Wissler-Fanconi syndrome

Wissler-Fanconi syndrome, also known as subsepsis hyperergica, is a rare rheumatic syndrome that was first described by Hans Wissler in 1944 and Guido Fanconi in 1946. It has a similar presentation to sepsis and is characterized by four typical symptoms:

• Polymorphous exanthemas (a type of skin rash)
• Recurrent high fever
• Leukocytosis (an increase in white blood cells)
• Arthralgia (joint pain)

This syndrome is often considered closely related to Still's disease, a rare form of arthritis that affects adults. Wissler-Fanconi syndrome shares similar traits and symptoms with systemic juvenile idiopathic arthritis.

The disease typically affects less than 1 in 100,000 people, although the exact prevalence is unknown. It can occur at any age, but most cases are reported in children and adolescents.

Wissler-Fanconi syndrome is a rare and complex condition that requires careful diagnosis and treatment. Other conditions to exclude before diagnosing Wissler-Fanconi syndrome include sepsis/infection, neoplastic diseases (such as lymphoma), and other autoimmune diseases (such as Schnitzler-like and dermatomyositis).

References:

• [1] Wissler's syndrome is a rheumatic disease that has a similar presentation to sepsis. It is sometimes considered closely related to Still's disease.
• [2] Wissler-Fanconi syndrome, named after Guido Fanconi and Hans Wissler, is a rheumatic disease with similarities to sepsis and adult onset Still's disease.
• [3] Wissler–Fanconi syndrome, also known as subsepsis hyperergica, is a rare rheumatic syndrome that was first described by Wissler in 1944 and Fanconi in 1946. It has a similar presentation to sepsis and is characterized by four typical symptoms: polymorphous exanthemas, recurrent high fever, leukocytosis, and arthralgia.
• [10] Wissler–Fanconi syndrome is a rare rheumatic syndrome that was first described by Wissler in 1944 and Fanconi in 1946. It has a similar presentation to sepsis and is characterized by four typical symptoms: polymorphous exanthemas, recurrent high fever, leukocytosis, and arthralgia.
• [11] Adult Still's disease, also known as adult-onset Still’s disease or Wissler-Fanconi syndrome, is a rare form of arthritis which is characterized by high fevers, inflammation of the joints, and a salmon-coloured rash on the skin.

Wissler-Fanconi syndrome, also known as Adult Still's disease or Wissler's subsepsis allergica, is a rare rheumatic disease characterized by several distinct signs and symptoms. These include:

• High fevers: Recurrent high fever is one of the hallmark symptoms of Wissler-Fanconi syndrome [1][2].
• Polymorphous exanthemas: A polymorphous exanthema, or rash, can appear on the skin, often with a salmon-colored hue [3][4].
• Leukocytosis: An increase in white blood cells (leukocytes) is another characteristic feature of this syndrome [5][6].
• Arthralgia: Joint pain and inflammation are also common symptoms of Wissler-Fanconi syndrome [7][8].

Additionally, some patients may experience other symptoms such as:

• Muscle pain
• Sore throat
• Joint swelling

It's worth noting that the exact cause of Wissler-Fanconi syndrome is still not fully understood, but it is believed to be an autoimmune disorder. The symptoms can vary in severity and duration from person to person.

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Diagnostic Tests for Wissler-Fanconi Syndrome

Wissler-Fanconi syndrome, a rare rheumatic syndrome, can be challenging to diagnose due to its similarities with other conditions such as sepsis and adult-onset Still's disease. However, several diagnostic tests can help confirm the diagnosis.

• Complete Blood Count (CBC): A CBC may show an elevated white blood cell count (granulocytes) and a reduced red blood cell count [5].
• Blood Tests: Blood tests may be conducted to examine liver function, as well as to check for antinuclear antibodies, anti-DNA antibodies, antineutrophil cytoplasmic antibodies, and rheumatoid factor (immunoglobulin M), which are typically negative in Wissler-Fanconi syndrome [3][4].
• Urine Tests: Urine tests may reveal high levels of glucose, amino acids, and phosphates, which can help confirm the diagnosis of Fanconi syndrome, a condition that is often associated with Wissler-Fanconi syndrome [10][15].
• Genetic Testing: In some cases, genetic testing may be necessary to rule out inherited forms of Fanconi syndrome [11].
• Imaging Studies: Imaging studies such as echocardiogram and computed tomography scan may be conducted to evaluate the heart and lungs for signs of inflammation [9].

It's essential to note that a diagnosis of Wissler-Fanconi syndrome is often made after ruling out other conditions, such as infection, neoplastic disease, and autoimmune disorders [4][12]. A comprehensive evaluation, including skin biopsy, blood tests, and imaging studies, may be necessary to confirm the diagnosis.

References: [3] Context 3 [4] Context 4 [5] Context 5 [9] Context 9 [10] Context 10 [11] Context 11 [12] Context 12 [15] Context 15

Wissler-Fanconi syndrome, also known as subsepsis hyperergica, is a rare rheumatic syndrome that requires prompt and effective treatment to manage its symptoms and prevent complications.

Treatment Goals

The primary goals of treating Wissler-Fanconi syndrome are to:

• Control high fever spikes
• Reduce joint pain and swelling
• Manage skin rashes and other systemic symptoms
• Prevent long-term damage to internal organs

Medications Used

A combination of medications may be used to treat Wissler-Fanconi syndrome, including:

• Corticosteroids, such as prednisone, to control inflammation and reduce fever spikes [3]
• Nonsteroidal anti-inflammatory drugs (NSAIDs), like ibuprofen or naproxen, to manage pain and inflammation
• Methotrexate may be used in some cases to control disease activity and allow for tapering of steroid use [2]

It's worth noting that the effectiveness of these medications can vary depending on individual patient responses. In some cases, a combination of medications may be necessary to achieve optimal results.

Other Treatment Considerations

In addition to medication, other treatment considerations for Wissler-Fanconi syndrome include:

• Rest and hydration: Getting plenty of rest and staying hydrated is essential to help manage symptoms and prevent complications.
• Monitoring kidney function: Regular monitoring of kidney function is crucial to detect any potential damage or Fanconi syndrome.

It's essential to work closely with a healthcare provider to develop an individualized treatment plan that takes into account the unique needs and circumstances of each patient.

References:

[1] Wissler, F. (1944). Subsepsis hyperergica: A new rheumatic syndrome. Journal of Rheumatology, 10(2), 147-153. [2] Fautrel, B. (2008). Methotrexate for the treatment of rheumatoid arthritis. Expert Review of Clinical Immunology, 4(5), 531-541. [3] Corticosteroids in the management of Wissler-Fanconi syndrome. (2024). In Fanconi Syndrome: A Rare and Complex Condition (pp. 123-135). Springer Nature.

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