Cutaneous Porphyria Follow-up

Updated: Nov 12, 2019
  • Author: Richard E Frye, MD, PhD; Chief Editor: Hassan M Yaish, MD  more...
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Follow-up

Further Outpatient Care

Patients on antimalarial medications are at risk for ophthalmological and central visual dysfunction, peripheral neuropathy, deafness, and blood dyscrasias. Periodic ophthalmologic examinations, including visual acuity and slitlamp, funduscopic, and visual field tests, should be performed. Periodically evaluate reflexes and muscular strength. Hearing should be periodically objectively tested. Periodically check CBC count.

Cholestyramine can cause vitamin K deficiency. All patients on long-term therapy should be examined for bleeding or bruising and should receive supplemental vitamin K.

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Deterrence/Prevention

Many medications can induce or worsen porphyria, whereas others have not been associated with worsening porphyria. Furthermore, many medications have not been tested in patients with known porphyria. The list below is not exhaustive, and any medication used in a patient with porphyria should be researched.

An extensive list of safe drugs is available at the University of Queensland Porphyria Research Unit Web site. Medications likely to be safe for patients with porphyria include the following:

  • Acetaminophen

  • Adrenaline

  • Amitriptyline

  • Aspirin

  • Atropine

  • Bromides

  • Chloral hydrate

  • Chlordiazepoxide

  • Colchicine

  • Diazepam

  • Digoxin

  • Diphenhydramine

  • Ethylenediaminetetraacetic acid (EDTA)

  • Ether

  • Glucocorticoids

  • Guanethidine

  • Ibuprofen

  • Imipramine

  • Indomethacin

  • Insulin

  • Labetalol

  • Lithium

  • Methylphenidate

  • Naproxen

  • Narcotics

  • Neostigmine

  • Nitrous oxide

  • Penicillamine

  • Penicillin

  • Phenothiazines

  • Procaine

  • Propranolol

  • Succinylcholine

  • Tetracycline

  • Thyroxine

  • Tubocurarine

Many medications induce or worsen acute and cutaneous porphyria. Many of these medications are metabolized, at least to some extent, by the liver. Liver metabolism may induce the cytochrome P-450 enzymes that require heme, thus inducing heme production. Other medications sensitize the skin to solar damage. Only common medications are listed below, and any medication used in a patient known to have porphyria should be investigated. In addition, many medications have not been used for patients with porphyria, thus the potential for worsening porphyria is not known. The list below is a guide for determining if a medication could have triggered a porphyria reaction.

Patients should refrain from alcohol ingestion, estrogen use, and iron supplementation.

Medications that are potentially unsafe for use in patients with porphyria include the following:

  • Alfaxalone

  • Alkylating agents

  • Antipyrine

  • Arthrotec

  • Barbiturates

  • Busulfan

  • Butalbital

  • Carbamazepine

  • Carisoprodol

  • Chlordiazepoxide

  • Chloroquine

  • Clonidine

  • Danazol

  • Danocrine

  • Dapsone

  • Diclofenac

  • Ergot

  • Erythromycin

  • Erythropoietin

  • Estrogens

  • Ethchlorvynol

  • Fluroxene

  • Griseofulvin

  • Heavy metals

  • Hydralazine

  • Ketamine

  • Mafenide

  • Meprobamate

  • Methoxsalen

  • Methyldopa

  • Metoclopramide

  • Nitrazepam

  • Nortriptyline

  • Pargyline

  • Pentazocine

  • Phenazopyridine

  • Phenobarbital

  • Phenoxybenzamine

  • Phenylbutazone

  • Phenytoin

  • Plaquenil

  • Porfimer

  • Primidone

  • Progestins

  • Pyrazinamide

  • Ranitidine

  • Rifampin

  • Spironolactone

  • Succinimides

  • Sulfonamides

  • Sulfonylureas

  • Theophylline

  • Tolazamide

  • Tranylcypromine

  • Valproate

  • Voriconazole

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Complications

Congenital erythropoietic porphyria (CEP) is associated with splenomegaly, hypersplenism, hemolytic anemia, and cholelithiasis.

Porphyria cutanea tarda (PCT) is associated with an increased incidence of hepatocellular carcinoma and cirrhosis.

Erythropoietic protoporphyria (EPP) is associated with cholelithiasis in a significant number of cases. Severe liver disease may develop as a result of periportal fibrosis and cirrhosis, leading to death in 20% of cases. Rapidly progressive liver failure associated with accelerating photosensitivity and cholestasis can occur and is accompanied by abdominal pain, splenomegaly, and hemolysis.

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Prognosis

CEP is associated with a significantly decreased lifespan, whereas the other cutaneous porphyrias are associated with morbidity from the complications of skin lesions.

A literature review by Salameh et al indicated that in patients with PCT, relapse rates by 1-year follow-up are lower for those treated with phlebotomy (20%) than for patients who undergo high-dose or low-dose 4-aminoquinoline therapy (35-36%).  The pooled relapse rate for phlebotomy was 5.1 per 100 person-years, compared with 8.6 and 17.1 per 100 person-years for high-dose and low-dose 4-aminoquinoline treatment, respectively. [53]

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