Porphyria Cutanea Tarda M003
Current RMA Instruments
| Reasonable Hypothesis SOP | 69 of 2021 |
| Balance of Probabilities SOP | 70 of 2021 |
Changes from previous Instruments
ICD Coding
- ICD-9-CM Codes: 277.1
- ICD-10-AM Codes: E80.1
Brief description
This is predominantly a skin ("cutanea") disease, caused by a deficiency of a liver enzyme involved in the metabolism of porphyrin. This leads to an excess of porphyrins in the blood ("porphyria"). The term ‘tarda’ refers to the typically late onset of the condition. Porphyrins are photosensitising when transported to the skin and cause skin damage on exposure to light. The condition can be familial (hereditary) or acquired.
Confirming the diagnosis
This diagnosis is based on the clinical presentation, laboratory screening for total porphyrin levels in either plasma or urine, and then specific testing, including porphyrin fractionation, if the screening test is positive.
The relevant medical specialist is a dermatologist.
Additional diagnoses covered by SOP
- chemical, symptomatic, or toxic porphyria
Conditions excluded from SOP
- Erythropoietic protoporphyria#
- Hepatoerythropoietic porphyria#
- Hereditary coproporphyria#
- X-linked protoporphyria#
- other porphyrias#
# non-SOP condition
Clinical onset
Porphyria cutanea tarda (PCT) is generally a disease of adults. It usually presents in mid or later life. The usual major manifesation is chronic blistering of the skin. Other manifestations include skin fragility, scarring, and hyper- and hypopigmentation affecting sun-exposed areas. Liver involvement/damage is also common.
Clinical worsening
PCT is a readily treatable condition with a good prognosis. Relapses may occur following successful treatment. Phlebotomy (drawing blood) and hydroxychloroquine therapy and the main forms of treatment.
Source URL: https://clik.dva.gov.au/ccps-medical-research-library/sops-grouped-icd-body-system/n-p/porphyria-cutanea-tarda-m003-e801
Rulebase for porphyria cutanea tarda
<h5>Current RMA Instruments</h5><table border="1" cellspacing="1" cellpadding="1"><tbody><tr><td><address><a href="http://www.rma.gov.au/assets/SOP/2021/f6b57ca8e1/069.pdf" target="_blank">Reasonable Hypothesis SOP</a></address></td><td>69 of 2021</td></tr><tr><td><address><address><a href="http://www.rma.gov.au/assets/SOP/2021/e25df436a1/070.pdf" target="_blank">Balance of Probabilities SOP</a></address></address></td><td>70 of 2021</td></tr></tbody></table><h5>Changes from previous Instruments</h5><p><drupal-media data-entity-type="media" data-entity-uuid="c79ea6b1-75ac-4da8-ba2c-7e3951b5f183" data-view-mode="wysiwyg"></drupal-media></p><h5>ICD Coding</h5><ul><li>ICD-9-CM Codes: 277.1</li><li>ICD-10-AM Codes: E80.1</li></ul><h5>Brief description</h5><p>This is predominantly a skin ("cutanea") disease, caused by a deficiency of a liver enzyme involved in the metabolism of porphyrin. This leads to an excess of porphyrins in the blood ("porphyria"). The term ‘tarda’ refers to the typically late onset of the condition. Porphyrins are photosensitising when transported to the skin and cause skin damage on exposure to light. The condition can be familial (hereditary) or acquired.</p><h5>Confirming the diagnosis</h5><p>This diagnosis is based on the clinical presentation, laboratory screening for total porphyrin levels in either plasma or urine, and then specific testing, including porphyrin fractionation, if the screening test is positive.</p><p>The relevant medical specialist is a dermatologist.</p><h5>Additional diagnoses covered by SOP</h5><ul><li>chemical, symptomatic, or toxic porphyria</li></ul><h5>Conditions excluded from SOP</h5><ul><li>Erythropoietic protoporphyria<sup><font size="2">#</font></sup></li><li>Hepatoerythropoietic porphyria<sup><font size="2">#</font></sup></li><li>Hereditary coproporphyria<sup><font size="2">#</font></sup></li><li>X-linked protoporphyria<sup><font size="2">#</font></sup></li><li>other porphyrias<sup><font size="2">#</font></sup></li></ul><p><sup><font size="2"># </font></sup><span lang="EN-AU" xml:lang="EN-AU">non-SOP condition</span></p><h5>Clinical onset</h5><p>Porphyria cutanea tarda (PCT) is generally a disease of adults. It usually presents in mid or later life. The usual major manifesation is chronic blistering of the skin. Other manifestations include skin fragility, scarring, and hyper- and hypopigmentation affecting sun-exposed areas. Liver involvement/damage is also common.</p><h5>Clinical worsening</h5><p>PCT is a readily treatable condition with a good prognosis. Relapses may occur following successful treatment. Phlebotomy (drawing blood) and hydroxychloroquine therapy and the main forms of treatment.</p><p> </p><p> </p>
Source URL: https://clik.dva.gov.au/ccps-medical-research-library/statements-principles/n-p/rulebase-porphyria-cutanea-tarda
Alcohol dependence or alcohol abuse
Current RMA Instruments
| Reasonable Hypothesis SOP | 69 of 2021 |
| Balance of Probabilities SOP | 70 of 2021 |
Changes from previous Instruments
ICD Coding
- ICD-9-CM Codes: 277.1
- ICD-10-AM Codes: E80.1
Brief description
This is predominantly a skin ("cutanea") disease, caused by a deficiency of a liver enzyme involved in the metabolism of porphyrin. This leads to an excess of porphyrins in the blood ("porphyria"). The term ‘tarda’ refers to the typically late onset of the condition. Porphyrins are photosensitising when transported to the skin and cause skin damage on exposure to light. The condition can be familial (hereditary) or acquired.
Confirming the diagnosis
This diagnosis is based on the clinical presentation, laboratory screening for total porphyrin levels in either plasma or urine, and then specific testing, including porphyrin fractionation, if the screening test is positive.
The relevant medical specialist is a dermatologist.
Additional diagnoses covered by SOP
- chemical, symptomatic, or toxic porphyria
Conditions excluded from SOP
- Erythropoietic protoporphyria#
- Hepatoerythropoietic porphyria#
- Hereditary coproporphyria#
- X-linked protoporphyria#
- other porphyrias#
# non-SOP condition
Clinical onset
Porphyria cutanea tarda (PCT) is generally a disease of adults. It usually presents in mid or later life. The usual major manifesation is chronic blistering of the skin. Other manifestations include skin fragility, scarring, and hyper- and hypopigmentation affecting sun-exposed areas. Liver involvement/damage is also common.
Clinical worsening
PCT is a readily treatable condition with a good prognosis. Relapses may occur following successful treatment. Phlebotomy (drawing blood) and hydroxychloroquine therapy and the main forms of treatment.
Source URL: https://clik.dva.gov.au/ccps-medical-research-library/statements-principles/n-p/porphyria-cutanea-tarda-m003-e801/rulebase-porphyria-cutanea-tarda/alcohol-dependence-or-alcohol-abuse
Alcoholic liver disease
Current RMA Instruments
| Reasonable Hypothesis SOP | 69 of 2021 |
| Balance of Probabilities SOP | 70 of 2021 |
Changes from previous Instruments
ICD Coding
- ICD-9-CM Codes: 277.1
- ICD-10-AM Codes: E80.1
Brief description
This is predominantly a skin ("cutanea") disease, caused by a deficiency of a liver enzyme involved in the metabolism of porphyrin. This leads to an excess of porphyrins in the blood ("porphyria"). The term ‘tarda’ refers to the typically late onset of the condition. Porphyrins are photosensitising when transported to the skin and cause skin damage on exposure to light. The condition can be familial (hereditary) or acquired.
Confirming the diagnosis
This diagnosis is based on the clinical presentation, laboratory screening for total porphyrin levels in either plasma or urine, and then specific testing, including porphyrin fractionation, if the screening test is positive.
The relevant medical specialist is a dermatologist.
Additional diagnoses covered by SOP
- chemical, symptomatic, or toxic porphyria
Conditions excluded from SOP
- Erythropoietic protoporphyria#
- Hepatoerythropoietic porphyria#
- Hereditary coproporphyria#
- X-linked protoporphyria#
- other porphyrias#
# non-SOP condition
Clinical onset
Porphyria cutanea tarda (PCT) is generally a disease of adults. It usually presents in mid or later life. The usual major manifesation is chronic blistering of the skin. Other manifestations include skin fragility, scarring, and hyper- and hypopigmentation affecting sun-exposed areas. Liver involvement/damage is also common.
Clinical worsening
PCT is a readily treatable condition with a good prognosis. Relapses may occur following successful treatment. Phlebotomy (drawing blood) and hydroxychloroquine therapy and the main forms of treatment.
Source URL: https://clik.dva.gov.au/ccps-medical-research-library/statements-principles/n-p/porphyria-cutanea-tarda-m003-e801/rulebase-porphyria-cutanea-tarda/alcoholic-liver-disease
Cirrhosis of the liver
Current RMA Instruments
| Reasonable Hypothesis SOP | 69 of 2021 |
| Balance of Probabilities SOP | 70 of 2021 |
Changes from previous Instruments
ICD Coding
- ICD-9-CM Codes: 277.1
- ICD-10-AM Codes: E80.1
Brief description
This is predominantly a skin ("cutanea") disease, caused by a deficiency of a liver enzyme involved in the metabolism of porphyrin. This leads to an excess of porphyrins in the blood ("porphyria"). The term ‘tarda’ refers to the typically late onset of the condition. Porphyrins are photosensitising when transported to the skin and cause skin damage on exposure to light. The condition can be familial (hereditary) or acquired.
Confirming the diagnosis
This diagnosis is based on the clinical presentation, laboratory screening for total porphyrin levels in either plasma or urine, and then specific testing, including porphyrin fractionation, if the screening test is positive.
The relevant medical specialist is a dermatologist.
Additional diagnoses covered by SOP
- chemical, symptomatic, or toxic porphyria
Conditions excluded from SOP
- Erythropoietic protoporphyria#
- Hepatoerythropoietic porphyria#
- Hereditary coproporphyria#
- X-linked protoporphyria#
- other porphyrias#
# non-SOP condition
Clinical onset
Porphyria cutanea tarda (PCT) is generally a disease of adults. It usually presents in mid or later life. The usual major manifesation is chronic blistering of the skin. Other manifestations include skin fragility, scarring, and hyper- and hypopigmentation affecting sun-exposed areas. Liver involvement/damage is also common.
Clinical worsening
PCT is a readily treatable condition with a good prognosis. Relapses may occur following successful treatment. Phlebotomy (drawing blood) and hydroxychloroquine therapy and the main forms of treatment.
Source URL: https://clik.dva.gov.au/ccps-medical-research-library/statements-principles/n-p/porphyria-cutanea-tarda-m003-e801/rulebase-porphyria-cutanea-tarda/cirrhosis-liver
Haemochromatosis
Current RMA Instruments
| Reasonable Hypothesis SOP | 69 of 2021 |
| Balance of Probabilities SOP | 70 of 2021 |
Changes from previous Instruments
ICD Coding
- ICD-9-CM Codes: 277.1
- ICD-10-AM Codes: E80.1
Brief description
This is predominantly a skin ("cutanea") disease, caused by a deficiency of a liver enzyme involved in the metabolism of porphyrin. This leads to an excess of porphyrins in the blood ("porphyria"). The term ‘tarda’ refers to the typically late onset of the condition. Porphyrins are photosensitising when transported to the skin and cause skin damage on exposure to light. The condition can be familial (hereditary) or acquired.
Confirming the diagnosis
This diagnosis is based on the clinical presentation, laboratory screening for total porphyrin levels in either plasma or urine, and then specific testing, including porphyrin fractionation, if the screening test is positive.
The relevant medical specialist is a dermatologist.
Additional diagnoses covered by SOP
- chemical, symptomatic, or toxic porphyria
Conditions excluded from SOP
- Erythropoietic protoporphyria#
- Hepatoerythropoietic porphyria#
- Hereditary coproporphyria#
- X-linked protoporphyria#
- other porphyrias#
# non-SOP condition
Clinical onset
Porphyria cutanea tarda (PCT) is generally a disease of adults. It usually presents in mid or later life. The usual major manifesation is chronic blistering of the skin. Other manifestations include skin fragility, scarring, and hyper- and hypopigmentation affecting sun-exposed areas. Liver involvement/damage is also common.
Clinical worsening
PCT is a readily treatable condition with a good prognosis. Relapses may occur following successful treatment. Phlebotomy (drawing blood) and hydroxychloroquine therapy and the main forms of treatment.
Source URL: https://clik.dva.gov.au/ccps-medical-research-library/statements-principles/n-p/porphyria-cutanea-tarda-m003-e801/rulebase-porphyria-cutanea-tarda/haemochromatosis
Haemodialysis
Current RMA Instruments
| Reasonable Hypothesis SOP | 69 of 2021 |
| Balance of Probabilities SOP | 70 of 2021 |
Changes from previous Instruments
ICD Coding
- ICD-9-CM Codes: 277.1
- ICD-10-AM Codes: E80.1
Brief description
This is predominantly a skin ("cutanea") disease, caused by a deficiency of a liver enzyme involved in the metabolism of porphyrin. This leads to an excess of porphyrins in the blood ("porphyria"). The term ‘tarda’ refers to the typically late onset of the condition. Porphyrins are photosensitising when transported to the skin and cause skin damage on exposure to light. The condition can be familial (hereditary) or acquired.
Confirming the diagnosis
This diagnosis is based on the clinical presentation, laboratory screening for total porphyrin levels in either plasma or urine, and then specific testing, including porphyrin fractionation, if the screening test is positive.
The relevant medical specialist is a dermatologist.
Additional diagnoses covered by SOP
- chemical, symptomatic, or toxic porphyria
Conditions excluded from SOP
- Erythropoietic protoporphyria#
- Hepatoerythropoietic porphyria#
- Hereditary coproporphyria#
- X-linked protoporphyria#
- other porphyrias#
# non-SOP condition
Clinical onset
Porphyria cutanea tarda (PCT) is generally a disease of adults. It usually presents in mid or later life. The usual major manifesation is chronic blistering of the skin. Other manifestations include skin fragility, scarring, and hyper- and hypopigmentation affecting sun-exposed areas. Liver involvement/damage is also common.
Clinical worsening
PCT is a readily treatable condition with a good prognosis. Relapses may occur following successful treatment. Phlebotomy (drawing blood) and hydroxychloroquine therapy and the main forms of treatment.
Source URL: https://clik.dva.gov.au/ccps-medical-research-library/statements-principles/n-p/porphyria-cutanea-tarda-m003-e801/rulebase-porphyria-cutanea-tarda/haemodialysis
Halogenated aromatic hydrocarbons
Current RMA Instruments
| Reasonable Hypothesis SOP | 69 of 2021 |
| Balance of Probabilities SOP | 70 of 2021 |
Changes from previous Instruments
ICD Coding
- ICD-9-CM Codes: 277.1
- ICD-10-AM Codes: E80.1
Brief description
This is predominantly a skin ("cutanea") disease, caused by a deficiency of a liver enzyme involved in the metabolism of porphyrin. This leads to an excess of porphyrins in the blood ("porphyria"). The term ‘tarda’ refers to the typically late onset of the condition. Porphyrins are photosensitising when transported to the skin and cause skin damage on exposure to light. The condition can be familial (hereditary) or acquired.
Confirming the diagnosis
This diagnosis is based on the clinical presentation, laboratory screening for total porphyrin levels in either plasma or urine, and then specific testing, including porphyrin fractionation, if the screening test is positive.
The relevant medical specialist is a dermatologist.
Additional diagnoses covered by SOP
- chemical, symptomatic, or toxic porphyria
Conditions excluded from SOP
- Erythropoietic protoporphyria#
- Hepatoerythropoietic porphyria#
- Hereditary coproporphyria#
- X-linked protoporphyria#
- other porphyrias#
# non-SOP condition
Clinical onset
Porphyria cutanea tarda (PCT) is generally a disease of adults. It usually presents in mid or later life. The usual major manifesation is chronic blistering of the skin. Other manifestations include skin fragility, scarring, and hyper- and hypopigmentation affecting sun-exposed areas. Liver involvement/damage is also common.
Clinical worsening
PCT is a readily treatable condition with a good prognosis. Relapses may occur following successful treatment. Phlebotomy (drawing blood) and hydroxychloroquine therapy and the main forms of treatment.
Source URL: https://clik.dva.gov.au/ccps-medical-research-library/statements-principles/n-p/porphyria-cutanea-tarda-m003-e801/rulebase-porphyria-cutanea-tarda/halogenated-aromatic-hydrocarbons
Hepatic haemosiderosis
Current RMA Instruments
| Reasonable Hypothesis SOP | 69 of 2021 |
| Balance of Probabilities SOP | 70 of 2021 |
Changes from previous Instruments
ICD Coding
- ICD-9-CM Codes: 277.1
- ICD-10-AM Codes: E80.1
Brief description
This is predominantly a skin ("cutanea") disease, caused by a deficiency of a liver enzyme involved in the metabolism of porphyrin. This leads to an excess of porphyrins in the blood ("porphyria"). The term ‘tarda’ refers to the typically late onset of the condition. Porphyrins are photosensitising when transported to the skin and cause skin damage on exposure to light. The condition can be familial (hereditary) or acquired.
Confirming the diagnosis
This diagnosis is based on the clinical presentation, laboratory screening for total porphyrin levels in either plasma or urine, and then specific testing, including porphyrin fractionation, if the screening test is positive.
The relevant medical specialist is a dermatologist.
Additional diagnoses covered by SOP
- chemical, symptomatic, or toxic porphyria
Conditions excluded from SOP
- Erythropoietic protoporphyria#
- Hepatoerythropoietic porphyria#
- Hereditary coproporphyria#
- X-linked protoporphyria#
- other porphyrias#
# non-SOP condition
Clinical onset
Porphyria cutanea tarda (PCT) is generally a disease of adults. It usually presents in mid or later life. The usual major manifesation is chronic blistering of the skin. Other manifestations include skin fragility, scarring, and hyper- and hypopigmentation affecting sun-exposed areas. Liver involvement/damage is also common.
Clinical worsening
PCT is a readily treatable condition with a good prognosis. Relapses may occur following successful treatment. Phlebotomy (drawing blood) and hydroxychloroquine therapy and the main forms of treatment.
Source URL: https://clik.dva.gov.au/ccps-medical-research-library/statements-principles/n-p/porphyria-cutanea-tarda-m003-e801/rulebase-porphyria-cutanea-tarda/hepatic-haemosiderosis
Hepatitis
Current RMA Instruments
| Reasonable Hypothesis SOP | 69 of 2021 |
| Balance of Probabilities SOP | 70 of 2021 |
Changes from previous Instruments
ICD Coding
- ICD-9-CM Codes: 277.1
- ICD-10-AM Codes: E80.1
Brief description
This is predominantly a skin ("cutanea") disease, caused by a deficiency of a liver enzyme involved in the metabolism of porphyrin. This leads to an excess of porphyrins in the blood ("porphyria"). The term ‘tarda’ refers to the typically late onset of the condition. Porphyrins are photosensitising when transported to the skin and cause skin damage on exposure to light. The condition can be familial (hereditary) or acquired.
Confirming the diagnosis
This diagnosis is based on the clinical presentation, laboratory screening for total porphyrin levels in either plasma or urine, and then specific testing, including porphyrin fractionation, if the screening test is positive.
The relevant medical specialist is a dermatologist.
Additional diagnoses covered by SOP
- chemical, symptomatic, or toxic porphyria
Conditions excluded from SOP
- Erythropoietic protoporphyria#
- Hepatoerythropoietic porphyria#
- Hereditary coproporphyria#
- X-linked protoporphyria#
- other porphyrias#
# non-SOP condition
Clinical onset
Porphyria cutanea tarda (PCT) is generally a disease of adults. It usually presents in mid or later life. The usual major manifesation is chronic blistering of the skin. Other manifestations include skin fragility, scarring, and hyper- and hypopigmentation affecting sun-exposed areas. Liver involvement/damage is also common.
Clinical worsening
PCT is a readily treatable condition with a good prognosis. Relapses may occur following successful treatment. Phlebotomy (drawing blood) and hydroxychloroquine therapy and the main forms of treatment.
Source URL: https://clik.dva.gov.au/ccps-medical-research-library/statements-principles/n-p/porphyria-cutanea-tarda-m003-e801/rulebase-porphyria-cutanea-tarda/hepatitis
Inability to obtain appropriate clinical management for porphyria cutanea tarda
Current RMA Instruments
| Reasonable Hypothesis SOP | 69 of 2021 |
| Balance of Probabilities SOP | 70 of 2021 |
Changes from previous Instruments
ICD Coding
- ICD-9-CM Codes: 277.1
- ICD-10-AM Codes: E80.1
Brief description
This is predominantly a skin ("cutanea") disease, caused by a deficiency of a liver enzyme involved in the metabolism of porphyrin. This leads to an excess of porphyrins in the blood ("porphyria"). The term ‘tarda’ refers to the typically late onset of the condition. Porphyrins are photosensitising when transported to the skin and cause skin damage on exposure to light. The condition can be familial (hereditary) or acquired.
Confirming the diagnosis
This diagnosis is based on the clinical presentation, laboratory screening for total porphyrin levels in either plasma or urine, and then specific testing, including porphyrin fractionation, if the screening test is positive.
The relevant medical specialist is a dermatologist.
Additional diagnoses covered by SOP
- chemical, symptomatic, or toxic porphyria
Conditions excluded from SOP
- Erythropoietic protoporphyria#
- Hepatoerythropoietic porphyria#
- Hereditary coproporphyria#
- X-linked protoporphyria#
- other porphyrias#
# non-SOP condition
Clinical onset
Porphyria cutanea tarda (PCT) is generally a disease of adults. It usually presents in mid or later life. The usual major manifesation is chronic blistering of the skin. Other manifestations include skin fragility, scarring, and hyper- and hypopigmentation affecting sun-exposed areas. Liver involvement/damage is also common.
Clinical worsening
PCT is a readily treatable condition with a good prognosis. Relapses may occur following successful treatment. Phlebotomy (drawing blood) and hydroxychloroquine therapy and the main forms of treatment.
Source URL: https://clik.dva.gov.au/ccps-medical-research-library/statements-principles/n-p/porphyria-cutanea-tarda-m003-e801/rulebase-porphyria-cutanea-tarda/inability-obtain-appropriate-clinical-management-porphyria-cutanea-tarda
Infection with the human immunodeficiency virus (HIV)
Current RMA Instruments
| Reasonable Hypothesis SOP | 69 of 2021 |
| Balance of Probabilities SOP | 70 of 2021 |
Changes from previous Instruments
ICD Coding
- ICD-9-CM Codes: 277.1
- ICD-10-AM Codes: E80.1
Brief description
This is predominantly a skin ("cutanea") disease, caused by a deficiency of a liver enzyme involved in the metabolism of porphyrin. This leads to an excess of porphyrins in the blood ("porphyria"). The term ‘tarda’ refers to the typically late onset of the condition. Porphyrins are photosensitising when transported to the skin and cause skin damage on exposure to light. The condition can be familial (hereditary) or acquired.
Confirming the diagnosis
This diagnosis is based on the clinical presentation, laboratory screening for total porphyrin levels in either plasma or urine, and then specific testing, including porphyrin fractionation, if the screening test is positive.
The relevant medical specialist is a dermatologist.
Additional diagnoses covered by SOP
- chemical, symptomatic, or toxic porphyria
Conditions excluded from SOP
- Erythropoietic protoporphyria#
- Hepatoerythropoietic porphyria#
- Hereditary coproporphyria#
- X-linked protoporphyria#
- other porphyrias#
# non-SOP condition
Clinical onset
Porphyria cutanea tarda (PCT) is generally a disease of adults. It usually presents in mid or later life. The usual major manifesation is chronic blistering of the skin. Other manifestations include skin fragility, scarring, and hyper- and hypopigmentation affecting sun-exposed areas. Liver involvement/damage is also common.
Clinical worsening
PCT is a readily treatable condition with a good prognosis. Relapses may occur following successful treatment. Phlebotomy (drawing blood) and hydroxychloroquine therapy and the main forms of treatment.
Source URL: https://clik.dva.gov.au/ccps-medical-research-library/statements-principles/n-p/porphyria-cutanea-tarda-m003-e801/rulebase-porphyria-cutanea-tarda/infection-human-immunodeficiency-virus-hiv
Oral oestrogen therapy
Current RMA Instruments
| Reasonable Hypothesis SOP | 69 of 2021 |
| Balance of Probabilities SOP | 70 of 2021 |
Changes from previous Instruments
ICD Coding
- ICD-9-CM Codes: 277.1
- ICD-10-AM Codes: E80.1
Brief description
This is predominantly a skin ("cutanea") disease, caused by a deficiency of a liver enzyme involved in the metabolism of porphyrin. This leads to an excess of porphyrins in the blood ("porphyria"). The term ‘tarda’ refers to the typically late onset of the condition. Porphyrins are photosensitising when transported to the skin and cause skin damage on exposure to light. The condition can be familial (hereditary) or acquired.
Confirming the diagnosis
This diagnosis is based on the clinical presentation, laboratory screening for total porphyrin levels in either plasma or urine, and then specific testing, including porphyrin fractionation, if the screening test is positive.
The relevant medical specialist is a dermatologist.
Additional diagnoses covered by SOP
- chemical, symptomatic, or toxic porphyria
Conditions excluded from SOP
- Erythropoietic protoporphyria#
- Hepatoerythropoietic porphyria#
- Hereditary coproporphyria#
- X-linked protoporphyria#
- other porphyrias#
# non-SOP condition
Clinical onset
Porphyria cutanea tarda (PCT) is generally a disease of adults. It usually presents in mid or later life. The usual major manifesation is chronic blistering of the skin. Other manifestations include skin fragility, scarring, and hyper- and hypopigmentation affecting sun-exposed areas. Liver involvement/damage is also common.
Clinical worsening
PCT is a readily treatable condition with a good prognosis. Relapses may occur following successful treatment. Phlebotomy (drawing blood) and hydroxychloroquine therapy and the main forms of treatment.
Source URL: https://clik.dva.gov.au/ccps-medical-research-library/statements-principles/n-p/porphyria-cutanea-tarda-m003-e801/rulebase-porphyria-cutanea-tarda/oral-oestrogen-therapy
Porphyrin-generating hepatocellular tumour
Current RMA Instruments
| Reasonable Hypothesis SOP | 69 of 2021 |
| Balance of Probabilities SOP | 70 of 2021 |
Changes from previous Instruments
ICD Coding
- ICD-9-CM Codes: 277.1
- ICD-10-AM Codes: E80.1
Brief description
This is predominantly a skin ("cutanea") disease, caused by a deficiency of a liver enzyme involved in the metabolism of porphyrin. This leads to an excess of porphyrins in the blood ("porphyria"). The term ‘tarda’ refers to the typically late onset of the condition. Porphyrins are photosensitising when transported to the skin and cause skin damage on exposure to light. The condition can be familial (hereditary) or acquired.
Confirming the diagnosis
This diagnosis is based on the clinical presentation, laboratory screening for total porphyrin levels in either plasma or urine, and then specific testing, including porphyrin fractionation, if the screening test is positive.
The relevant medical specialist is a dermatologist.
Additional diagnoses covered by SOP
- chemical, symptomatic, or toxic porphyria
Conditions excluded from SOP
- Erythropoietic protoporphyria#
- Hepatoerythropoietic porphyria#
- Hereditary coproporphyria#
- X-linked protoporphyria#
- other porphyrias#
# non-SOP condition
Clinical onset
Porphyria cutanea tarda (PCT) is generally a disease of adults. It usually presents in mid or later life. The usual major manifesation is chronic blistering of the skin. Other manifestations include skin fragility, scarring, and hyper- and hypopigmentation affecting sun-exposed areas. Liver involvement/damage is also common.
Clinical worsening
PCT is a readily treatable condition with a good prognosis. Relapses may occur following successful treatment. Phlebotomy (drawing blood) and hydroxychloroquine therapy and the main forms of treatment.
Source URL: https://clik.dva.gov.au/ccps-medical-research-library/statements-principles/n-p/porphyria-cutanea-tarda-m003-e801/rulebase-porphyria-cutanea-tarda/porphyrin-generating-hepatocellular-tumour
Sun exposure within 5 days before clinical worsening of porphyria cutanea tarda
Current RMA Instruments
| Reasonable Hypothesis SOP | 69 of 2021 |
| Balance of Probabilities SOP | 70 of 2021 |
Changes from previous Instruments
ICD Coding
- ICD-9-CM Codes: 277.1
- ICD-10-AM Codes: E80.1
Brief description
This is predominantly a skin ("cutanea") disease, caused by a deficiency of a liver enzyme involved in the metabolism of porphyrin. This leads to an excess of porphyrins in the blood ("porphyria"). The term ‘tarda’ refers to the typically late onset of the condition. Porphyrins are photosensitising when transported to the skin and cause skin damage on exposure to light. The condition can be familial (hereditary) or acquired.
Confirming the diagnosis
This diagnosis is based on the clinical presentation, laboratory screening for total porphyrin levels in either plasma or urine, and then specific testing, including porphyrin fractionation, if the screening test is positive.
The relevant medical specialist is a dermatologist.
Additional diagnoses covered by SOP
- chemical, symptomatic, or toxic porphyria
Conditions excluded from SOP
- Erythropoietic protoporphyria#
- Hepatoerythropoietic porphyria#
- Hereditary coproporphyria#
- X-linked protoporphyria#
- other porphyrias#
# non-SOP condition
Clinical onset
Porphyria cutanea tarda (PCT) is generally a disease of adults. It usually presents in mid or later life. The usual major manifesation is chronic blistering of the skin. Other manifestations include skin fragility, scarring, and hyper- and hypopigmentation affecting sun-exposed areas. Liver involvement/damage is also common.
Clinical worsening
PCT is a readily treatable condition with a good prognosis. Relapses may occur following successful treatment. Phlebotomy (drawing blood) and hydroxychloroquine therapy and the main forms of treatment.
Source URL: https://clik.dva.gov.au/ccps-medical-research-library/statements-principles/n-p/porphyria-cutanea-tarda-m003-e801/rulebase-porphyria-cutanea-tarda/sun-exposure-within-5-days-clinical-worsening-porphyria-cutanea-tarda
Treatment with a drug or a class of drugs from list 1
Current RMA Instruments
| Reasonable Hypothesis SOP | 69 of 2021 |
| Balance of Probabilities SOP | 70 of 2021 |
Changes from previous Instruments
ICD Coding
- ICD-9-CM Codes: 277.1
- ICD-10-AM Codes: E80.1
Brief description
This is predominantly a skin ("cutanea") disease, caused by a deficiency of a liver enzyme involved in the metabolism of porphyrin. This leads to an excess of porphyrins in the blood ("porphyria"). The term ‘tarda’ refers to the typically late onset of the condition. Porphyrins are photosensitising when transported to the skin and cause skin damage on exposure to light. The condition can be familial (hereditary) or acquired.
Confirming the diagnosis
This diagnosis is based on the clinical presentation, laboratory screening for total porphyrin levels in either plasma or urine, and then specific testing, including porphyrin fractionation, if the screening test is positive.
The relevant medical specialist is a dermatologist.
Additional diagnoses covered by SOP
- chemical, symptomatic, or toxic porphyria
Conditions excluded from SOP
- Erythropoietic protoporphyria#
- Hepatoerythropoietic porphyria#
- Hereditary coproporphyria#
- X-linked protoporphyria#
- other porphyrias#
# non-SOP condition
Clinical onset
Porphyria cutanea tarda (PCT) is generally a disease of adults. It usually presents in mid or later life. The usual major manifesation is chronic blistering of the skin. Other manifestations include skin fragility, scarring, and hyper- and hypopigmentation affecting sun-exposed areas. Liver involvement/damage is also common.
Clinical worsening
PCT is a readily treatable condition with a good prognosis. Relapses may occur following successful treatment. Phlebotomy (drawing blood) and hydroxychloroquine therapy and the main forms of treatment.
Source URL: https://clik.dva.gov.au/ccps-medical-research-library/statements-principles/n-p/porphyria-cutanea-tarda-m003-e801/rulebase-porphyria-cutanea-tarda/treatment-drug-or-class-drugs-list-1
Treatment with a drug or a class of drugs from list 2
Current RMA Instruments
| Reasonable Hypothesis SOP | 69 of 2021 |
| Balance of Probabilities SOP | 70 of 2021 |
Changes from previous Instruments
ICD Coding
- ICD-9-CM Codes: 277.1
- ICD-10-AM Codes: E80.1
Brief description
This is predominantly a skin ("cutanea") disease, caused by a deficiency of a liver enzyme involved in the metabolism of porphyrin. This leads to an excess of porphyrins in the blood ("porphyria"). The term ‘tarda’ refers to the typically late onset of the condition. Porphyrins are photosensitising when transported to the skin and cause skin damage on exposure to light. The condition can be familial (hereditary) or acquired.
Confirming the diagnosis
This diagnosis is based on the clinical presentation, laboratory screening for total porphyrin levels in either plasma or urine, and then specific testing, including porphyrin fractionation, if the screening test is positive.
The relevant medical specialist is a dermatologist.
Additional diagnoses covered by SOP
- chemical, symptomatic, or toxic porphyria
Conditions excluded from SOP
- Erythropoietic protoporphyria#
- Hepatoerythropoietic porphyria#
- Hereditary coproporphyria#
- X-linked protoporphyria#
- other porphyrias#
# non-SOP condition
Clinical onset
Porphyria cutanea tarda (PCT) is generally a disease of adults. It usually presents in mid or later life. The usual major manifesation is chronic blistering of the skin. Other manifestations include skin fragility, scarring, and hyper- and hypopigmentation affecting sun-exposed areas. Liver involvement/damage is also common.
Clinical worsening
PCT is a readily treatable condition with a good prognosis. Relapses may occur following successful treatment. Phlebotomy (drawing blood) and hydroxychloroquine therapy and the main forms of treatment.
Source URL: https://clik.dva.gov.au/ccps-medical-research-library/statements-principles/n-p/porphyria-cutanea-tarda-m003-e801/rulebase-porphyria-cutanea-tarda/treatment-drug-or-class-drugs-list-2