    snippet: "Zunächst haben nur die Pädiater die Krankheit gesehen, neuerdings sind aber auch Fälle bei Erwachsenen bekanntgegeben worden."
    explanation: The abstract notes that early reports were pediatric, with adult cases reported later.
- phase: Chronic (SNOMEDCT:90734009)
  notes: Recurrent febrile exacerbations with possible cardiac complications.
  evidence:
  - reference: PMID:3092775
    supports: SUPPORT
    snippet: "The long-term evolution is marked by recurrent febrile exacerbations, sometimes complicated by pericardo-myocarditis which usually resolves without sequellae."
    explanation: The abstract summarizes recurrent febrile exacerbations and possible cardiac complications over time.
notes: |
  Early publications largely originated from continental Europe, particularly Germany, France, and Italy. At the time of the review, the disease was not recognized in the English-speaking literature.
differential_diagnoses:
- name: Sepsis
  description: Early Wissler-Fanconi presentations can be difficult to distinguish from septicemia.
  distinguishing_features:
  - Repeated blood and urine cultures can be negative despite high fever and leukocytosis.
  disease_term:
    preferred_term: infectious disease with sepsis
    term:
      id: MONDO:1040015
      label: infectious disease with sepsis
  evidence:
  - reference: PMID:8150635
    supports: SUPPORT
    snippet: "In the early stages it is difficult to differentiate from septicaemia."
    explanation: The abstract notes early overlap with septicemia.
  - reference: PMID:27843372
    supports: SUPPORT
    snippet: "Urine and multiple blood cultures were negative."
    explanation: The case report documents negative cultures despite systemic inflammatory presentation.
- name: Acute rheumatic fever
  description: Considered in the differential diagnosis because of overlapping febrile inflammatory presentations.
  disease_term:
    preferred_term: rheumatic fever
    term:
      id: MONDO:0017767
      label: rheumatic fever
  evidence:
  - reference: PMID:27843372
    supports: SUPPORT
    snippet: "Features of Wissler-Fanconi syndrome can be found in a differential diagnosis that includes true sepsis, acute rheumatic fever, rheumatoid arthritis, and adult onset Still’s disease."
    explanation: The case report explicitly lists acute rheumatic fever as part of the differential diagnosis.
- name: Rheumatoid arthritis
  description: Inflammatory arthritis with systemic features can overlap with Wissler-Fanconi syndrome.
  disease_term:
    preferred_term: rheumatoid arthritis
    term:
      id: MONDO:0008383
      label: rheumatoid arthritis
  evidence:
  - reference: PMID:27843372
    supports: SUPPORT
    snippet: "Features of Wissler-Fanconi syndrome can be found in a differential diagnosis that includes true sepsis, acute rheumatic fever, rheumatoid arthritis, and adult onset Still’s disease."
    explanation: The case report explicitly lists rheumatoid arthritis as part of the differential diagnosis.
- name: Adult-onset Still's disease
  description: Shares fever, rash, leukocytosis, and arthralgia; some authors consider Wissler-Fanconi syndrome related.
  disease_term:
    preferred_term: adult-onset Still disease
    term:
      id: MONDO:0019355
      label: adult-onset Still disease
  evidence:
  - reference: PMID:27843372
    supports: SUPPORT
    snippet: "Features of Wissler-Fanconi syndrome can be found in a differential diagnosis that includes true sepsis, acute rheumatic fever, rheumatoid arthritis, and adult onset Still’s disease."
    explanation: The case report explicitly lists adult-onset Still's disease as part of the differential diagnosis.
  - reference: PMID:8150635
    supports: SUPPORT
    snippet: "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."
    explanation: The abstract notes debated overlap with Still's disease.
- name: Autoimmune polyendocrine syndrome type 1
  description: Reported in a case with subsepsis allergica; included as a differential context.
  disease_term:
    preferred_term: autoimmune polyendocrine syndrome type 1
    term:
      id: MONDO:0009411
      label: autoimmune polyendocrine syndrome type 1
  evidence:
  - reference: PMID:2233764
    supports: PARTIAL
    snippet: "A 10.6 year old Turkish girl developed + the signs of a polyglandular autoimmune syndrome (PGA) type I since her first year of age."
    explanation: The report describes a patient with type I polyglandular autoimmune syndrome alongside subsepsis allergica.
treatments:
- name: Nonsteroidal anti-inflammatory drugs
  description: NSAIDs can reduce fever and inflammatory symptoms.
  treatment_term:
    preferred_term: NSAID therapy
    term:
      id: MAXO:0000221
      label: NSAID therapy
    therapeutic_agent:
    - preferred_term: naproxen
      term:
        id: NCIT:C680
        label: Naproxen
  evidence:
  - reference: PMID:27843372
    supports: PARTIAL
    snippet: "A combination of nonsteroidal anti-inflammatory drugs and steroids achieved complete remission."
    explanation: The case report documents remission with NSAID therapy.
- name: Corticosteroids
  description: Systemic corticosteroids (e.g., prednisolone) used as part of combination regimens to control inflammation.
  treatment_term:
    preferred_term: corticosteroid agent therapy
    term:
      id: MAXO:0000640
      label: corticosteroid agent therapy
  evidence:
  - reference: PMID:27843372
    supports: PARTIAL
    snippet: "A combination of nonsteroidal anti-inflammatory drugs and steroids achieved complete remission."
    explanation: The case report notes complete remission with steroids plus NSAIDs.
  - reference: PMID:1210460
    supports: PARTIAL
    snippet: "In the present case the disease could successfully be controlled using imuran in combination with corticosteroids."
    explanation: The abstract indicates corticosteroids were part of successful therapy.
- name: Dapsone
  description: Dapsone was used alongside NSAIDs and corticosteroids in a reported case.
  treatment_term:
    preferred_term: pharmacotherapy
    term:
      id: MAXO:0000058
      label: pharmacotherapy
    qualifiers:
    - predicate:
        preferred_term: therapeutic agent
        term:
          id: NCIT:C2259
          label: Therapeutic Agent
      value:
        preferred_term: dapsone
        term:
          id: NCIT:C415
          label: Dapsone
  evidence:
  - reference: PMID:27843372
    supports: PARTIAL
    snippet: "A combination of naproxen, dapsone, and prednisolone therapy resulted in significant improvement of the patient’s arthralgias and rash."
    explanation: The case report documents improvement with dapsone as part of combination therapy.
- name: Azathioprine
  description: Immunosuppressive therapy used in combination with corticosteroids.
  treatment_term:
    preferred_term: pharmacotherapy
    term:
      id: MAXO:0000058
      label: pharmacotherapy
    qualifiers:
    - predicate:
        preferred_term: therapeutic agent
        term:
          id: NCIT:C2259
          label: Therapeutic Agent
      value:
        preferred_term: azathioprine
        term:
          id: NCIT:C290
          label: Azathioprine
  evidence:
  - reference: PMID:1210460
    supports: PARTIAL
    snippet: "In the present case the disease could successfully be controlled using imuran in combination with corticosteroids."
    explanation: The abstract reports successful control with azathioprine (imuran) plus steroids.