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A metabolic disorder - or rather a group of disorders - that result from improper breakdown of haem. The same pigment that makes blood red causes problems if not dealt with completely by the body. Some sufferers have skin problems, especially after exposure to light which seems to sensitize the skin. More dangerous is an acute attack - vomiting, constipation and even mental disturbances like depression, hallucinations or schizophrenia. Attacks can be brought on by diet, certain pharmaceuticals and alcohol.

Interestingly, George The Third may have been a sufferer. His behaviour under the influence of severe attacks, combined with symptoms such as the colour of his stools suggests porphyria. Since certain types of porphyria are inherited, the disease can be traced through the royal family (in much the same way as haemophillia was).

Culled from various internet sources, and a play I saw once.

Some have suggested Porphyria as being the origin of the ancient legends of vampirism. Among the manifestations caused by the resulting anemia are pale skin, red eye discolourations, retracted gingiva and increased sensitivity towards sunlight. Some have even claimed that the anemia might present as an extreme thirst for blood, although I doubt that medical evidence for this is very supportive.

Acute Intermittent Porphyria (AIP):

AIP is non-gender specific hereditary. People with AIP are at risk of developing an acute attack. With AIP, unlike other forms of porphyria, the skin will not be affected.

Variegate Porphyria (VP) and Hereditary Coproporphyria (HC):

Like AIP, VP and HC are hereditary and acute attacks are a possibility, unlike AIP patients tend to have skin problems.

Plumboporphyria (PP):

PP is an extremely rare version of an extremely rare condition. In the few known cases, it has been seen to be very similar to AIP.

Porphyria Cutanea Tarda (PCT):

This form of porphyria can be inherited, but this is rare. It is a condition that may because of excessive alcohol ingestion. Other causes are the contraceptive pill and other non-natural oestrogen sources. PCT sufferers will have skin problems, but no acute attacks. Also, the unsafe drugs list is inapplicable, although patients should avoid alcohol.

Erthyropoeitic Protoporphyria (EPP):

Also rare, this form is hereditary. Skin problems may be an issue, but the unsafe drugs are not.

Congenital Porphyria (CP)

This is the rarest of the porphyria. It is primarily a skin condition. Both parents need to have porphyria.

The acute attack

When the levels of the porphyrin get too high an acute attack occurs. Symptoms include:

  • abdominal pain
  • cramps
  • constipation
  • nausea
  • vomiting
  • anxiety
  • depression
  • paralysis
  • death
  • hypertension
  • increased photosensitivity
  • Nerve damage (esp on the back causing excrutiating back pain requiring morphine)

Skin problems

Problems include ice-pick scabs and blisters. The skin is generally weak and so is easily scraped off. This problem is heightened with exposure to sunlight.

Unsafe Drugs

Bracketed drugs are those in which there is conflicting experimental evidence of porphyrinogenicity - some positive, - some negative

* Those marked with an asterisk have been associated with acute attacks of porphyria.

A:

  • ALCURONIUM
  • *ALPHAXALONE: ALPHADOLONE
  • ALPRAZOLAM
  • ALUMINIUM PREPARATIONS
  • AMIDOPYRINE
  • AMINOGLUTETHIMIDE
  • AMINOPHYLLINE
  • AMIODARONE
  • *AMITRIPTYLINE
  • (AMPHETAMINES)
  • *AMYLOBARBITONE
  • ANTIPYRINE
  • *AURANOFIN
  • *AUROTHIOMALATE
  • AZAPROPAZONE

    B:

  • BACLOFEN
  • *BARBITURATES
  • *BEMEGRIDE
  • BENDROFLUAZIDE
  • BENOXAPROFEN
  • BENZBROMARONE
  • (BENZYLTHIOURACIL)
  • (BEPRIDIL)
  • BROMOCRIPTINE
  • BUSULPHAN
  • *BUTYLSCOPOLAMINE

    C:

  • CAPTOPRIL
  • *CARBAMAZEPINE
  • *CARBROMAL
  • *CARISOPRODOL
  • (CEFUROXIME)
  • (CEPHALEXIN)
  • (CEPHALOSPORINS)
  • (CEPHRADINE)
  • (CHLORAMBUCIL)
  • *CHLORAMPHENICOL
  • *CHLORDIAZEPOXIDE
  • *CHLORMEZANONE
  • CHLOROFORM
  • *CHLORPROPAMIDE
  • CIMETIDINE
  • CINNARIZINE
  • CLEMASTINE
  • (CLOBAZAM)
  • (CLOMIPRAMINE HCl)
  • (CLONAZEPAM)
  • CLONIDINE HCL
  • *CLORAZEPATE
  • COCAINE
  • (COLISTIN)
  • CO-TRIMOXAZOLE
  • CYCLOPHOSPHAMIDE
  • CYCLOSERINE
  • CYCLOSPORIN

    D:

  • DANAZOL
  • *DAPSONE
  • DEXFENFLURAMINE
  • DEXTROPROPOXYPHENE
  • DIAZEPAM
  • *DICHLORALPHENAZONE
  • *DICLOFENAC Na
  • DIENOESTROL
  • DIETHYLPROPION
  • DIHYDRALAZINE
  • *DIHYDROERGOTAMINE
  • DILTIAZEM
  • *DIMENHYDRINATE
  • *DIPHENHYDRAMINE
  • (DOTHIEPIN HCl)
  • DOXYCYCLINE
  • *DYDROGESTERONE)

    E:

  • *ECONAZOLE NO3
  • *ENALAPRIL
  • ENFLURANE
  • *ERGOT COMPOUNDS
  • ERGOMETRINE MALEATE
  • ERGOTAMINE TARTRATE
  • *ERYTHROMYCIN
  • *ESTRAMUSTINE
  • ETHAMSYLATE
  • *ETHANOL
  • ETHIONAMIDE
  • *ETHOSUXIMIDE
  • *ETHOTOIN
  • ETIDOCAINE
  • ETOMIDATE

    F:

  • FENFLURAMINE
  • *FLUCLOXACILLIN
  • *FLUFENAMIC ACID
  • FLUNITRAZEPAM
  • FLUPENTHIXOL
  • FLURAZEPAM
  • *FRUSEMIDE

    G:

  • *GLIBENCLAMIDE
  • *GLUTETHIMIDE
  • *GLIPIZIDE
  • GRAMICIDIN
  • *GRISEOFULVIN

    H:

  • (HALOPERIDOL)
  • *HALOTHANE
  • *HYDANTOINS
  • *HYDRALAZINE
  • *HYDROCHLOROTHIAZIDE
  • *HYDROXYZINE
  • HYOSCINE

    I:

  • *IMIPRAMINE
  • IPRONIAZID
  • ISOMETHEPTENE MUCATE
  • (ISONIAZID)

    K:

  • KEBUZONE
  • KETOCONAZOLE

    L:

  • *LEVONORGESTROL
  • LIGNOCAINE
  • *LISINOPRIL
  • LOPRAZOLAM
  • LOXAPINE
  • *LYNESTRENOL
  • LYSURIDE MALEATE

    M:

  • MAPROTILINE HCl
  • MEBEVERINE HCl
  • *MECILLINAM
  • *MEDROXYPROGESTERONE
  • (MEFENAMIC ACID)
  • MEGESTROL ACETATE
  • *MEPHENYTOIN
  • MEPIVACAINE
  • *MEPROBAMATE
  • MERCAPTOPURINE
  • MERCURY COMPOUNDS
  • MESTRANOL
  • (METAPRAMINE HCl)
  • METHAMPHETAMINE
  • METHOHEXITONE
  • METHOTREXATE
  • METHOXYFLURANE
  • METHSUXIMIDE
  • *METHYL DOPA
  • *METHYL SULPHONAL
  • *METHYPRYLONE
  • METHYSERGIDE
  • *METOCLOPRAMIDE
  • METYRAPONE
  • MIANSERIN HCl
  • MICONAZOLE
  • (MIFEPRISTONE)
  • MINOXIDIL

    N:

  • *NADROLONE
  • *NALIDIXIC ACID
  • NATAMYCIN
  • *NANDROLONE
  • (NICERGOLINE)
  • *NIFEDIPINE
  • *NIKETHAMIDE
  • NITRAZEPAM
  • *NITROFURANTOIN
  • NORDAZEPAM
  • NORETHYNODREL
  • *NORETHISTERONE
  • (NORTRIPTYLINE)
  • NOVOBIOCIN

    O:

  • *ORAL CONTRACEPTIVES
  • *ORPHENADRINE
  • OXANAMIDE
  • (OXAZEPAM)
  • OXYBUTYNIN HCl
  • OXYCODONE
  • *OXYMETAZOLINE
  • *OXYPHENBUTAZONE
  • OXYTETRACYCLINE

    P:

  • 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

    Q:

  • QUINALBARBITONE

    R:

  • RIFAMPICIN

    S:

  • SIMVASTATIN
  • SODIUM AUROTHIOMALATE
  • SODIUM OXYBATE
  • (SODIUM VALPROATE)
  • *SPIRONOLACTONE
  • STANOZOLOL
  • SUCCINIMIDES
  • *SULPHACETAMIDE
  • *SULPHADIAZINE
  • *SULPHADIMIDINE
  • *SULPHADOXINE
  • *SULPHAMETHOXAZOLE
  • *SULPHASALAZINE
  • *SULPHONYLUREAS
  • SULPHINPYRAZONE
  • SULPIRIDE
  • SULTHIAME
  • SULTOPRIDE

    T:

  • *TAMOXIFEN
  • *TERFENADINE
  • TETRAZEPAM
  • *THEOPHYLLINE
  • *THIOPENTONE Na
  • THIORIDAZINE
  • TILIDATE
  • TINIDAZOLE
  • *TOLAZAMIDE
  • *TOLBUTAMIDE
  • TRANYLCYPROMINE
  • TRAZODONE HCl
  • TRIMETHOPRIM
  • (TRIMIPRAMINE)
  • TROXIDONE

    V:

  • VALPROATE
  • VALPROMIDE
  • VERALIPRIDE
  • *VERAPAMIL
  • *VIBRAMYCIN
  • VILOXAZINE HCl
  • (VINBLASTINE)
  • (VINCRISTINE)

    Z:

  • ZUCLOPENTHIXOL

    SAFE DRUGS

    A:

  • ACETAZOLAMIDE
  • ACETYLCHOLINE
  • ACTINOMYCIN D
  • ACYCLOVIR
  • ADENOSINE MONOPHOSPHATE
  • ADRENALINE
  • ALCLOFENAC
  • ALLOPURINOL
  • ALPHA TOCOPHERYL ACETATE
  • AMETHOCAINE
  • AMILORIDE
  • AMINOCAPROIC ACID
  • AMINOGLYCOSIDES
  • AMOXYCILLIN
  • AMPHOTERICIN
  • AMPICILLIN
  • ASCORBIC ACID
  • ASPIRIN
  • ATENOLOL
  • ATROPINE
  • AZATHIOPRINE

    B:

  • BECLOMETHASONE
  • BENZHEXOL HCl
  • BETA-CAROTENE
  • BIGUANIDES
  • (BROMAZEPAM)
  • BROMIDES
  • BUFLOMEDIL HCl
  • BUMETANIDE
  • BUPIVACAINE
  • BUPRENORPHINE
  • BUSERELIN
  • BUTACAINE SO4

    C:

  • 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 (ACTH)
  • COUMARINS
  • CYCLIZINE
  • CYCLOPENTHIAZIDE
  • CYCLOPROPANE
  • (CYPROTERONE ACETATE)

    D:

  • DANTHRON
  • DESFERRIOXAMINE
  • DEXAMETHASONE
  • (DEXTROMORAMIDE)
  • DEXTROSE
  • DIAMORPHINE
  • DIAZOXIDE
  • DICYCLOMINE HCl
  • DIFLUNISAL
  • DIGOXIN
  • DIHYDROCODEINE
  • DIMERCAPROL
  • DIMETHICONE
  • DINOPROST
  • DIPHENOXYLATE HCl
  • DIPYRIDAMOLE
  • (DISOPYRAMIDE)
  • DOMPERIDONE
  • DOXORUBICIN HCl
  • DROPERIDOL

    E:

  • (ESTAZOLAM)
  • ETHACRYNIC ACID
  • ETHAMBUTOL
  • (ETHINYL OESTRADIOL)
  • ETHOHEPTAZINE CITRATE
  • ETOPOSIDE

    F:

  • FAMOTIDINE
  • FENBRUFEN
  • (FENOFIBRATE)
  • FENOPROFEN
  • FENTANYL
  • FLUCYTOSINE
  • FLUMAZENIL
  • FLUOXETINE HCl
  • FLURBIPROFEN
  • FLUVOXAMINE MALEATE
  • FOLIC ACID
  • FRUCTOSE
  • FUSIDIC ACID
  • F.S.H.

    G:

  • GENTAMICIN
  • GLAFENINE
  • GLUCAGON
  • GLUCOSE
  • GLYCERYL TRINITRATE
  • GOSERELIN
  • GUANETHIDINE
  • GUANFACINE HCl

    H:

  • HAEM ARGINATE
  • (HALOPERIDOL)
  • HEPARIN
  • HEPTAMINOL HCl
  • HEXAMINE
  • (HYDROCORTISONE)

    I:

  • IBUPROFEN
  • INDOMETHACIN
  • INSULIN
  • IRON

    J:

  • JOSAMYCIN

    K:

  • (KETAMINE)
  • KETOPROFEN
  • KETOTIFEN

    L:

  • LABETALOL
  • L.H.R.H.
  • LIQUORICE
  • LITHIUM SALTS
  • LOFEPRAMINE
  • LOPERAMIDE
  • (LORAZEPAM)

    M:

  • 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

    N:

  • NADOLOL
  • NAFTIDROFURYL OXALATE
  • (NAPROXEN SODIUM)
  • NATAMYCIN
  • NEFOPAM HCl
  • NEOSTIGMINE
  • NETILMICIN
  • NIFLUMIC ACID
  • NITROUS OXIDE
  • NORFLOXACIN

    O:

  • OFLOXACIN
  • OXOLINIC ACID
  • OXYBUPROCAINE
  • (OXYPHENBUTAZONE)
  • OXYTOCIN

    P:

  • (PANCURONIUM BROMIDE)
  • PARACETAMOL
  • PARALDEHYDE
  • PARAPENZOLATE Br
  • PENICILLAMINE
  • PENICILLIN
  • PENTOLINIUM
  • PERICYAZINE
  • PETHIDINE
  • PHENFORMIN
  • PHENOPERIDINE
  • PHENTOLAMINE MESYLATE
  • PIPOTHIAZINE 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)

    Q:

  • QUINIDINE
  • QUININE

    R:

  • (RANITIDINE)
  • RESERPINE
  • RESORCINOL

    S:

  • SALBUTAMOL
  • SENNA
  • SODIUM BROMIDE
  • SODIUM Ca EDTA
  • SODIUM FUSIDATE
  • SORBITOL
  • STREPTOMYCIN
  • SULBUTIAMINE
  • SULINDAC
  • SULFADOXINE
  • SUXAMETHONIUM

    T:

  • 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

    V:

  • VANCOMYCIN
  • (VINCRISTINE)
  • VITAMINS

    W:

  • WARFARIN SODIUM

    Z:

  • ZIDOVUDINE
  • ZINC PREPARATIONS (TOPICAL)

    Source: Michael R. Moore, Porphyria Research Unit, The University Of Queensland, NRCET, Department Of Medicine 1999

  • This was adapted from a report I wrote as a final exam for my genetics class.

    Porphyrias are a group of diseases which are all deficiencies of any of the eight enzymes involved in heme synthesis. One of the major causes of symptoms in patients with porphyria is the accumulation of heme precursors. To make heme, the body needs some raw materials in addition to the eight enzymes. Heme procursors collect mainly in the marrow and liver. The main heme precursors that cause problems in porphyria are toxic metabolites: delta-aminolevulinic acid and porphobilinogen, which are neurotoxins, and porphyrins. These precursors are transferred from the tissues where they accumulate into the blood plasma and from there they are excreted in urine and stools. Accumulation of these precursors can cause photosensitivity and nerve damage, even paralysis.

    The most common porphyrias are porphyria cutanea tarda (PCT), acute intermittent porphyria, and eryhtropoietic protoporphyria. These are very different diseases, with distinct symptoms, tests needed for proper diagnosis, and treatments. Additionally, they all share some symptoms with rarer porphyrias. Thus, porphyria is often misdiagnosed. Test results from patients with porphyria will be markedly abnormal, but one needs to select and interpret tests with porphyria in mind.

    All porphyrias except for one are hereditary. PCT is not inherited directly. However, suspectibility to PCT is often inherited, in which case it is called familial (type II) PCT.

    There are several systems for classifying porphyria. The preferred system is to classify by specific enzyme deficiency. Deficiencies of different enzymes are responsible for different porphyrias. There is also a system which breaks porphyrias down into acute porphyrias, which manifest themselves primarily via neurologic symptoms, and cutaneous porphyrias, which cause photosensitivity. In addition, there is a third system which splits up the diseases into hepatic porphyrias, where the heme precursors originate in the liver, and erythropoietic porphyrias, where they originiate in the marrow.

    See my Porphyria Cutanea Tarda writeup for bibliography.

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