ICD-10: D72.12

Drug rash with eosinophilia and systemic symptoms syndrome

Clinical Information

Inclusion Terms

  • DRESS syndrome

Additional Information

Description

Clinical Description of ICD-10 Code D72.12

ICD-10 Code D72.12 refers to a specific condition known as Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) syndrome. This syndrome is a severe drug-induced hypersensitivity reaction characterized by a combination of symptoms that can affect multiple organ systems. Understanding the clinical features, diagnostic criteria, and management of DRESS syndrome is crucial for healthcare providers.

Key Features of DRESS Syndrome

  1. Eosinophilia:
    - A hallmark of DRESS syndrome is the presence of eosinophilia, which is an elevated level of eosinophils (a type of white blood cell) in the blood. This is indicative of an allergic or hypersensitivity reaction[1].

  2. Rash:
    - Patients typically present with a widespread rash that may be maculopapular or morbilliform. The rash can progress to more severe forms, including exfoliative dermatitis or even toxic epidermal necrolysis in some cases[2].

  3. Systemic Symptoms:
    - DRESS syndrome is characterized by systemic involvement, which may include:

    • Fever
    • Lymphadenopathy (swollen lymph nodes)
    • Hepatitis (liver inflammation)
    • Nephritis (kidney inflammation)
    • Pneumonitis (lung inflammation)
    • Myocarditis (heart inflammation) in severe cases[3][4].
  4. Timing:
    - Symptoms typically appear 2 to 8 weeks after exposure to the offending drug, which is a critical factor in diagnosing DRESS syndrome[5].

Common Drugs Associated with DRESS Syndrome

DRESS syndrome can be triggered by various medications, including but not limited to:
- Anticonvulsants (e.g., phenytoin, carbamazepine)
- Antibiotics (e.g., sulfonamides, minocycline)
- Allopurinol (used for gout)
- Certain non-steroidal anti-inflammatory drugs (NSAIDs) and other agents[6].

Diagnosis

The diagnosis of DRESS syndrome is primarily clinical, based on the presence of the characteristic symptoms and a history of recent drug exposure. The following criteria are often used to aid in diagnosis:
- Fever greater than 38°C (100.4°F)
- Rash
- Lymphadenopathy
- Hematological abnormalities (eosinophilia, atypical lymphocytes)
- Organ involvement (e.g., liver, kidney, lungs) confirmed by laboratory tests[7].

Management

Management of DRESS syndrome involves:
- Immediate discontinuation of the offending drug: This is the most critical step in preventing further complications.
- Supportive care: This may include hydration, electrolyte management, and treatment of specific organ involvement.
- Corticosteroids: Systemic corticosteroids are often used to reduce inflammation and immune response, especially in severe cases[8].

Prognosis

The prognosis for patients with DRESS syndrome varies. While many patients recover completely with appropriate management, some may experience long-term sequelae, particularly if there is significant organ damage. Mortality rates can be as high as 10% in severe cases, emphasizing the importance of early recognition and treatment[9].

Conclusion

ICD-10 code D72.12 encapsulates a serious and potentially life-threatening condition that requires prompt recognition and intervention. Understanding the clinical features, associated drugs, and management strategies is essential for healthcare providers to effectively address this syndrome and improve patient outcomes. If you suspect a case of DRESS syndrome, immediate consultation with a specialist in allergy or dermatology may be warranted for further evaluation and management.


References

  1. ICD-10-CM Codes Eosinophilic-Associated Diseases.
  2. ICD-10 Code for Drug rash with eosinophilia and systemic symptoms.
  3. ICD-10-CM Diagnosis Code D72.12 - Drug rash with eosinophilia and systemic symptoms.
  4. Impact of penicillin allergy label on clinical outcomes.
  5. ICD-10 Coordination and Maintenance Committee Meeting.
  6. Article - Billing and Coding: Lab: Flow Cytometry (A57689).
  7. ICD 10 NCD Manual.
  8. ICD-10-CM.
  9. 2025 ICD-10-CM Diagnosis Code D72.82.

Clinical Information

Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) syndrome is a severe drug-induced hypersensitivity reaction characterized by a constellation of clinical features. Understanding its clinical presentation, signs, symptoms, and patient characteristics is crucial for timely diagnosis and management.

Clinical Presentation

DRESS syndrome typically manifests 2 to 8 weeks after exposure to the offending drug, making it essential for clinicians to have a high index of suspicion, especially in patients with recent medication changes. The syndrome is marked by:

  • Fever: Often the first symptom, it can be high-grade and persistent.
  • Rash: A widespread rash that may be morbilliform (resembling measles) or exfoliative. It can involve the face, trunk, and extremities, and may progress to more severe forms such as erythroderma.
  • Lymphadenopathy: Enlarged lymph nodes are commonly observed, indicating systemic involvement.
  • Hematological abnormalities: Eosinophilia (elevated eosinophil count) is a hallmark of DRESS, along with possible leukopenia (low white blood cell count) and thrombocytopenia (low platelet count).

Signs and Symptoms

The clinical signs and symptoms of DRESS syndrome can be categorized as follows:

Dermatological Signs

  • Rash: Typically starts as a maculopapular eruption, which can evolve into more severe forms, including vesicular or pustular lesions.
  • Exfoliation: In severe cases, the skin may begin to peel, resembling toxic epidermal necrolysis.

Systemic Symptoms

  • Fever: Often exceeding 38°C (100.4°F).
  • Malaise: Generalized feeling of discomfort or illness.
  • Myalgia: Muscle pain may be present.
  • Arthralgia: Joint pain is also common.

Organ Involvement

DRESS syndrome can affect multiple organ systems, leading to:
- Hepatic involvement: Elevated liver enzymes (transaminases) indicating liver dysfunction.
- Renal impairment: Possible acute kidney injury.
- Pulmonary symptoms: Such as cough or dyspnea, indicating potential lung involvement.
- Cardiac symptoms: Rarely, myocarditis may occur.

Patient Characteristics

Certain patient characteristics may predispose individuals to DRESS syndrome:

  • Age: While DRESS can occur at any age, it is more frequently reported in adults.
  • Gender: Some studies suggest a slight male predominance.
  • Genetic predisposition: Certain genetic factors, such as specific human leukocyte antigen (HLA) alleles, have been associated with an increased risk of DRESS syndrome, particularly with drugs like carbamazepine and allopurinol.
  • History of drug allergies: Patients with a history of drug allergies or previous hypersensitivity reactions may be at higher risk.
  • Comorbidities: Conditions such as HIV or autoimmune diseases may increase susceptibility to drug reactions.

Conclusion

DRESS syndrome (ICD-10 code D72.12) is a serious condition that requires prompt recognition and management. Clinicians should be vigilant for its characteristic signs and symptoms, particularly in patients with recent drug exposure. Early identification and discontinuation of the offending agent, along with supportive care, are critical to improving patient outcomes. Given the potential for severe complications, awareness of this syndrome is essential in clinical practice.

Approximate Synonyms

The ICD-10 code D72.12 refers specifically to "Drug rash with eosinophilia and systemic symptoms syndrome" (DRESS syndrome). This condition is characterized by a severe drug-induced reaction that includes a combination of fever, rash, lymphadenopathy, hematological abnormalities (such as eosinophilia), and internal organ involvement. Below are alternative names and related terms associated with this syndrome:

Alternative Names for DRESS Syndrome

  1. Drug Reaction with Eosinophilia and Systemic Symptoms: This is the full form of the acronym DRESS and is often used interchangeably.
  2. DRESS Syndrome: The commonly used abbreviation for the condition.
  3. Drug-Induced Hypersensitivity Syndrome: This term emphasizes the hypersensitivity aspect of the reaction.
  4. Eosinophilic Drug Reaction: This name highlights the eosinophilia that is a hallmark of the syndrome.
  5. Drug-Induced Eosinophilia: A term that focuses on the eosinophilic response to drug exposure.
  1. Eosinophilia: A condition characterized by an elevated eosinophil count in the blood, which is a significant feature of DRESS syndrome.
  2. Systemic Symptoms: Refers to the wide-ranging effects on the body that can occur with DRESS, including fever and organ involvement.
  3. Cutaneous Adverse Drug Reactions (CADR): A broader category that includes various skin reactions to drugs, of which DRESS is a severe form.
  4. Severe Cutaneous Adverse Reactions (SCARs): This term encompasses severe reactions like DRESS, Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN).
  5. Drug-Induced Eosinophilic Pneumonia: While not synonymous, this condition can occur in the context of drug reactions and may share some clinical features with DRESS.

Clinical Context

DRESS syndrome is often triggered by specific medications, including anticonvulsants, antibiotics, and allopurinol. The syndrome typically manifests 2 to 8 weeks after exposure to the offending drug, making it crucial for healthcare providers to maintain a high index of suspicion when patients present with the characteristic symptoms following drug exposure.

Conclusion

Understanding the alternative names and related terms for ICD-10 code D72.12 is essential for accurate diagnosis and communication among healthcare professionals. Recognizing the various terminologies can aid in the identification and management of this serious condition, ensuring timely intervention and improved patient outcomes.

Diagnostic Criteria

The diagnosis of Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) syndrome, which is classified under the ICD-10 code D72.12, involves a combination of clinical criteria and laboratory findings. This condition is a severe drug-induced hypersensitivity reaction characterized by a triad of symptoms: a drug rash, eosinophilia, and systemic involvement. Below are the key criteria used for diagnosis:

Clinical Criteria

  1. Drug Exposure: A history of exposure to a potential offending drug is essential. Common drugs associated with DRESS include anticonvulsants, antibiotics, and allopurinol.

  2. Rash: The presence of a widespread rash is a hallmark of DRESS. The rash can vary in appearance but often resembles a morbilliform or erythematous eruption.

  3. Eosinophilia: Laboratory tests typically reveal eosinophilia, defined as an eosinophil count greater than 1,500 cells per microliter of blood. This finding is crucial for the diagnosis.

  4. Systemic Symptoms: Patients often exhibit systemic symptoms, which may include:
    - Fever
    - Lymphadenopathy
    - Hepatitis (elevated liver enzymes)
    - Hematological abnormalities (such as thrombocytopenia or leukopenia)
    - Renal involvement (elevated creatinine levels)

  5. Organ Involvement: DRESS syndrome can affect multiple organ systems, including the liver, kidneys, lungs, heart, and gastrointestinal tract. The presence of at least one organ involvement is typically required for diagnosis.

Laboratory Findings

  • Eosinophil Count: As mentioned, eosinophilia is a critical laboratory finding.
  • Liver Function Tests: Elevated liver enzymes (ALT, AST) may indicate hepatic involvement.
  • Renal Function Tests: Assessment of kidney function through serum creatinine levels is important.
  • Complete Blood Count (CBC): This may reveal other hematological abnormalities, such as leukopenia or thrombocytopenia.

Diagnostic Considerations

  • Exclusion of Other Conditions: It is important to rule out other potential causes of the symptoms, such as viral infections (e.g., Epstein-Barr virus, cytomegalovirus) or other autoimmune conditions.
  • Timing of Symptoms: Symptoms typically appear 2 to 8 weeks after drug exposure, which can help differentiate DRESS from other drug reactions.

Conclusion

The diagnosis of DRESS syndrome (ICD-10 code D72.12) is based on a combination of clinical presentation, laboratory findings, and a history of drug exposure. Recognizing the symptoms and understanding the criteria are crucial for timely diagnosis and management, as DRESS can lead to significant morbidity and mortality if not addressed promptly. If you suspect DRESS syndrome, it is essential to consult a healthcare professional for further evaluation and management.

Treatment Guidelines

Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) syndrome, classified under ICD-10 code D72.12, is a severe drug-induced hypersensitivity reaction characterized by a triad of symptoms: fever, rash, and hematological abnormalities, particularly eosinophilia. This condition can lead to significant morbidity and, in some cases, mortality if not managed appropriately. Below is a detailed overview of standard treatment approaches for DRESS syndrome.

Understanding DRESS Syndrome

Definition and Symptoms

DRESS syndrome typically manifests 2 to 8 weeks after exposure to the offending drug. Key symptoms include:
- Fever: Often the first sign, indicating systemic involvement.
- Rash: Can vary from mild to severe, often resembling other dermatological conditions.
- Eosinophilia: Elevated eosinophil counts in the blood, which is a hallmark of the syndrome.
- Lymphadenopathy: Swollen lymph nodes may also be present.
- Organ involvement: This can include hepatitis, nephritis, pneumonitis, and myocarditis, leading to multi-organ dysfunction in severe cases[1][2].

Standard Treatment Approaches

Immediate Actions

  1. Discontinuation of the Offending Drug: The first and most critical step in managing DRESS syndrome is the immediate withdrawal of the suspected drug. This action can significantly reduce the severity of symptoms and prevent further complications[3].

Supportive Care

  1. Symptomatic Treatment: Supportive care is essential and may include:
    - Antipyretics: To manage fever.
    - Topical corticosteroids: For localized skin rashes.
    - Hydration: Ensuring adequate fluid intake to support kidney function and overall health.

Pharmacological Interventions

  1. Corticosteroids: Systemic corticosteroids are the cornerstone of treatment for DRESS syndrome. They help to reduce inflammation and modulate the immune response. The typical regimen may start with high doses (e.g., prednisone 1-2 mg/kg/day) and can be tapered based on clinical response and resolution of symptoms[4][5].

  2. Immunosuppressive Agents: In cases where corticosteroids are insufficient or if there is significant organ involvement, additional immunosuppressive therapies may be considered. These can include:
    - Cyclosporine: Often used for its immunomodulatory effects.
    - Mycophenolate mofetil: Another option for managing severe cases.
    - Intravenous immunoglobulin (IVIG): May be beneficial in severe cases, particularly with significant skin involvement or organ dysfunction[6].

Monitoring and Follow-Up

  1. Regular Monitoring: Patients with DRESS syndrome require close monitoring for potential complications, including organ failure. Regular blood tests to assess eosinophil levels, liver function, and renal function are crucial during treatment[7].

  2. Long-term Follow-up: After recovery, patients should be monitored for potential late-onset complications, including autoimmune disorders, which can occur following DRESS syndrome. Education about avoiding the offending drug and similar agents is also essential for long-term management[8].

Conclusion

DRESS syndrome is a serious condition that necessitates prompt recognition and intervention. The standard treatment approach focuses on the immediate cessation of the offending drug, supportive care, and the use of corticosteroids to manage inflammation and immune response. Close monitoring and follow-up are critical to ensure recovery and prevent complications. As with any medical condition, individual treatment plans may vary based on the patient's specific circumstances and the severity of the syndrome.

For further information or specific case management, consulting with a specialist in dermatology or immunology may be beneficial.

Related Information

Description

  • Eosinophilia is a hallmark symptom
  • Widespread rash can occur
  • Systemic symptoms include fever and lymphadenopathy
  • Symptoms appear 2 to 8 weeks after drug exposure
  • Common drugs associated with DRESS syndrome include anticonvulsants and antibiotics
  • Diagnosis based on clinical features and recent drug exposure
  • Management involves discontinuing offending drug and providing supportive care

Clinical Information

  • Fever often first symptom
  • Rash widespread and persistent
  • Lymphadenopathy indicates systemic involvement
  • Eosinophilia hallmark of DRESS syndrome
  • Severe rash can lead to erythroderma
  • Hepatic involvement common in DRESS
  • Renal impairment possible complication
  • Pulmonary symptoms indicate lung involvement
  • Cardiac symptoms rare but possible
  • Genetic predisposition increases risk
  • History of drug allergies increases risk

Approximate Synonyms

  • Drug Reaction with Eosinophilia
  • DRESS Syndrome
  • Drug-Induced Hypersensitivity
  • Eosinophilic Drug Reaction
  • Drug-Induced Eosinophilia

Diagnostic Criteria

  • History of drug exposure
  • Widespread rash appearance
  • Eosinophil count > 1,500 cells/μL
  • Fever or lymphadenopathy present
  • Elevated liver enzymes or hepatitis
  • Hematological abnormalities present
  • Renal involvement indicated
  • Organ system involvement (at least one)

Treatment Guidelines

  • Discontinue offending drug
  • Antipyretics for fever
  • Topical corticosteroids for rashes
  • Hydration for overall health
  • Corticosteroids 1-2mg/kg/day for inflammation
  • Immunosuppressive agents for severe cases
  • Regular monitoring of eosinophil levels and organ function

Coding Guidelines

Use Additional Code

  • code for adverse effect, if applicable, to identify drug (T36-T50 with fifth or sixth character 5)

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