Hereditary Coproporphyria Clinical Presentation

Author: Thomas G DeLoughery, MD; Chief Editor: Emmanuel C Besa, MD more...

Updated: Jan 10, 2012

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History
Physical
Causes
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References

History

Coproporphyria displays both neurovisceral and skin manifestations.

Neurovisceral symptoms
- These symptoms consist of autonomic neuropathies (constipation, colicky abdominal pain, vomiting, hypertension), peripheral neuropathy, seizures, delirium, coma, and depression.
- The sequence of events in attacks usually is abdominal pain then psychiatric symptoms (eg, hysteria), then peripheral neuropathies.
- Patients can have very severe abdominal pain that lasts for several days. Pain of short duration (minutes) or chronic abdominal pain does not develop in coproporphyria. The pain often is epigastric and is colicky in nature.
- The exact mechanism by which the porphyrin precursors lead to these symptoms is unknown.
- Patients often are free of pain between attacks. Constipation is common and can be very severe. Nausea and vomiting frequently are present.
Skin manifestations
- The skin disease is similar to porphyria cutanea tarda. With long-term (not acute) sun exposure, patients can develop vesicals and bullae.
- If patients are symptomatic with coproporphyria, they tend to have neurovisceral symptoms rather than skin symptoms.
Blisters form in sun-exposed areas and can evolve into chronic scarred areas of fragile skin.
Patients also may develop excessive hair growth in sun-exposed areas.
Neurologic manifestations
- Patients with coproporphyria can have central nervous system signs, including seizures, mental status changes, cortical blindness, and coma.
- Patients often experience peripheral neuropathies that predominantly are motor neuropathies and can mimic Guillain-Barré syndrome. The weakness usually starts in the lower limbs and ascends, but neuropathies occur in any nerve distribution.
- Diffuse pain, especially in the upper body, can be observed. Patients also can develop autonomic neuropathies, including hypertension and tachycardia.
Psychiatric manifestations
- Patients can have a wide variety of psychiatric symptoms.
- Patients usually have concurrent neurologic or abdominal symptoms.

Vital signs
- Tachycardia develops in 30-80% of patients.
- Fever can be present in some patients.
- Hypertension develops in 50% of cases and may persist between attacks.
Neurologic manifestations
- Typically, the neuropathy is a motor neuropathy that is more predominant in the lower limbs.
- Areflexia is observed during the examination; however, any nerve can be involved. Cranial neuropathies also are observed, and the patient may have cortical blindness.
Abdominal manifestations: Despite the intense pain, the findings on abdominal examination often are nonspecific.
Skin manifestations
- Patients can have blisters, chronic erosion, and areas of excessive hair growth.
- Skin damage develops in sun-exposed areas of the skin.

Causes

Like AIP, coproporphyria is due to a combination of a genetic enzyme defect and acquired causes that become symptomatic in rare cases.^[4]In patients with coproporphyria, the function of coproporphyrinogen oxidase is only 40-60% of normal.^[5]Also, like AIP, most patients with defects in coproporphyrinogen oxidase never have any symptoms. The classic inducers of porphyria are chemicals or situations that boost heme synthesis. This includes fasting and many medications.

Although extensive lists of safe and unsafe drugs exist, many of these are based on anecdotes or laboratory evidence rather than meeting strict criteria. In general, drugs that lead to increased activity of the hepatic P450 system (eg, phenobarbital, sulfonamides, estrogens, alcohol) are associated with porphyria. A large and detailed list, shown below, is available at Porphyria: A Patient's Guide. Fasting for several days also can trigger an attack. Many attacks will occur, however, without any obvious provocation.

Table 1. Drugs Thought Safe in Porphyria* (Open Table in a new window)

Acetazolamide acetylcholine Actinomycin D Acyclovir Adenosine monophosphate Adrenaline Alclofenac Allopurinol Alpha tocopheryl Acetate Amethocaine Amiloride Aminocaproic acid Aminoglycosides Amoxicillin Amphotericin Ampicillin Ascorbic acid Aspirin Atenolol Atropine Azathioprine Beclomethasone Benzhexol HCl Beta-carotene Biguanides [Bromazepam] Bromides Buflomedil HCl Bumetanide Bupivacaine Buprenorphine Buserelin Butacaine SO₄ Canthaxanthin Carbimazole [Carpipramine HCl] Chloral hydrate [Chlormethiazole] [Chloroquine] [Chlorothiazide] Chlorpheniramine Chlorpromazine Ciprofloxacin Cisapride Cisplatin Clavulanic acid Clofibrate Clomiphene Cloxacillin Co-codamol Codeine phosphate Colchicine [Corticosteroids] Corticotrophin (adrenocorticotropic hormone [ACTH])	Coumarins Cyclizine Cyclopenthiazide Cyclopropane [Cyproterone acetate] Danthron Desferrioxamine Dexamethasone [Dextromoramide] Dextrose Diamorphine Diazoxide Dicyclomine HCl Diflunisal Digoxin Dihydrocodeine Dimercaprol Dimethicone Dinoprost Diphenoxylate HCl Dipyridamole [Disopyramide] Domperidone Doxorubicin HCl Droperidol [Estazolam] Ethacrynic acid Ethambutol [Ethinyl oestradiol] Ethoheptazine citrate Etoposide Famotidine Fenbufen [Fenofibrate] Fenoprofen Fentanyl Flucytosine Flumazenil Fluoxetine HCl Flurbiprofen Fluvoxamine Maleate Folic acid Fructose Fusidic acid Follicle-stimulating hormone Gentamicin Glafenine Glucagon Glucose Glyceryl trinitrate Goserelin Guanethidine Guanfacine HCl Haem arginate [Haloperidol] Heparin Heptaminol HCl Hexamine [Hydrocortisone] Ibuprofen Indomethacin Insulin Iron Josamycin [Ketamine]	Ketoprofen Ketotifen Labetalol Luteinizing hormone–releasing hormone Liquorice Lithium Salts lofepramine Loperamide [Lorazepam] Magnesium-sulphate [Mebendazole] Mecamylamine Meclofenoxate HCl Meclozine Mefloquine HCl [Melphalan] Meptazinol Mequitazine Metformin Methadone [Methotrimeprazine] Methylphenidate Methyluracil Metipropranolol Metopimazine Metoprolol [Metronidazole] [Midazolam] Minaprine HCl Minaxolone Morphine Nadolol Naftidrofuryl Oxalate [Naproxen sodium] Natamycin Nefopam HCl Neostigmine Netilmicin Niflumic acid Nitrous oxide Norfloxacin Ofloxacin Oxolinic acid Oxybuprocaine [Oxyphenbutazone] Oxytocin [Pancuronium bromide] Paracetamol Paraldehyde Parapenzolate Br Penicillamine Penicillin Pentolinium Pericyazine Pethidine Phenformin Phenoperidine Phentolamine mesylate Pipotiazine	Palmitate Piracetam Pirbuterol Pirenzepine Pizotifen [Prazosin] [Prednisolone] Primaquine Probucol Procainamide HCl Procaine Prochlorperazine Proguanil HCl Promazine Propantheline Br Propofol Propranolol Propylthiouracil [Proxymetacaine] Pseudoephedrine HCl Pyridoxine [Pyrimethamine] Quinidine Quinine [Ranitidine] Reserpine Resorcinol Salbutamol Senna Sodium bromide Sodium ethylenediaminetetraacetic acid Sodium fusidate Sorbitol Streptomycin Sulbutiamine Sulindac Sulfadoxine Suxamethonium Talampicillin Temazepam Tetracaine [Tetracyclines] Thiouracils Thyroxine Tiaprofenic acid Ticarcillin Tienilic acid Timolol maleate Tolazoline Tranexamic acid Triacetyloleandomycin Triamterene Triazolam [Trichlormethiazide] Trifluoperazine Trimeprazine Tartrate Trimetazidine HCl Tripelennamine Tubocurarine Vancomycin [Vincristine] Vitamins Warfarin sodium Zidovudine Zinc Preparations
*Bracketed [] drugs are those in which experimental evidence of porphyrin genicity is conflicting.

Table 2. Drugs Thought Unsafe in Porphyria^† (Open Table in a new window)

Alcuronium Alphaxalone Alphadolone Alprazolam Aluminium Preparations Amidopyrine Aminoglutethimide Aminophylline Amiodarone Amitriptyline [Amphetamines] Amylobarbitone Antipyrine Auranofin Aurothiomalate Azapropazone Baclofen Barbiturates Bemegride Bendrofluazide Benoxaprofen Benzbromarone [Benzylthiouracil] [Bepridil] Bromocriptine Busulphan Butylscopolamine Captopril Carbamazepine Carbromal Carisoprodol [Cefuroxime] [Cephalexin] [Cephalosporins] [Cephradine] [Chlorambucil] Chloramphenicol Chlordiazepoxide Chlormezanone Chloroform Chlorpropamide Cinnarizine Clemastine [Clobazam] [Clomipramine HCl] [Clonazepam] Clonidine HCl Clorazepate Cocaine [Colistin] Co-trimoxazole	Cyclophosphamide Cycloserine Cyclosporin Danazol Dapsone Dexfenfluramine Dextropropoxyphene Diazepam Dichloralphenazone Diclofenac Na Dienoestrol Diethylpropion Dihydralazine Dihydroergotamine Diltiazem Dimenhydrinate Diphenhydramine [Dothiepin HCl] Doxycycline Dydrogesterone Econazole NO₃ Enalapril Enflurane Ergot compounds Ergometrine maleate Ergotamine tartrate Erythromycin Estramustine Ethamsylate Ethanol Ethionamide Ethosuximide Ethotoin Etidocaine Etomidate Fenfluramine Flucloxacillin Flufenamic acid Flunitrazepam Flupenthixol Flurazepam Frusemide Glibenclamide Glutethimide Glipizide Gramicidin Griseofulvin [Haloperidol] Halothane Hydantoins Hydralazine Hydrochlorothiazide Hydroxyzine Hyoscine Imipramine Iproniazid Isometheptene mucate [Isoniazid] Kebuzone Ketoconazole Levonorgestrel Lignocaine Lisinopril Loprazolam Loxapine *Lynestrenol Lysuride	Maleate Maprotiline HCl Mebeverine HCl Mecillinam Medroxyprogesterone [Mefenamic acid] Megestrol acetate Mephenytoin Mepivacaine Meprobamate Mercaptopurine Mercury compounds Mestranol [Metapramine HCl] Methamphetamine Methohexitone Methotrexate Methoxyflurane Methsuximide Methyldopa Methylsulphonal Methyprylone Methysergide Metoclopramide Metyrapone Mianserin HCl Miconazole [Mifepristone] Minoxidil Nandrolone Nalidixic acid Natamycin Nandrolone [Nicergoline] Nifedipine Nikethamide Nitrazepam Nitrofurantoin Nordazepam Norethynodrel Norethisterone [Nortriptyline] Novobiocin Oral contraceptives Orphenadrine Oxanamide [Oxazepam] Oxybutynin HCl Oxycodone Oxymetazoline Oxyphenbutazone Oxytetracycline Paramethadione Pargyline Pentazocine Perhexiline Phenacetin Phenelzine Phenobarbitone Phenoxybenzamine Phensuximide Phenylbutazone Phenylhydrazine Phenytoin Pipebuzone Pipemidic Acid Piritramide *Piroxicam	Pivampicillin Pivmecillinam Prazepam Prenylamine Prilocaine Primidone [Probenecid] Progesterone Progabide Promethazine [Propanidid] Pyrazinamide Pyrrocaine Quinalbarbitone Rifampicin Simvastatin Sodium aurothiomalate Sodium oxybate [Sodium valproate] Spironolactone Stanozolol Succinimides Sulfacetamide Sulfadiazine Sulfadimidine Sulfadoxine Sulfamethoxazole Sulfasalazine Sulfonylureas Sulfinpyrazone Sulpiride Sulthiame Sultopride Tamoxifen Terfenadine Tetrazepam Theophylline Thiopentone Na Thioridazine Tilidate Tinidazole Tolazamide Tolbutamide Tranylcypromine Trazodone HCl Trimethoprim [Trimipramine] Troxidone Valproate Valpromide Veralipride Verapamil Vibramycin Viloxazine HCl [Vinblastine] [Vincristine] Zuclopenthixol
*These drugs have been associated with acute attacks of porphyria. †Bracketed [] drugs are those in which experimental evidence of porphyringenicity is conflicting.

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Contributor Information and Disclosures

Author

Thomas G DeLoughery, MD Professor of Medicine, Pathology, and Pediatrics, Divisions of Hematology/Oncology and Laboratory Medicine, Associate Director, Department of Transfusion Medicine, Division of Clinical Pathology, Oregon Health and Science University School of Medicine

Thomas G DeLoughery, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Blood Banks, American College of Physicians, American Society of Hematology, International Society on Thrombosis and Haemostasis, and Wilderness Medical Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Clarence Sarkodee-Adoo, MD Consulting Staff, Department of Bone Marrow Transplantation, City of Hope Samaritan BMT Program

Disclosure: Takeda Millenium Honoraria Speaking and teaching

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Marcel E Conrad, MD Distinguished Professor of Medicine (Retired), University of South Alabama College of Medicine

Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, and Southwest Oncology Group

Disclosure: No financial interests None None

Rajalaxmi McKenna, MD, FACP Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Clinical Oncology, American Society of Hematology, and New York Academy of Sciences

Disclosure: Nothing to disclose.

References

Whatley SD, Mason NG, Woolf JR, et al. Diagnostic strategies for autosomal dominant acute porphyrias: retrospective analysis of 467 unrelated patients referred for mutational analysis of the HMBS, CPOX, or PPOX gene. Clin Chem. Jul 2009;55(7):1406-14. [Medline].
Barbaro M, Kotajärvi M, Harper P, Floderus Y. Identification of an AluY-mediated deletion of exon 5 in the CPOX gene by MLPA analysis in patients with hereditary coproporphyria. Clin Genet. Jan 13 2011;[Medline].
Ventura P, Cappellini MD, Rocchi E. The acute porphyrias: a diagnostic and therapeutic challenge in internal and emergency medicine. Intern Emerg Med. Aug 2009;4(4):297-308. [Medline].
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Corrigall AV, Campbell JA, Siziba K, Kirsch RE, Meissner PN. The expression of protoporphyrinogen oxidase in human tissues. Cell Mol Biol (Noisy-le-grand). Jul 1 2009;55(2):89-95. [Medline].
Roshal M, Turgeon J, Rainey PM. Rapid quantitative method using spin columns to measure porphobilinogen in urine. Clin Chem. Feb 2008;54(2):429-31. [Medline].
Harper P, Wahlin S. Treatment options in acute porphyria, porphyria cutanea tarda, and erythropoietic protoporphyria. Curr Treat Options Gastroenterol. Dec 2007;10(6):444-55. [Medline].
Ma E, Mar V, Varigos G, Nicoll A, Ross G. Haem arginate as effective maintenance therapy for hereditary coproporphyria. Australas J Dermatol. May 2011;52(2):135-8. [Medline].
Anderson KE. The Porphyrias. In: Zakim D, Boyer TD, eds. Hepatology: A Textbook of Liver Disease. Philadelphia, Pa:. WB Saunders;1996:417-463.
Anderson KE, Bloomer JR, Bonkovsky HL, et al. Recommendations for the diagnosis and treatment of the acute porphyrias. Ann Intern Med. Mar 15 2005;142(6):439-50. [Medline].
Bickers DR, Pathak MA, Lim HW. The Porphyrias. In: Fitzpatrick B, et al, eds. Dermatology in General Medicine. New York, NY:. McGraw-Hill, Inc;1993:1854-1893.
Bonkovsky HL, Barnard GF. Diagnosis of porphyric syndromes: a practical approach in the era of molecular biology. Semin Liver Dis. 1998;18(1):57-65. [Medline].
Daniell WE, Stockbridge HL, Labbe RF, et al. Environmental chemical exposures and disturbances of heme synthesis. Environ Health Perspect. Feb 1997;105 Suppl 1:37-53. [Medline].
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Hahn M, Bonkovsky HL. Multiple chemical sensitivity syndrome and porphyria. A note of caution and concern. Arch Intern Med. Feb 10 1997;157(3):281-5. [Medline].
Kalman DR, Bonkovsky HL. Management of acute attacks in the porphyrias. Clin Dermatol. Mar-Apr 1998;16(2):299-306. [Medline].
Kauppinen R, Mustajoki P. Prognosis of acute porphyria: occurrence of acute attacks, precipitating factors, and associated diseases. Medicine (Baltimore). Jan 1992;71(1):1-13. [Medline].
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Table 1. Drugs Thought Safe in Porphyria*

Acetazolamide acetylcholine Actinomycin D Acyclovir Adenosine monophosphate Adrenaline Alclofenac Allopurinol Alpha tocopheryl Acetate Amethocaine Amiloride Aminocaproic acid Aminoglycosides Amoxicillin Amphotericin Ampicillin Ascorbic acid Aspirin Atenolol Atropine Azathioprine Beclomethasone Benzhexol HCl Beta-carotene Biguanides [Bromazepam] Bromides Buflomedil HCl Bumetanide Bupivacaine Buprenorphine Buserelin Butacaine SO₄ Canthaxanthin Carbimazole [Carpipramine HCl] Chloral hydrate [Chlormethiazole] [Chloroquine] [Chlorothiazide] Chlorpheniramine Chlorpromazine Ciprofloxacin Cisapride Cisplatin Clavulanic acid Clofibrate Clomiphene Cloxacillin Co-codamol Codeine phosphate Colchicine [Corticosteroids] Corticotrophin (adrenocorticotropic hormone [ACTH])	Coumarins Cyclizine Cyclopenthiazide Cyclopropane [Cyproterone acetate] Danthron Desferrioxamine Dexamethasone [Dextromoramide] Dextrose Diamorphine Diazoxide Dicyclomine HCl Diflunisal Digoxin Dihydrocodeine Dimercaprol Dimethicone Dinoprost Diphenoxylate HCl Dipyridamole [Disopyramide] Domperidone Doxorubicin HCl Droperidol [Estazolam] Ethacrynic acid Ethambutol [Ethinyl oestradiol] Ethoheptazine citrate Etoposide Famotidine Fenbufen [Fenofibrate] Fenoprofen Fentanyl Flucytosine Flumazenil Fluoxetine HCl Flurbiprofen Fluvoxamine Maleate Folic acid Fructose Fusidic acid Follicle-stimulating hormone Gentamicin Glafenine Glucagon Glucose Glyceryl trinitrate Goserelin Guanethidine Guanfacine HCl Haem arginate [Haloperidol] Heparin Heptaminol HCl Hexamine [Hydrocortisone] Ibuprofen Indomethacin Insulin Iron Josamycin [Ketamine]	Ketoprofen Ketotifen Labetalol Luteinizing hormone–releasing hormone Liquorice Lithium Salts lofepramine Loperamide [Lorazepam] Magnesium-sulphate [Mebendazole] Mecamylamine Meclofenoxate HCl Meclozine Mefloquine HCl [Melphalan] Meptazinol Mequitazine Metformin Methadone [Methotrimeprazine] Methylphenidate Methyluracil Metipropranolol Metopimazine Metoprolol [Metronidazole] [Midazolam] Minaprine HCl Minaxolone Morphine Nadolol Naftidrofuryl Oxalate [Naproxen sodium] Natamycin Nefopam HCl Neostigmine Netilmicin Niflumic acid Nitrous oxide Norfloxacin Ofloxacin Oxolinic acid Oxybuprocaine [Oxyphenbutazone] Oxytocin [Pancuronium bromide] Paracetamol Paraldehyde Parapenzolate Br Penicillamine Penicillin Pentolinium Pericyazine Pethidine Phenformin Phenoperidine Phentolamine mesylate Pipotiazine	Palmitate Piracetam Pirbuterol Pirenzepine Pizotifen [Prazosin] [Prednisolone] Primaquine Probucol Procainamide HCl Procaine Prochlorperazine Proguanil HCl Promazine Propantheline Br Propofol Propranolol Propylthiouracil [Proxymetacaine] Pseudoephedrine HCl Pyridoxine [Pyrimethamine] Quinidine Quinine [Ranitidine] Reserpine Resorcinol Salbutamol Senna Sodium bromide Sodium ethylenediaminetetraacetic acid Sodium fusidate Sorbitol Streptomycin Sulbutiamine Sulindac Sulfadoxine Suxamethonium Talampicillin Temazepam Tetracaine [Tetracyclines] Thiouracils Thyroxine Tiaprofenic acid Ticarcillin Tienilic acid Timolol maleate Tolazoline Tranexamic acid Triacetyloleandomycin Triamterene Triazolam [Trichlormethiazide] Trifluoperazine Trimeprazine Tartrate Trimetazidine HCl Tripelennamine Tubocurarine Vancomycin [Vincristine] Vitamins Warfarin sodium Zidovudine Zinc Preparations
*Bracketed [] drugs are those in which experimental evidence of porphyrin genicity is conflicting.

Table 2. Drugs Thought Unsafe in Porphyria^†

Alcuronium Alphaxalone Alphadolone Alprazolam Aluminium Preparations Amidopyrine Aminoglutethimide Aminophylline Amiodarone Amitriptyline [Amphetamines] Amylobarbitone Antipyrine Auranofin Aurothiomalate Azapropazone Baclofen Barbiturates Bemegride Bendrofluazide Benoxaprofen Benzbromarone [Benzylthiouracil] [Bepridil] Bromocriptine Busulphan Butylscopolamine Captopril Carbamazepine Carbromal Carisoprodol [Cefuroxime] [Cephalexin] [Cephalosporins] [Cephradine] [Chlorambucil] Chloramphenicol Chlordiazepoxide Chlormezanone Chloroform Chlorpropamide Cinnarizine Clemastine [Clobazam] [Clomipramine HCl] [Clonazepam] Clonidine HCl Clorazepate Cocaine [Colistin] Co-trimoxazole	Cyclophosphamide Cycloserine Cyclosporin Danazol Dapsone Dexfenfluramine Dextropropoxyphene Diazepam Dichloralphenazone Diclofenac Na Dienoestrol Diethylpropion Dihydralazine Dihydroergotamine Diltiazem Dimenhydrinate Diphenhydramine [Dothiepin HCl] Doxycycline Dydrogesterone Econazole NO₃ Enalapril Enflurane Ergot compounds Ergometrine maleate Ergotamine tartrate Erythromycin Estramustine Ethamsylate Ethanol Ethionamide Ethosuximide Ethotoin Etidocaine Etomidate Fenfluramine Flucloxacillin Flufenamic acid Flunitrazepam Flupenthixol Flurazepam Frusemide Glibenclamide Glutethimide Glipizide Gramicidin Griseofulvin [Haloperidol] Halothane Hydantoins Hydralazine Hydrochlorothiazide Hydroxyzine Hyoscine Imipramine Iproniazid Isometheptene mucate [Isoniazid] Kebuzone Ketoconazole Levonorgestrel Lignocaine Lisinopril Loprazolam Loxapine *Lynestrenol Lysuride	Maleate Maprotiline HCl Mebeverine HCl Mecillinam Medroxyprogesterone [Mefenamic acid] Megestrol acetate Mephenytoin Mepivacaine Meprobamate Mercaptopurine Mercury compounds Mestranol [Metapramine HCl] Methamphetamine Methohexitone Methotrexate Methoxyflurane Methsuximide Methyldopa Methylsulphonal Methyprylone Methysergide Metoclopramide Metyrapone Mianserin HCl Miconazole [Mifepristone] Minoxidil Nandrolone Nalidixic acid Natamycin Nandrolone [Nicergoline] Nifedipine Nikethamide Nitrazepam Nitrofurantoin Nordazepam Norethynodrel Norethisterone [Nortriptyline] Novobiocin Oral contraceptives Orphenadrine Oxanamide [Oxazepam] Oxybutynin HCl Oxycodone Oxymetazoline Oxyphenbutazone Oxytetracycline Paramethadione Pargyline Pentazocine Perhexiline Phenacetin Phenelzine Phenobarbitone Phenoxybenzamine Phensuximide Phenylbutazone Phenylhydrazine Phenytoin Pipebuzone Pipemidic Acid Piritramide *Piroxicam	Pivampicillin Pivmecillinam Prazepam Prenylamine Prilocaine Primidone [Probenecid] Progesterone Progabide Promethazine [Propanidid] Pyrazinamide Pyrrocaine Quinalbarbitone Rifampicin Simvastatin Sodium aurothiomalate Sodium oxybate [Sodium valproate] Spironolactone Stanozolol Succinimides Sulfacetamide Sulfadiazine Sulfadimidine Sulfadoxine Sulfamethoxazole Sulfasalazine Sulfonylureas Sulfinpyrazone Sulpiride Sulthiame Sultopride Tamoxifen Terfenadine Tetrazepam Theophylline Thiopentone Na Thioridazine Tilidate Tinidazole Tolazamide Tolbutamide Tranylcypromine Trazodone HCl Trimethoprim [Trimipramine] Troxidone Valproate Valpromide Veralipride Verapamil Vibramycin Viloxazine HCl [Vinblastine] [Vincristine] Zuclopenthixol
*These drugs have been associated with acute attacks of porphyria. †Bracketed [] drugs are those in which experimental evidence of porphyringenicity is conflicting.

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Alcuronium Alphaxalone Alphadolone Alprazolam Aluminium Preparations Amidopyrine Aminoglutethimide Aminophylline Amiodarone Amitriptyline [Amphetamines] Amylobarbitone Antipyrine Auranofin Aurothiomalate Azapropazone Baclofen Barbiturates Bemegride Bendrofluazide Benoxaprofen Benzbromarone [Benzylthiouracil] [Bepridil] Bromocriptine Busulphan Butylscopolamine Captopril Carbamazepine Carbromal Carisoprodol [Cefuroxime] [Cephalexin] [Cephalosporins] [Cephradine] [Chlorambucil] Chloramphenicol Chlordiazepoxide Chlormezanone Chloroform Chlorpropamide Cinnarizine Clemastine [Clobazam] [Clomipramine HCl] [Clonazepam] Clonidine HCl Clorazepate Cocaine [Colistin] Co-trimoxazole	Cyclophosphamide Cycloserine Cyclosporin Danazol Dapsone Dexfenfluramine Dextropropoxyphene Diazepam Dichloralphenazone Diclofenac Na Dienoestrol Diethylpropion Dihydralazine Dihydroergotamine Diltiazem Dimenhydrinate Diphenhydramine [Dothiepin HCl] Doxycycline Dydrogesterone Econazole NO₃ Enalapril Enflurane Ergot compounds Ergometrine maleate Ergotamine tartrate Erythromycin Estramustine Ethamsylate Ethanol Ethionamide Ethosuximide Ethotoin Etidocaine Etomidate Fenfluramine Flucloxacillin Flufenamic acid Flunitrazepam Flupenthixol Flurazepam Frusemide Glibenclamide Glutethimide Glipizide Gramicidin Griseofulvin [Haloperidol] Halothane Hydantoins Hydralazine Hydrochlorothiazide Hydroxyzine Hyoscine Imipramine Iproniazid Isometheptene mucate [Isoniazid] Kebuzone Ketoconazole Levonorgestrel Lignocaine Lisinopril Loprazolam Loxapine *Lynestrenol Lysuride	Maleate Maprotiline HCl Mebeverine HCl Mecillinam Medroxyprogesterone [Mefenamic acid] Megestrol acetate Mephenytoin Mepivacaine Meprobamate Mercaptopurine Mercury compounds Mestranol [Metapramine HCl] Methamphetamine Methohexitone Methotrexate Methoxyflurane Methsuximide Methyldopa Methylsulphonal Methyprylone Methysergide Metoclopramide Metyrapone Mianserin HCl Miconazole [Mifepristone] Minoxidil Nandrolone Nalidixic acid Natamycin Nandrolone [Nicergoline] Nifedipine Nikethamide Nitrazepam Nitrofurantoin Nordazepam Norethynodrel Norethisterone [Nortriptyline] Novobiocin Oral contraceptives Orphenadrine Oxanamide [Oxazepam] Oxybutynin HCl Oxycodone Oxymetazoline Oxyphenbutazone Oxytetracycline Paramethadione Pargyline Pentazocine Perhexiline Phenacetin Phenelzine Phenobarbitone Phenoxybenzamine Phensuximide Phenylbutazone Phenylhydrazine Phenytoin Pipebuzone Pipemidic Acid Piritramide *Piroxicam	Pivampicillin Pivmecillinam Prazepam Prenylamine Prilocaine Primidone [Probenecid] Progesterone Progabide Promethazine [Propanidid] Pyrazinamide Pyrrocaine Quinalbarbitone Rifampicin Simvastatin Sodium aurothiomalate Sodium oxybate [Sodium valproate] Spironolactone Stanozolol Succinimides Sulfacetamide Sulfadiazine Sulfadimidine Sulfadoxine Sulfamethoxazole Sulfasalazine Sulfonylureas Sulfinpyrazone Sulpiride Sulthiame Sultopride Tamoxifen Terfenadine Tetrazepam Theophylline Thiopentone Na Thioridazine Tilidate Tinidazole Tolazamide Tolbutamide Tranylcypromine Trazodone HCl Trimethoprim [Trimipramine] Troxidone Valproate Valpromide Veralipride Verapamil Vibramycin Viloxazine HCl [Vinblastine] [Vincristine] Zuclopenthixol
*These drugs have been associated with acute attacks of porphyria. †Bracketed [] drugs are those in which experimental evidence of porphyringenicity is conflicting.

Alcuronium Alphaxalone Alphadolone Alprazolam Aluminium Preparations Amidopyrine Aminoglutethimide Aminophylline Amiodarone Amitriptyline [Amphetamines] Amylobarbitone Antipyrine Auranofin Aurothiomalate Azapropazone Baclofen Barbiturates Bemegride Bendrofluazide Benoxaprofen Benzbromarone [Benzylthiouracil] [Bepridil] Bromocriptine Busulphan Butylscopolamine Captopril Carbamazepine Carbromal Carisoprodol [Cefuroxime] [Cephalexin] [Cephalosporins] [Cephradine] [Chlorambucil] Chloramphenicol Chlordiazepoxide Chlormezanone Chloroform Chlorpropamide Cinnarizine Clemastine [Clobazam] [Clomipramine HCl] [Clonazepam] Clonidine HCl Clorazepate Cocaine [Colistin] Co-trimoxazole	Cyclophosphamide Cycloserine Cyclosporin Danazol Dapsone Dexfenfluramine Dextropropoxyphene Diazepam Dichloralphenazone Diclofenac Na Dienoestrol Diethylpropion Dihydralazine Dihydroergotamine Diltiazem Dimenhydrinate Diphenhydramine [Dothiepin HCl] Doxycycline Dydrogesterone Econazole NO₃ Enalapril Enflurane Ergot compounds Ergometrine maleate Ergotamine tartrate Erythromycin Estramustine Ethamsylate Ethanol Ethionamide Ethosuximide Ethotoin Etidocaine Etomidate Fenfluramine Flucloxacillin Flufenamic acid Flunitrazepam Flupenthixol Flurazepam Frusemide Glibenclamide Glutethimide Glipizide Gramicidin Griseofulvin [Haloperidol] Halothane Hydantoins Hydralazine Hydrochlorothiazide Hydroxyzine Hyoscine Imipramine Iproniazid Isometheptene mucate [Isoniazid] Kebuzone Ketoconazole Levonorgestrel Lignocaine Lisinopril Loprazolam Loxapine *Lynestrenol Lysuride	Maleate Maprotiline HCl Mebeverine HCl Mecillinam Medroxyprogesterone [Mefenamic acid] Megestrol acetate Mephenytoin Mepivacaine Meprobamate Mercaptopurine Mercury compounds Mestranol [Metapramine HCl] Methamphetamine Methohexitone Methotrexate Methoxyflurane Methsuximide Methyldopa Methylsulphonal Methyprylone Methysergide Metoclopramide Metyrapone Mianserin HCl Miconazole [Mifepristone] Minoxidil Nandrolone Nalidixic acid Natamycin Nandrolone [Nicergoline] Nifedipine Nikethamide Nitrazepam Nitrofurantoin Nordazepam Norethynodrel Norethisterone [Nortriptyline] Novobiocin Oral contraceptives Orphenadrine Oxanamide [Oxazepam] Oxybutynin HCl Oxycodone Oxymetazoline Oxyphenbutazone Oxytetracycline Paramethadione Pargyline Pentazocine Perhexiline Phenacetin Phenelzine Phenobarbitone Phenoxybenzamine Phensuximide Phenylbutazone Phenylhydrazine Phenytoin Pipebuzone Pipemidic Acid Piritramide *Piroxicam	Pivampicillin Pivmecillinam Prazepam Prenylamine Prilocaine Primidone [Probenecid] Progesterone Progabide Promethazine [Propanidid] Pyrazinamide Pyrrocaine Quinalbarbitone Rifampicin Simvastatin Sodium aurothiomalate Sodium oxybate [Sodium valproate] Spironolactone Stanozolol Succinimides Sulfacetamide Sulfadiazine Sulfadimidine Sulfadoxine Sulfamethoxazole Sulfasalazine Sulfonylureas Sulfinpyrazone Sulpiride Sulthiame Sultopride Tamoxifen Terfenadine Tetrazepam Theophylline Thiopentone Na Thioridazine Tilidate Tinidazole Tolazamide Tolbutamide Tranylcypromine Trazodone HCl Trimethoprim [Trimipramine] Troxidone Valproate Valpromide Veralipride Verapamil Vibramycin Viloxazine HCl [Vinblastine] [Vincristine] Zuclopenthixol
*These drugs have been associated with acute attacks of porphyria. †Bracketed [] drugs are those in which experimental evidence of porphyringenicity is conflicting.

Hereditary Coproporphyria Clinical Presentation

History

Physical

Causes