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ICD-10 Guide
ICD-10 CodesD47.01

D47.01

Billable

Cutaneous mastocytosis

BILLABLE STATUSYes
IMPLEMENTATION DATEOctober 1, 2015
LAST UPDATED09/05/2025

Code Description

ICD-10 D47.01 is a billable code used to indicate a diagnosis of cutaneous mastocytosis.

Key Diagnostic Point:

Cutaneous mastocytosis is a rare skin disorder characterized by an abnormal accumulation of mast cells in the skin. This condition typically presents in infancy or early childhood, manifesting as reddish-brown macules, papules, or plaques that may be itchy or have a tendency to urticate upon pressure or friction, a phenomenon known as Darier's sign. The mast cells in cutaneous mastocytosis can release histamine and other mediators, leading to symptoms such as flushing, pruritus, and in some cases, anaphylaxis. While cutaneous mastocytosis is generally considered benign, there is a potential for progression to systemic mastocytosis, particularly in cases that persist into adulthood. Surveillance is crucial, as patients may develop systemic symptoms or complications over time. Diagnosis is typically confirmed through skin biopsy, which reveals an increased number of mast cells. Treatment focuses on symptom management, including antihistamines for pruritus and avoidance of triggers that may provoke mast cell degranulation. Regular follow-up is recommended to monitor for any signs of progression to systemic involvement.

Code Complexity Analysis

Complexity Rating: Medium

Medium Complexity

Complexity Factors

  • Variability in presentation and symptoms among patients
  • Potential for progression to systemic mastocytosis
  • Need for thorough documentation of clinical findings
  • Differentiation from other skin conditions with similar presentations

Audit Risk Factors

  • Inadequate documentation of symptoms and clinical findings
  • Failure to note the presence of Darier's sign
  • Lack of follow-up documentation for monitoring progression
  • Misclassification of cutaneous mastocytosis as a more severe condition

Specialty Focus

Medical Specialties

Dermatology

Documentation Requirements

Detailed descriptions of skin lesions, biopsy results, and patient history.

Common Clinical Scenarios

Diagnosis of cutaneous mastocytosis in pediatric patients, management of pruritus, and monitoring for systemic symptoms.

Billing Considerations

Ensure accurate documentation of the clinical presentation and any associated symptoms to support the diagnosis.

Allergy and Immunology

Documentation Requirements

Records of allergic reactions, patient history of mast cell-related symptoms, and treatment responses.

Common Clinical Scenarios

Management of mast cell activation symptoms, evaluation of potential triggers, and treatment with antihistamines.

Billing Considerations

Document any systemic symptoms that may indicate progression to systemic mastocytosis.

Coding Guidelines

Inclusion Criteria

Use D47.01 When
  • Follow the official ICD
  • CM coding guidelines for neoplasms of uncertain behavior
  • Ensure that the diagnosis is supported by clinical documentation, including biopsy results and symptom descriptions

Exclusion Criteria

Do NOT use D47.01 When
  • Exclude conditions that may mimic cutaneous mastocytosis

Related ICD-10 Codes

Related CPT Codes

11100CPT Code

Biopsy of skin, excisional

Clinical Scenario

Used when a biopsy is performed to confirm the diagnosis of cutaneous mastocytosis.

Documentation Requirements

Document the reason for the biopsy, the site, and the findings.

Specialty Considerations

Dermatologists should ensure that the biopsy results are clearly linked to the diagnosis.

ICD-10 Impact

Diagnostic & Documentation Impact

Enhanced Specificity

ICD-10 Improvements

The transition to ICD-10 has allowed for more precise coding of cutaneous mastocytosis, improving the ability to track and manage this condition. The specificity of ICD-10 codes helps in understanding the prevalence and treatment outcomes of cutaneous mastocytosis.

ICD-9 vs ICD-10

The transition to ICD-10 has allowed for more precise coding of cutaneous mastocytosis, improving the ability to track and manage this condition. The specificity of ICD-10 codes helps in understanding the prevalence and treatment outcomes of cutaneous mastocytosis.

Reimbursement & Billing Impact

The transition to ICD-10 has allowed for more precise coding of cutaneous mastocytosis, improving the ability to track and manage this condition. The specificity of ICD-10 codes helps in understanding the prevalence and treatment outcomes of cutaneous mastocytosis.

Resources

Clinical References

  • •
    American Academy of Dermatology

Coding & Billing References

  • •
    American Academy of Dermatology

Frequently Asked Questions

What are the common symptoms of cutaneous mastocytosis?

Common symptoms include reddish-brown macules or papules, itching, and the presence of Darier's sign, where lesions urticate upon pressure.

How is cutaneous mastocytosis diagnosed?

Diagnosis is typically made through clinical evaluation and confirmed with a skin biopsy showing an increased number of mast cells.

Is cutaneous mastocytosis serious?

While cutaneous mastocytosis is generally benign, there is a risk of progression to systemic mastocytosis, which can be more serious.

What treatments are available for cutaneous mastocytosis?

Treatment focuses on symptom management, including antihistamines for itching and avoiding known triggers.