D47.01
BillableCutaneous mastocytosis
HCC Category Mapping
RxHCCHCC 21 — Hodgkin Lymphoma and Other Cancers
0.000What This Code Means
An abnormal growth of mast cells limited to the skin, causing itching, flushing, or skin lesions.
Coding Tips
- •Confirm diagnosis is limited to skin involvement; systemic involvement would require code D47.02
- •Document skin manifestations and any biopsy confirmation of mast cell infiltration
Clinical Significance
Cutaneous mastocytosis involves abnormal accumulation of mast cells limited to the skin, manifesting as urticaria pigmentosa, solitary mastocytoma, or diffuse cutaneous mastocytosis. While primarily a skin disease, it can cause significant symptoms from mast cell mediator release including flushing, pruritus, and blistering, particularly in pediatric patients where it often resolves spontaneously.
Documentation Requirements
- ✓Documentation should confirm cutaneous-only involvement with no systemic disease, supported by skin biopsy showing mast cell infiltration.
- ✓Tryptase levels should be documented to help exclude systemic disease.
- ✓Describe the skin lesion morphology, distribution, and any symptoms related to mast cell degranulation.
- ✓Darier sign (urtication upon rubbing) should be noted if present.
Excludes 1 — Do NOT code together
Commonly Confused Codes
D47.02 (systemic mastocytosis) involves extracutaneous organ involvement and requires different management.D47.09 (other mast cell neoplasms) covers other mast cell tumors not fitting cutaneous or systemic categories.L50.0 (allergic urticaria) may present similarly but lacks mast cell clonal proliferation.C96.20 (malignant mast cell neoplasm) is used for mast cell leukemia or sarcoma.