Specific code 2014 ICD-9-CM Diagnosis Code 277.1
Disorders of porphyrin metabolism
  • Billable Code
  • Short description: Dis porphyrin metabolism.
  • ICD-9-CM 277.1 will be replaced by an equivalent ICD-10-CM code (or codes) when the United States transitions from ICD-9-CM to ICD-10-CM on October 1, 2015.
  • ICD-9-CM 277.1 is a billable medical code that can be used to specify a diagnosis on a reimbursement claim.
Disease Synonyms
  • Acute intermittent porphyria
  • Disorder of porphyrin metabolism
  • Erythropoietic protoporphyria
  • Porphyria
  • Porphyria cutanea tarda
  • Porphyrin metabolism disorder
  • Porphyrinuria
  • Porphyruria
  • Protoporphyria
Clinical Information
  • A disease characterized by the presence of large amounts of porphyrins in the blood and urine
  • A diverse group of metabolic diseases characterized by errors in the biosynthetic pathway of heme in the liver, the bone marrow, or both. They are classified by the deficiency of specific enzymes, the tissue site of enzyme defect, or the clinical features that include neurological (acute) or cutaneous (skin lesions). Porphyrias can be hereditary or acquired as a result of toxicity to the hepatic or erythropoietic marrow tissues.
  • A group of genetic or acquired metabolic disorders characterized by defects in the enzymes that are involved in the heme synthesis.
  • Autosomal dominant disorder due to partial deficiency of ferrochelatase, characterized by an excess of protoporphyrin and a wide variety of photosensitive skin changes.
  • Group of disorders characterized by the excessive production of porphyrins or their precursors that arises from abnormalities in the regulation of the porphyrin-heme pathway; acquired porphyrias, which are due to inhibition of enzymes in the metabolic pathway by a drug, toxin or abnormal metabolite, are more common than those which are inherited.
  • Porphyrias are a group of genetic disorders caused by problems with how your body makes a substance called heme. Heme is found throughout the body, especially in your blood and bone marrow, where it carries oxygen. Porphyrias affect the skin or the nervous system. People with the skin type develop blisters, itching, and swelling of their skin when it is exposed to sunlight. The nervous system type is called acute porphyria. Symptoms include pain in the chest or abdomen, vomiting, and diarrhea or constipation. During an attack, symptoms can include muscle numbness, tingling, paralysis, cramping, and personality or mental changes. Certain triggers can cause an attack, including some medicines, smoking, drinking alcohol, infections, stress and sun exposure. Attacks develop over hours or days. They can last for days or weeks. Porphyria can be hard to diagnose. It requires blood, urine and stool tests. Each type has a different treatment. It usually involves medicine, treatment with heme or drawing blood. nih: national institute of diabetes and digestive and kidney diseases
Applies To
  • Hematoporphyria
  • Hematoporphyrinuria
  • Hereditary coproporphyria
  • Porphyria
  • Porphyrinuria
  • Protocoproporphyria
  • Protoporphyria
  • Pyrroloporphyria
Convert to ICD-10-CM: 277.1 converts approximately to:
  • 2014 ICD-10-CM E80.0 Hereditary erythropoietic porphyria
    Or:
  • 2014 ICD-10-CM E80.20 Unspecified porphyria
    Or:
  • 2014 ICD-10-CM E80.29 Other porphyria
ICD-9-CM Volume 2 Index entries containing back-references to 277.1:
  • Coproporphyria, hereditary 277.1
  • Disease, diseased - see also Syndrome
    • Günther's (congenital erythropoietic porphyria) 277.1
  • Disorder - see also Disease
    • porphyrin metabolism 277.1
  • Günther's disease or syndrome (congenital erythropoietic porphyria) 277.1
  • Hematoporphyria (acquired) (congenital) 277.1
  • Hematoporphyrinuria (acquired) (congenital) 277.1
  • Photocoproporphyria 277.1
  • Porphyria (acute) (congenital) (constitutional) (erythropoietic) (familial) (hepatica) (idiopathic) (idiosyncratic) (intermittent) (latent) (mixed hepatic) (photosensitive) (South African genetic) (Swedish) 277.1
    • acquired 277.1
    • cutaneatarda
      • hereditaria 277.1
      • symptomatica 277.1
    • due to drugs
      • correct substance properly administered 277.1
      • overdose or wrong substance given or taken 977.9
        • specified drug - see Table of Drugs and Chemicals
    • secondary 277.1
    • toxic NEC 277.1
    • variegata 277.1
  • Porphyrinuria (acquired) (congenital) (secondary) 277.1
  • Porphyruria (acquired) (congenital) 277.1
  • Protocoproporphyria 277.1
  • Protoporphyria (erythrohepatic) (erythropoietic) 277.1
  • Pyrroloporphyria 277.1
  • Syndrome - see also Disease
    • Günther's (congenital erythropoietic porphyria) 277.1
ICD-9-CM codes are used in medical billing and coding to describe diseases, injuries, symptoms and conditions. ICD-9-CM 277.1 is one of thousands of ICD-9-CM codes used in healthcare. Although ICD-9-CM and CPT codes are largely numeric, they differ in that CPT codes describe medical procedures and services. Can't find a code? Start at the root of ICD-9-CM, check the 2014 ICD-9-CM Index or use the search engine at the top of this page to lookup any code.