AutoICD API

E80.0

Billable

Hereditary erythropoietic porphyria

Hereditary erythropoietic porphyria

Status

Billable / Specific

Block

E70-E88

Parent Code

E80

ICD-11 Mapping

1 equivalent

Coding Notes

Inclusion Terms

Alternative clinical terms for this condition

  • Congenital erythropoietic porphyria
  • Erythropoietic protoporphyria

Includes

Conditions included under this code

  • defects of catalase and peroxidase

Excludes 1

Codes that cannot be used together with this code (mutual exclusion)

Excludes 2

Conditions not included here, but the patient may have both

Related Codes(7)
E80.1

Porphyria cutanea tarda

BillableE80.2

Other and unspecified porphyria

E80.3

Defects of catalase and peroxidase

BillableE80.4

Gilbert syndrome

BillableE80.5

Crigler-Najjar syndrome

BillableE80.6

Other disorders of bilirubin metabolism

BillableE80.7

Disorder of bilirubin metabolism, unspecified

Billable
ICD-11 Equivalents(1)

ICD-11 Equivalents

View full mapping

Corresponding ICD-11 codes from the WHO crosswalk mapping

5C58.12Erythropoietic porphyriasequivalentPrimary
Also Known As / Clinical Terms(149)

SNOMED CT

UMLS

Clinical Terms

  • Erythropoietic Porphyria
  • CEP
  • Pink tooth
  • Porphyrinuria
  • Porphyria, Erythropoietic
  • congenital porphyria
  • Hematoporphyrinuria
  • Erythropoietic Porphyrias
  • Congenital Erythropoietic Porphyria
  • Erythropoietic Porphyrias, Congenital
  • Porphyria, Congenital Erythropoietic
  • GUNTHERS DIS
  • Porphyria, Erythropoietic, Congenital
  • Protoporphyria
  • EPP (erythropoietic protoporphyria porphyria)
  • Ferrochelatase deficiency
  • erythropoietic protoporphyria
  • Uroporphyrinuria
  • PROTOPORPHYRIA, ERYTHROPOIETIC
  • Haematoporphyrinuria
  • FECH - Ferrochelatase deficiency
  • Congenital erythropoietic porphyria (disorder)
  • erythropoietic uroporphyria
  • Gunther Disease
  • Erythrohepatic protoporphyria
  • Erythropoietic protoporphyria (disorder)
  • Protoporphyrias, Erythropoietic
  • Gunthers Disease
  • EPP - erythropoietic protoporphyria
  • CEP - Congenital erythropoietic porphyria
  • Porphyrias, Erythropoietic
  • Erythropoietic Protoporphyrias
  • Uroporphyrinogen III synthase deficiency
  • GUNTHER DIS
  • Hematoporphyria congenita
  • Haem synthase deficiency
  • gunther's disease
  • Porphyrias, Congenital Erythropoietic
  • epp
  • Erythropoietic uroporphyria associated with myeloid malignancy
  • Homozygous erythropoietic protoporphyria
  • Porphyria erythropoietica
  • Heme synthase deficiency
  • X-linked dominant erythropoietic protoporphyria
  • Erythropoietic protoporphyria due to ferrochelatase deficiency
  • Porphyruria
  • magnus syndrome
  • Congenital photosensitive porphyria
  • Haematoporphyria congenita
  • Congenital porphyria (disorder)
  • Erythropoietic Porphyria, Congenital
  • PORPHYRIA ERYTHROPOIETIC CONGEN
  • Erythropoietic protoporphyria due to gain of function of erythroid-specific aminolevulinic acid synthase 2
  • Congenital Erythropoietic Porphyrias
  • protoporphyria erythropoietic
  • Uroporphyria
  • CONGEN ERYTHROPOIETIC PORPHYRIA
Frequently Asked Questions
What is the ICD-10 code for hereditary erythropoietic porphyria?

The ICD-10-CM code for hereditary erythropoietic porphyria is E80.0. The full clinical description is "Hereditary erythropoietic porphyria". E80.0 is a billable/specific code that can be used on insurance claims and medical billing.

What does ICD-10 code E80.0 mean?

ICD-10-CM code E80.0 represents “Hereditary erythropoietic porphyria”. It is classified under Chapter 4: Endocrine, Nutritional and Metabolic Diseases and is a billable/specific code that can be used on a claim.

Is E80.0 a billable code?

Yes, E80.0 is a billable/specific ICD-10-CM code and can be used to indicate a diagnosis on a medical claim.

What chapter is E80.0 in?

E80.0 is in Chapter 4: Endocrine, Nutritional and Metabolic Diseases (codes E00-E89).

What codes cannot be used with E80.0?

E80.0 has Excludes1 notes indicating codes that cannot be used together with it, including: transitory endocrine and metabolic disorders specific to newborn (P70-P74); androgen insensitivity syndrome (E34.5-); congenital adrenal hyperplasia (E25.0); and 3 more.

What SNOMED CT codes does E80.0 map to?

E80.0 maps to 12 SNOMED CT concepts: 22935002, 190913009, 51022005, 67312003, 860859009, and 7 more. SNOMED CT is a clinical terminology used in electronic health records.

What are the UMLS CUIs for E80.0?

E80.0 is linked to 2 UMLS Concept Unique Identifiers: C5886774, C0162568. The UMLS (Unified Medical Language System) integrates multiple biomedical vocabularies maintained by the U.S. National Library of Medicine.

How does E80.0 relate to ICF functioning codes?

ICF (International Classification of Functioning, Disability and Health) codes describe how conditions like hereditary erythropoietic porphyria affect a person's functioning — body functions, activities, participation, and environmental factors. AutoICD provides ICF Core Sets for 12+ conditions and can map clinical text to ICF categories automatically. Browse the ICF directory to explore functioning codes.

What is the ICD-11 equivalent of E80.0?

E80.0 maps to the ICD-11 code: 5C58.12 (Erythropoietic porphyrias).

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Includes SNOMED Clinical Terms® (SNOMED CT®) used by permission of SNOMED International. Includes content from the UMLS Metathesaurus, courtesy of the U.S. National Library of Medicine.