ICD-10-CM 2027
ICD-10 code for Pigmentosa urticaria
From the official ICD-10-CM alphabetic index entry “Urticaria › pigmentosa”. Page updated September 29, 2026.
About coding Pigmentosa urticaria
The ICD-10-CM code for Pigmentosa urticaria is D47.01 (Cutaneous mastocytosis). The official index lists 3 more specific codes, so check the documentation for details such as type, cause, site or severity before settling on D47.01.
Within D47.0 (mast cell neoplasms of uncertain behavior), D47.01 is specifically for cutaneous mastocytosis. Related codes cover systemic mastocytosis (D47.02) and other mast cell neoplasms of uncertain behavior (D47.09).
Don't report D47.01 together with the conditions in its Excludes1 note: congenital (diffuse) (maculopapular) cutaneous mastocytosis (Q82.2), congenital urticaria pigmentosa (Q82.2) and extracutaneous mastocytoma (D47.09).
As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.
More specific Pigmentosa urticaria codes (official index)
Don't confuse with (Excludes1 for D47.01)
Common questions about Pigmentosa urticaria ICD-10 codes
What is the ICD-10 code for Pigmentosa urticaria?
- D47.01 — Cutaneous mastocytosis. More specific codes apply when the documentation supports them: Q82.2, Q82.2, Q82.2.
What is the ICD-10 code for congenital pigmentosa urticaria?
- Q82.2 — Congenital cutaneous mastocytosis.
Is D47.01 billable?
- Yes. D47.01 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can D47.01 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict D47.01 as a principal diagnosis.
Is D47.01 a CC or MCC?
- D47.01 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.