ICD-10: L98.2

Febrile neutrophilic dermatosis, commonly known as Sweet syndrome, is a rare skin condition characterized by a sudden onset of fever and painful skin lesions. It is classified under ICD-10 code L98.2. Understanding its clinical presentation, signs, symptoms, and patient characteristics is crucial for accurate diagnosis and management.

Clinical Presentation

Sweet syndrome typically presents with a combination of systemic and dermatological symptoms. The condition can occur in various contexts, including idiopathic cases, associated with malignancies, infections, or autoimmune diseases.

Signs and Symptoms

1. Fever: Patients often experience a high fever, which is one of the hallmark symptoms of Sweet syndrome. The fever can be abrupt and may reach significant levels, often exceeding 38°C (100.4°F) [12][14].

2. Skin Lesions: The skin manifestations are characterized by:
   - Erythematous Papules and Plaques: These lesions are typically tender, raised, and can appear on the face, neck, and upper extremities. They may also be found on the trunk and lower extremities [12][14].
   - Bullae and Ulcerations: In some cases, the lesions can progress to form bullae or ulcerations, which can be painful and may lead to secondary infections [12][14].

3. Neutrophilia: Laboratory findings often reveal an elevated white blood cell count, particularly neutrophils, which is indicative of an inflammatory response [12][14].

4. Other Symptoms: Patients may also report malaise, fatigue, and arthralgia (joint pain), contributing to the overall systemic nature of the syndrome [12][14].

Patient Characteristics

Sweet syndrome can affect individuals of any age but is most commonly seen in adults, particularly women. The following characteristics are often noted:

1. Demographics:
   - Age: Most frequently diagnosed in adults aged 30 to 60 years.
   - Gender: There is a notable female predominance, with women being affected more often than men [12][14].

2. Associated Conditions:
   - Sweet syndrome can be associated with various underlying conditions, including:

   • Malignancies: Particularly hematological cancers such as leukemia and lymphoma.
   • Infections: Certain infections, including those caused by bacteria or viruses, can trigger the syndrome.
   • Autoimmune Diseases: Conditions like inflammatory bowel disease or rheumatoid arthritis may also be linked to the onset of Sweet syndrome [12][14].

3. Triggers: In some cases, the syndrome may be precipitated by medications, particularly those that induce hypersensitivity reactions, or by physical trauma [12][14].

Conclusion

Febrile neutrophilic dermatosis (Sweet syndrome) is a complex condition that requires a high index of suspicion for diagnosis. Its clinical presentation is marked by fever, painful skin lesions, and neutrophilia, with a demographic tendency towards middle-aged women. Understanding these characteristics is essential for healthcare providers to facilitate timely diagnosis and appropriate management, especially considering its potential associations with serious underlying conditions. Early recognition and treatment can significantly improve patient outcomes.

Febrile neutrophilic dermatosis, commonly known as Sweet Syndrome, is characterized by a specific set of clinical and histopathological criteria that guide its diagnosis. The ICD-10 code for this condition is L98.2, which is used for billing and coding purposes in healthcare settings. Below are the key criteria used for diagnosing Sweet Syndrome:

Clinical Criteria

1. Acute Onset: Patients typically present with a sudden onset of fever and malaise, often accompanied by systemic symptoms such as fatigue and weight loss.

2. Skin Lesions: The hallmark of Sweet Syndrome is the presence of painful, erythematous papules or plaques. These lesions are often located on the face, neck, and upper extremities, but can appear anywhere on the body.

3. Neutrophilic Infiltration: The skin lesions are characterized by a dense neutrophilic infiltrate upon histological examination. This is a critical component for diagnosis, as it distinguishes Sweet Syndrome from other dermatoses.

4. Associated Conditions: Sweet Syndrome can be associated with underlying conditions, including hematological malignancies (such as leukemia), infections, or autoimmune diseases. The presence of these associations can support the diagnosis.

Histopathological Criteria

1. Histological Examination: A skin biopsy typically reveals a dense infiltrate of neutrophils in the upper dermis, often with little or no evidence of vasculitis. This neutrophilic infiltration is a defining feature of the condition.

2. Absence of Other Causes: It is essential to rule out other conditions that may present with similar symptoms or skin lesions, such as infections or other inflammatory dermatoses.

Diagnostic Considerations

• Laboratory Tests: Blood tests may show elevated white blood cell counts, particularly neutrophils, and inflammatory markers such as C-reactive protein (CRP) may be elevated.

• Response to Treatment: A positive response to corticosteroid therapy can also support the diagnosis, as patients often improve rapidly with appropriate treatment.

Conclusion

The diagnosis of febrile neutrophilic dermatosis (Sweet Syndrome) relies on a combination of clinical presentation, histopathological findings, and the exclusion of other potential causes. The ICD-10 code L98.2 is specifically designated for this condition, facilitating accurate documentation and treatment planning in clinical practice. Understanding these criteria is crucial for healthcare providers to ensure timely and effective management of this condition.

Febrile neutrophilic dermatosis, commonly known as Sweet syndrome, is characterized by the sudden onset of fever, neutrophilia, and painful skin lesions. The ICD-10 code for this condition is L98.2. Understanding the standard treatment approaches for Sweet syndrome is crucial for effective management and patient care.

Overview of Sweet Syndrome

Sweet syndrome is often associated with underlying conditions such as infections, malignancies, or autoimmune diseases. The skin lesions typically present as erythematous papules or plaques, often accompanied by systemic symptoms like fever and malaise. The exact etiology remains unclear, but it is believed to involve an abnormal immune response, particularly involving neutrophils[3][4].

Standard Treatment Approaches

1. Corticosteroids

Corticosteroids are the first-line treatment for Sweet syndrome. They help reduce inflammation and alleviate symptoms. The treatment can be administered in various forms:

• Oral corticosteroids: Prednisone is commonly prescribed, with dosages typically starting at 0.5 to 1 mg/kg/day. The dosage may be tapered based on the patient's response and the resolution of symptoms[3][5].
• Topical corticosteroids: For localized lesions, high-potency topical steroids may be effective, although they are less commonly used than systemic treatments[4].

2. Immunosuppressive Agents

In cases where corticosteroids are ineffective or if the patient has recurrent episodes, immunosuppressive agents may be considered. These include:

• Dapsone: This medication has shown efficacy in some patients with Sweet syndrome, particularly those with underlying conditions like myeloproliferative disorders[4][6].
• Azathioprine or Methotrexate: These agents may be used in chronic or refractory cases, especially when corticosteroids alone do not provide adequate control[3][5].

3. Supportive Care

Supportive care is essential in managing symptoms and improving the patient's quality of life. This may include:

• Pain management: Analgesics can be prescribed to manage pain associated with skin lesions.
• Hydration and nutrition: Ensuring adequate fluid intake and nutrition is important, especially if the patient experiences fever and malaise[4].

4. Treatment of Underlying Conditions

Since Sweet syndrome can be associated with various underlying conditions, addressing these is crucial. For instance:

• Infections: If an infection is identified, appropriate antimicrobial therapy should be initiated.
• Malignancies: In cases linked to cancer, treating the underlying malignancy may lead to resolution of Sweet syndrome symptoms[3][6].

Conclusion

The management of febrile neutrophilic dermatosis (Sweet syndrome) primarily involves the use of corticosteroids, with additional options including immunosuppressive agents and supportive care. It is essential to monitor the patient closely and adjust treatment based on their response and any underlying conditions. Early recognition and appropriate management can significantly improve outcomes for patients suffering from this condition. If symptoms persist or worsen, further evaluation and a multidisciplinary approach may be necessary to ensure comprehensive care.

Febrile neutrophilic dermatosis, commonly known as Sweet syndrome, is a rare skin condition characterized by the sudden onset of fever and painful skin lesions. The ICD-10-CM code for this condition is L98.2, which specifically identifies febrile neutrophilic dermatosis.

Clinical Features

Symptoms

Sweet syndrome typically presents with the following clinical features:

• Fever: Patients often experience a high fever, which can be one of the first symptoms to appear.
• Skin Lesions: The hallmark of Sweet syndrome is the development of painful, erythematous papules or plaques. These lesions are usually located on the face, neck, and upper extremities but can appear anywhere on the body.
• Neutrophilia: Laboratory tests often reveal an elevated white blood cell count, particularly neutrophils, which is indicative of an inflammatory response.

Pathophysiology

The exact cause of Sweet syndrome remains unclear, but it is believed to be related to an abnormal immune response. It can be triggered by various factors, including infections, malignancies, medications, and autoimmune diseases. The condition is associated with an infiltration of neutrophils into the skin, leading to the characteristic lesions and systemic symptoms.

Diagnosis

Clinical Diagnosis

Diagnosis of Sweet syndrome is primarily clinical, based on the characteristic symptoms and skin findings. A thorough medical history and physical examination are essential.

Histopathological Examination

A skin biopsy may be performed to confirm the diagnosis. Histological findings typically show a dense infiltrate of neutrophils in the upper dermis, often with a lack of vasculitis, which helps differentiate it from other dermatoses.

Laboratory Tests

Blood tests may reveal elevated white blood cell counts, particularly neutrophils, and inflammatory markers such as C-reactive protein (CRP).

Treatment

First-Line Therapy

The primary treatment for Sweet syndrome involves the use of corticosteroids, which can be administered orally or topically, depending on the severity of the condition. Systemic corticosteroids are often effective in rapidly reducing inflammation and alleviating symptoms.

Additional Treatments

In cases where corticosteroids are insufficient or contraindicated, other immunosuppressive agents such as dapsone, colchicine, or azathioprine may be considered. Treatment of any underlying conditions or triggers is also crucial for managing Sweet syndrome effectively.

Prognosis

The prognosis for patients with Sweet syndrome is generally favorable, especially with prompt treatment. Most patients experience a complete resolution of symptoms and skin lesions, although recurrences can occur, particularly if the underlying trigger is not addressed.

Conclusion

Febrile neutrophilic dermatosis (Sweet syndrome) is a distinctive condition marked by fever and painful skin lesions, classified under ICD-10 code L98.2. Understanding its clinical features, diagnostic criteria, and treatment options is essential for effective management. Early recognition and intervention can lead to a favorable outcome for affected individuals.

Febrile neutrophilic dermatosis, commonly known as Sweet's syndrome, is classified under the ICD-10 code L98.2. This condition is characterized by the sudden onset of fever, neutrophilia, and painful skin lesions. Below are alternative names and related terms associated with this diagnosis.

Alternative Names

1. Sweet's Syndrome: The most widely recognized name for febrile neutrophilic dermatosis, named after Dr. Robert Sweet, who first described the condition in 1964.
2. Acute Febrile Neutrophilic Dermatosis: This term emphasizes the acute nature of the condition and its association with fever and neutrophilic infiltration.
3. Sweet Disease: A colloquial term that is often used interchangeably with Sweet's syndrome.

Related Terms

1. Neutrophilic Dermatosis: A broader category that includes various skin conditions characterized by neutrophilic infiltration.
2. Dermatosis: A general term for any skin disease, which can encompass a wide range of conditions, including Sweet's syndrome.
3. Fever: While not a direct synonym, fever is a significant symptom associated with Sweet's syndrome and is part of its diagnostic criteria.
4. Neutrophilia: Refers to an elevated level of neutrophils in the blood, which is a hallmark of Sweet's syndrome.

Clinical Context

Sweet's syndrome can be associated with various underlying conditions, including malignancies, infections, and autoimmune diseases. Understanding these alternative names and related terms can aid healthcare professionals in recognizing and diagnosing the condition effectively.

In summary, febrile neutrophilic dermatosis (L98.2) is primarily known as Sweet's syndrome, with several related terms that highlight its clinical features and associations.