ICD-10: M35.6

Relapsing panniculitis [Weber-Christian]

Additional Information

Approximate Synonyms

Relapsing panniculitis, also known as Weber-Christian disease, is classified under the ICD-10 code M35.6. This condition is characterized by recurrent episodes of inflammation in the subcutaneous fat, leading to painful nodules and systemic symptoms. Understanding the alternative names and related terms for this condition can enhance clarity in medical communication and documentation.

Alternative Names for Relapsing Panniculitis

  1. Weber-Christian Disease: This is the most commonly used alternative name, named after the physicians who first described the condition.
  2. Relapsing Panniculitis: This term emphasizes the recurrent nature of the disease, highlighting its episodic flare-ups.
  3. Panniculitis: A broader term that refers to inflammation of the panniculus, or subcutaneous fat, which can encompass various types of panniculitis, including Weber-Christian disease.
  1. Subcutaneous Fat Inflammation: This term describes the primary pathological process involved in relapsing panniculitis, focusing on the affected tissue.
  2. Systemic Involvement: Refers to the potential for systemic symptoms associated with the disease, such as fever, malaise, and weight loss, which can occur during flare-ups.
  3. Connective Tissue Disease: Relapsing panniculitis may be associated with other connective tissue disorders, making this term relevant in differential diagnosis.
  4. Chronic Panniculitis: This term may be used to describe the long-term nature of the condition, although it is less specific than relapsing panniculitis.

Clinical Context

Relapsing panniculitis is often considered in the context of other inflammatory conditions. It is important for healthcare providers to differentiate it from other types of panniculitis, such as:

  • Lobular Panniculitis: Inflammation that primarily affects the lobules of the fat tissue.
  • Septal Panniculitis: Inflammation that affects the septa (the connective tissue between fat lobules).

Conclusion

Understanding the alternative names and related terms for ICD-10 code M35.6: Relapsing panniculitis [Weber-Christian] is crucial for accurate diagnosis and treatment. This knowledge aids healthcare professionals in communicating effectively about the condition and considering it in the context of broader systemic issues. If you have further questions or need more specific information, feel free to ask!

Description

Relapsing panniculitis, also known as Weber-Christian disease, is classified under the ICD-10 code M35.6. This condition is characterized by recurrent episodes of inflammation in the subcutaneous fat, leading to painful nodules and lesions. Below is a detailed clinical description and relevant information regarding this diagnosis.

Clinical Description

Definition

Relapsing panniculitis is an inflammatory condition that primarily affects the subcutaneous tissue, particularly the fat layer beneath the skin. It is categorized as a type of panniculitis, which refers to inflammation of the panniculus, the layer of fat and connective tissue beneath the skin.

Etiology

The exact cause of relapsing panniculitis remains unclear, but it is believed to be related to autoimmune processes. Some cases may be triggered by infections, trauma, or other underlying systemic diseases. The condition can occur in individuals with no identifiable underlying cause, making it idiopathic in some instances.

Symptoms

Patients with relapsing panniculitis typically present with:
- Painful nodules: These are often located on the extremities, trunk, and buttocks. The nodules can vary in size and may be tender to the touch.
- Erythema: The skin overlying the nodules may appear red and inflamed.
- Fever and malaise: Some patients experience systemic symptoms such as fever, fatigue, and general malaise during flare-ups.
- Recurrence: The hallmark of this condition is the relapsing nature, where episodes of inflammation can occur intermittently over time.

Diagnosis

Diagnosis of relapsing panniculitis is primarily clinical, based on the characteristic presentation of painful nodules and the history of recurrent episodes. Additional diagnostic tools may include:
- Histopathological examination: A biopsy of the affected tissue can reveal the characteristic inflammatory changes associated with panniculitis.
- Exclusion of other conditions: It is essential to rule out other causes of panniculitis, such as infections, vasculitis, or other systemic diseases.

Treatment

Management of relapsing panniculitis focuses on alleviating symptoms and controlling inflammation. Treatment options may include:
- Corticosteroids: Systemic or topical corticosteroids are commonly used to reduce inflammation and manage flare-ups.
- Immunosuppressive agents: In cases resistant to corticosteroids, medications such as methotrexate or azathioprine may be considered.
- Pain management: Analgesics can help manage pain associated with the nodules.

Prognosis

The prognosis for individuals with relapsing panniculitis varies. While some patients may experience frequent recurrences, others may have infrequent episodes. Long-term management may be necessary to control symptoms and prevent complications.

Conclusion

Relapsing panniculitis (ICD-10 code M35.6) is a complex condition characterized by recurrent inflammation of the subcutaneous fat. Understanding its clinical features, diagnostic criteria, and treatment options is crucial for effective management. Patients experiencing symptoms consistent with this condition should seek medical evaluation for appropriate diagnosis and treatment.

Clinical Information

Relapsing panniculitis, also known as Weber-Christian disease, is a rare inflammatory condition characterized by recurrent episodes of painful subcutaneous nodules. This condition is classified under ICD-10 code M35.6. Understanding its clinical presentation, signs, symptoms, and patient characteristics is crucial for accurate diagnosis and management.

Clinical Presentation

Overview

Weber-Christian disease typically presents with recurrent episodes of painful, erythematous nodules located in the subcutaneous tissue. These nodules can vary in size and may be accompanied by systemic symptoms. The condition is often chronic and can lead to significant morbidity due to its recurrent nature.

Signs and Symptoms

  1. Subcutaneous Nodules:
    - The hallmark of relapsing panniculitis is the presence of painful, tender nodules that can appear on various parts of the body, particularly the extremities and trunk. These nodules may fluctuate in size and can resolve spontaneously or with treatment.

  2. Erythema:
    - The affected areas often exhibit erythema (redness) surrounding the nodules, which can be a sign of inflammation.

  3. Systemic Symptoms:
    - Patients may experience systemic symptoms such as:

    • Fever
    • Malaise
    • Fatigue
    • Weight loss
      These symptoms can occur during flare-ups and may indicate a more severe inflammatory response.
  4. Recurrence:
    - The relapsing nature of the disease means that patients often experience multiple episodes over time, with periods of remission in between.

  5. Associated Conditions:
    - In some cases, Weber-Christian disease may be associated with other autoimmune or connective tissue disorders, which can complicate the clinical picture.

Patient Characteristics

Demographics

  • Age: Weber-Christian disease can occur in adults, typically presenting in middle-aged individuals, although it can affect younger adults as well.
  • Gender: There is a slight female predominance in the incidence of this condition, although it can affect both genders.

Risk Factors

  • Autoimmune Disorders: Patients with a history of autoimmune diseases may be at higher risk for developing relapsing panniculitis.
  • Genetic Predisposition: Some studies suggest a potential genetic component, although specific genetic markers have not been definitively identified.

Clinical History

  • Patients often report a history of recurrent episodes of painful nodules, which may have been misdiagnosed initially as infections or other dermatological conditions. A thorough clinical history is essential for accurate diagnosis.

Diagnosis and Management

Diagnosis of Weber-Christian disease is primarily clinical, supported by histological examination of biopsy samples, which typically show lobular panniculitis with a mixed inflammatory infiltrate. Management may include corticosteroids and other immunosuppressive therapies to control inflammation and reduce the frequency of flare-ups.

Conclusion

Relapsing panniculitis (Weber-Christian disease) is characterized by painful subcutaneous nodules, systemic symptoms, and a relapsing course. Understanding its clinical presentation and patient characteristics is vital for healthcare providers to ensure timely diagnosis and appropriate management. Given its potential association with other autoimmune conditions, a comprehensive evaluation is recommended for affected individuals.

Diagnostic Criteria

Relapsing panniculitis, also known as Weber-Christian disease, is a rare inflammatory condition characterized by recurrent episodes of painful nodules in the subcutaneous fat. The diagnosis of this condition, particularly for the ICD-10 code M35.6, involves a combination of clinical evaluation, imaging studies, and histopathological examination. Below, we outline the key criteria and considerations used in the diagnosis of relapsing panniculitis.

Clinical Criteria

  1. Symptoms: Patients typically present with recurrent painful subcutaneous nodules, which may be accompanied by systemic symptoms such as fever, malaise, and weight loss. The nodules often appear on the trunk and extremities and can vary in size.

  2. Duration and Recurrence: The relapsing nature of the condition is a critical factor. Episodes may last for weeks to months, with periods of remission in between. A history of multiple episodes is essential for diagnosis.

  3. Physical Examination: On examination, the nodules are usually tender and may be erythematous. The presence of induration and fluctuation can also be noted.

Laboratory and Imaging Studies

  1. Blood Tests: Routine laboratory tests may show nonspecific signs of inflammation, such as elevated C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). However, these findings are not specific to panniculitis.

  2. Imaging: Ultrasound or MRI may be utilized to assess the extent of subcutaneous involvement and to rule out other conditions such as abscesses or tumors. Imaging can help visualize the characteristic changes in the subcutaneous fat.

Histopathological Examination

  1. Biopsy: A skin biopsy of the affected area is often performed to confirm the diagnosis. Histological findings typically reveal a lobular panniculitis with a mixed inflammatory infiltrate, including lymphocytes, plasma cells, and histiocytes. The presence of necrosis or fat cell degeneration may also be observed.

  2. Differential Diagnosis: It is crucial to differentiate relapsing panniculitis from other forms of panniculitis, such as infectious, traumatic, or autoimmune panniculitis. This differentiation is often guided by the clinical presentation and histopathological findings.

Additional Considerations

  1. Exclusion of Other Conditions: The diagnosis of Weber-Christian disease requires the exclusion of other potential causes of panniculitis, including infections, vasculitis, and systemic diseases like lupus erythematosus or dermatomyositis.

  2. Response to Treatment: While not a diagnostic criterion, the response to corticosteroids or other immunosuppressive therapies can support the diagnosis, as patients often show improvement with these treatments.

Conclusion

The diagnosis of relapsing panniculitis (ICD-10 code M35.6) is multifaceted, relying on a combination of clinical presentation, imaging studies, and histopathological confirmation. Given the complexity and rarity of the condition, a thorough evaluation by a healthcare professional is essential to ensure accurate diagnosis and appropriate management. If you suspect you or someone else may have this condition, consulting a specialist in dermatology or rheumatology is advisable for further assessment and treatment options.

Treatment Guidelines

Relapsing panniculitis, also known as Weber-Christian disease, is a rare inflammatory condition characterized by recurrent episodes of painful subcutaneous nodules and systemic symptoms. The International Classification of Diseases (ICD-10) code for this condition is M35.6. Understanding the standard treatment approaches for this condition is crucial for effective management and improving patient outcomes.

Overview of Relapsing Panniculitis

Relapsing panniculitis primarily affects the subcutaneous fat layer, leading to inflammation and the formation of painful nodules. Patients may experience fever, malaise, and weight loss during flare-ups. The exact etiology remains unclear, but it is thought to involve immune-mediated mechanisms. Diagnosis is typically made through clinical evaluation and may be supported by histological examination of skin biopsies.

Standard Treatment Approaches

1. Pharmacological Treatments

Corticosteroids

Corticosteroids are often the first line of treatment for relapsing panniculitis. They help reduce inflammation and alleviate symptoms during acute flare-ups. Treatment may start with high doses, which are gradually tapered based on the patient's response and the severity of symptoms. Commonly used corticosteroids include prednisone and methylprednisolone.

Immunosuppressive Agents

In cases where corticosteroids are insufficient or if the patient experiences frequent relapses, immunosuppressive agents may be introduced. These can include:
- Methotrexate: Often used for its anti-inflammatory properties and ability to reduce the need for corticosteroids.
- Azathioprine: Another option that can help maintain remission and reduce flare frequency.
- Cyclophosphamide: In severe cases, this may be considered, particularly if there is a risk of systemic involvement.

Biologic Therapies

Emerging treatments include biologic agents that target specific pathways in the immune response. For instance, agents like TNF-alpha inhibitors (e.g., infliximab) have shown promise in treating various inflammatory conditions and may be beneficial for patients with refractory symptoms.

2. Supportive Care

Pain Management

Pain associated with relapsing panniculitis can be significant. Analgesics, including non-steroidal anti-inflammatory drugs (NSAIDs), may be used to manage pain and inflammation during flare-ups.

Lifestyle Modifications

Patients are often advised to adopt lifestyle changes that may help reduce flare-ups. This can include:
- Dietary adjustments: Some patients report improvement with anti-inflammatory diets.
- Stress management: Techniques such as yoga, meditation, or counseling may help reduce stress, which can trigger flare-ups.

3. Monitoring and Follow-Up

Regular follow-up appointments are essential to monitor disease progression, treatment efficacy, and potential side effects of medications. Adjustments to the treatment plan may be necessary based on the patient's response and any emerging complications.

Conclusion

The management of relapsing panniculitis (Weber-Christian disease) involves a combination of pharmacological treatments, supportive care, and lifestyle modifications. Corticosteroids remain the cornerstone of therapy, while immunosuppressive agents and biologics may be considered for more severe or refractory cases. Ongoing monitoring is crucial to ensure optimal management and improve the quality of life for affected individuals. As research continues, new treatment modalities may emerge, offering hope for better management of this challenging condition.

Related Information

Approximate Synonyms

  • Weber-Christian Disease
  • Relapsing Panniculitis
  • Panniculitis
  • Subcutaneous Fat Inflammation
  • Systemic Involvement
  • Connective Tissue Disease
  • Chronic Panniculitis

Description

  • Recurrent episodes of inflammation in subcutaneous fat
  • Painful nodules on extremities, trunk, and buttocks
  • Erythema overlying nodules, red and inflamed
  • Fever and malaise during flare-ups
  • Relapsing nature with intermittent episodes
  • Histopathological examination for diagnosis
  • Exclusion of other conditions such as infections
  • Corticosteroids to reduce inflammation and manage symptoms

Clinical Information

  • Painful subcutaneous nodules on extremities
  • Erythematous areas surrounding the nodules
  • Fever and malaise during flare-ups
  • Recurrence of episodes over time
  • Possible association with autoimmune disorders
  • Female predominance in incidence rate
  • Middle-aged adults typically affected

Diagnostic Criteria

  • Recurring painful subcutaneous nodules
  • Systemic symptoms like fever and malaise
  • Multiple episodes with periods of remission
  • Tender erythematous nodules on examination
  • Elevated CRP and ESR in blood tests
  • Lobular panniculitis with inflammatory infiltrate
  • Necrosis or fat cell degeneration in biopsy

Treatment Guidelines

  • Corticosteroids reduce inflammation
  • Methotrexate reduces corticosteroid use
  • Azathioprine maintains remission and reduces flare-ups
  • Cyclophosphamide used in severe cases
  • TNF-alpha inhibitors show promise for refractory symptoms
  • Analgesics manage pain during flare-ups
  • Dietary adjustments help reduce flare-ups

Coding Guidelines

Excludes 1

  • panniculitis NOS (M79.3-)
  • lupus panniculitis (L93.2)

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