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CUTANEOUS ASPECTS OF PORPHYRIA
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Photosensive Drugs

PORPHYRIA FACTS

CUTANEOUS ASPECTS OF PORPHYRIA

Both phototoxic and photoallergic reactions occur in sun-exposed areas of skin,
including the face, V area of the neck, and dorsa of the hands and forearms.

The hair-bearing scalp, postauricular and periorbital areas, and submental
portion of the chin are usually spared.

A widespread eruption suggests exposure to a systemic photosensitizer,
whereas a localized eruption indicates a reaction to a locally applied topical
photosensitizer

Acute phototoxic reaction
In acute phototoxic reactions, necrotic keratinocytes are observed. If the reaction
is severe, the necrosis is panepidermal.

Biopsy
A biopsy refers to a procedure that involves obtaining a tissue specimen for
microscopic analysis to establish a precise diagnosis.

Biopsies can be accomplished with a biopsy needle (passed through the skin
into the organ in question) or by open surgical incision.

Blistering
The histologic features of a SCLE-like reaction reveal an interface dermatitis that
is indistinguishable from non–drug-induced SCLE.

Like porphyria cutanea tarda, pseudoporphyria causes a subepidermal blister at
the level of the lamina lucida.

A characteristic feature of both pseudoporphyria and porphyria cutanea tarda is
festooning, which refers to the irregular configuration of the dermal papillae in
the floor of the bulla.

Corticosteroids
These agents have anti-inflammatory properties and cause profound and varied
metabolic effects.

Corticosteroids modify the body's immune response to diverse stimuli. Potent
class I and II topical steroids may be used.

Less potent topical steroids such as hydrocortisone valerate, desonide, or
fluticasone may be used twice a day in children to decrease risk of systemic
absorption.

Dermatitis
Dermatitis is the inflammation of the skin. Found in the cutaneous forms of
porphyria.

Dermatitis, allergic contact
A contact dermatitis due to allergic sensitization to various substances.

These substances subsequently produce inflammatory reactions in the skin of
those who have acquired hypersensitivity to them as a result of prior exposure.

Dermatitis, photoallergic
A delayed hypersensitivity involving the reaction between sunlight or other
radiant energy source and a chemical substance to which the individual has
been previously exposed and sensitised.

It manifests as a papulovesicular, eczematous, or exudative dermatitis occurring
chiefly on the light-exposed areas of the skin.

Dermatitis, phototoxic
A nonimmunologic, chemically induced type of photosensitivity producing a
sometimes vesiculating dermatitis. It results in hyperpigmentation and
desquamation of the light-exposed areas of the skin.

Dermatologist
A medical specialist expert in the treatment of disorders of the skin. A
dermatologist would be the specialist dealing with cutaneous symptoms of
porphyria.

Drug-induced photosensitivity

Patients with drug-induced photosensitivity reactions should be warned against
the use of tanning beds and about potential cross-reactions of the offending
drug. The risks of severe sunburn reactions including the potential for and
complications from widespread blistering reactions, should be discussed with the
patient.


Drug-induced photosensitive reactions also may include lupuslike reactions.
Drug-induced reactions usually resemble SCLE because of their scaling,
annular, and psoriasiform characteristics.
Hydrochlorothiazide is the drug most frequently associated with this reaction, but
calcium channel blockers, ACE inhibitors, griseofulvin, and terbinafine are other
agents that have been implicated. The rate of reaction is low for any of these
agents. Hydrochlorothiazide is commonly used in many combined
antihypertensive agents.
Patients with drug-induced reactions have anti-Ro (SS-A) antibodies.


Erythema
A name applied to redness of the skin produced by congestion of the capillaries,
which may result from a variety of causes, the aetiology or a specific type of
lesion often being indicated by a modifying term.

Frequency of photosensitivity
IThe incidence of drug-induced photosensitivity in the United States is uncertain,
however phototoxicity is considerably more common than photoallergic
reactions.

Hyperpigmentation
Darkening of the skin. In some cutaneous forms of porphyria hyperpigmentation
will occurr.

Keratin
Keratin is a protein that is a primary constituent of hair, nails and skin.


Lichenoid reactions that occur in a photodistribution are often difficult to
distinguish from idiopathic lichen planus.
These reactions are characterized by violaceous or erythematous papules and
plaques that sometimes have Wickham striae.
Hydrochlorothiazide and captopril are known causes of drug-induced lichenoid
reactions.


Mortality/Morbidity:
Drug-induced photosensitivity is associated with death only in rare individuals
who are exposed to large amounts of sunlight after taking large doses of
psoralens.

Although mortality is rare, drug-induced photosensitivity can cause significant
morbidity in some individuals, who must severely limit their exposure to natural
or artificial light.

The carcinogenic potential due to prolonged exposure to these photosensitizing
drugs has been suggested; its clinical relevance remains to be determined.

Pharmacotherapy in photosensitivity
The goal of pharmacotherapy is to reduce morbidity and to prevent
complications. Broad-spectrum sunscreens with coverage in the UV-A and UV-B
ranges are recommended. Sunscreens containing avobenzone (Parsol 1789)
absorb light in the UV-A range. Physical sunscreen agents, such as titanium
dioxide and zinc oxide, have full UV spectrum protection. Some chemical
sunscreens are sensitizers and may induce contact dermatitis and photoallergy.
Therefore, selecting a sunscreen without a photosensitivity potential is
imperative.

phlebotomy
An entry into a vein to release blood. It is done to treat an excess of red blood
cells and is done periodically. One form of treatment for PCT [porphyria
cutaneous tarda].


Photoallergic reactions in skin

Photoallergic reactions can be caused by either topical or systemic
administration of the chemical.

Photoallergic reactions typically develop in sensitized individuals 24-48 hours
after exposure. The reaction usually manifests as a pruritic eczematous eruption.
Erythema and vesiculation are present in the acute phase.
More chronic exposure results in erythema, lichenification, and scaling.
Hyperpigmentation does not occur in photoallergic reactions.

Photoallergic reactions are histologically similar to contact dermatitis. Epidermal
spongiosis with a dermal lymphocytic infiltrate is a prominent feature.

photosensitive rash
A rash that occurs from the use of a particular drug when exposed to sunlight.
<pharmacology> Medications known to produce a photosensitive skin reaction
include: captopril, chlordiazepoxide, furosemide, griseofulvin, oral
contraceptives, phenothiazines, sulphonamides, tetracycline, demeclocycline
and thiazide diuretics. Most of these pharmaceuticals are considered UNSAFE
for use with porphyria patients.

photosensitivity
An abnormal cutaneous response involving the interaction between
photosensitising substances and sunlight or filtered or artificial light at
wavelengths of 280-400 mm. There are two main
types: photoallergy and photoxicity. Many porphyria patients also have
photosensitivity.

photosensitivity disorders
Abnormal responses to sunlight or artificial light due to extreme reactivity of
light-absorbing molecules in tissues. It refers almost exclusively to skin
photosensitivity, including sunburn, reactions due to repeated prolonged
exposure in the absence of photosensitizing factors, and reactions requiring
photosensitizing factors such as photosensitizing agents and certain diseases.
With restricted reference to skin tissue, it does not include photosensitivity of the
eye to light, as in photophobia or photosensitive epilepsy.

Photosensitizing drugs may also cause a lichen planus&#8211;like eruption in
sun-exposed areas. Drugs likely to cause this type of reaction include
demeclocycline, hydrochlorothiazide, enalapril, quinine, quinidine, chloroquine,
and hydroxychloroquine.


Phototoxic injury
Repeated phototoxic injury results in premature photoaging and an increased
risk of cutaneous malignancies.

Phototoxic reactions in skin

Most phototoxic reactions result from the systemic administration of drugs.

Acute phototoxicity often begins as an exaggerated sunburn reaction with
erythema and edema within minutes to hours of light exposure.

Vesicles and bullae may develop with severe reactions.

The lesions often heal; hyperpigmentation resolves in a matter of weeks to
months.

Chronic phototoxicity may appear as an exaggerated sunburn reaction.

Often, lichenification occurs because of repeated rubbing and scratching of the
photosensitive area.

Thus, distinguishing phototoxic reactions from photoallergic reactions strictly
based on physical appearance of the lesions may be difficult.

Other less common skin manifestations of phototoxicity include pigmentary
changes.

A blue-gray pigmentation is associated with several agents, including
amiodarone, chlorpromazine, and some tricyclic antidepressants.

Reactions to psoralen-containing botanicals (phytophotodermatitis) and drugs
may resolve, with a brownish discoloration.

Frequently, the pigmentary change is preceded by a typical sunburn reaction.

If the reaction is not severe, some patients may not notice the erythema

Phototoxic reactions in nails
Photo-onycholysis, or separation of the distal nail plate from the nail bed, is
another manifestation of phototoxicity.

Photo-onycholysis has been reported with the use of many systemic
medications, including tetracycline, psoralen, chloramphenicol, fluoroquinolones,
oral contraceptives, quinine, and mercaptopurine.

Photo-onycholysis may be the only manifestation of phototoxicity in individuals
with heavily pigmented

Pseudoporphyria, which involves porphyria cutanea tarda&#8211;like changes of skin
fragility and subepidermal blisters on the dorsa of hands, may occur after
exposure to naproxen, nalidixic acid, tetracycline, sulfonylureas, furosemide,
dapsone, amiodarone, bumetanide, and pyridoxine.

Frequent use of sun-tanning beds and chronic renal failure are other
predisposing factors.

Racial incidence
The racial incidence of drug-induced photosensitivity reactions is unknown.
Photosensitivity reactions can occur in races with heavily pigmented skin.

Sexual preference
Men are more likely to have photoallergic reactions than women.

Skin abnormalities -
Congenital structural abnormalities of the skin.

Skin diseases, genetic
Diseases of the skin with a genetic component, usually the result of various
inborn errors of metabolism. The cutaneous forms of porphyria are considered
genetic skin diseases.

Skin diseases, metabolic
Diseases of the skin associated with underlying metabolic disorders. The
cutaneous forms of porphyria are considered metabolic skin diseases.


Sun protection measures
Appropriate sun protection measures prevent drug-induced photosensitivity
reactions.

If sunscreens are not the cause of the photosensitivity, they should be used
liberally.

The sun protection factor (SPF) may not be a reliable indicator of protection
against drug-induced photosensitivity.

The SPF refers to the degree of protection against sunlight-induced sunburn,
primarily that caused by UV-B.

Most drug-induced photosensitivity reactions are caused by wavelengths within
the UV-A range.

Therefore, sunscreens that absorb UV-A should be prescribed.

Sunscreens that contain Parsol 1789, titanium dioxide, and zinc oxide are more
effective in blocking out UV-A radiation than sunscreens that contain other
ingredients.
+++++++++++++

PIGMENTATION

What is pigment and what causes skin color changes in porphyria?

Pigments are organic coloring molecules within the body.

They may be found in the bile, blood, urine, choroid (eye), or skin and hair.

Blood, bile and urinary pigments are derived from hemoglobin or the products of
hemoglobin catabolism.

The endogenous pigments of the choroid, skin and hair are called melanins.

SOURCE:
Pigementation change
Richard Jacobsen, MD PHD
Hematology
++++++++++++++++

Skin is the largest organ of the body.

It is made up of two layers, the upper 'epidermis' and the lower 'dermis'.

The epidermis and the dermis are further divided into other layers.

The lower most layer of the epidermis is known as the basal layer and it contains
organelles called 'melanosomes'.

SOURCE:
Valarie Backstrom MNS RN
Dermatology
+++++++++++

Melanin in skin cells are responsibe for hyperpigmentation in porphyria patients.

SOURCE:
Robert Johnson MD
+++++++++++

Melanosomes contain cells called melanocytes which produce a pigment called
'melanin'.

The color of skin depends mainly on this melanin and the amount of melanin
present in the other layers of the epidermis.

The thickness of epidermis and vascularity of the epidermis are other factors
affecting the color of skin.

SOURCE:
Valarie Backstrom MNS RN
Dermatology
+++++++++++

Abnormal pigmentation, and milia formation are commonly associated with PCT
patients.

SOURCE:
Porphyria: Don't Forget to Look At the Skin
Dr. Michael W. Rich
Professor of Medicine
Internal Medicine
Northeastern Ohio Universities
College of Medicine, Rootstown
Summa Health System, Akron.
+++++++++++++


What is hyperpigmentation?

Darkening of the skin.

SOURCE:
Stedman's Medical Encylcopedia
++++++++++++++++

Are changes in pigementation associated with PCT?

In PCT time milia, areas of pigmentation and de-pigmentation may occur.

SOURCE:
Medic Handbook
1995
++++++++++++++++

The abnormal porphyrin pigment is often grossly evident in visible light and
yields a pink fluorescence in Wood lamp ("black light") radiation

SOURCE:
Porphyria Cutaneous Tarda
Maureen B Poh-Fitzpatrick, MD,
Professor Emerita and Special Lecturer,
Department of Dermatology,
Columbia University College of Physicians and Surgeons,
Clinical Professor of Medicine,
Division of Dermatology,
University of Tennessee College of Medicine
eMedicine Journal
+++++++++++++++

Can darkening skin patches similar to bruises appear as part of skin
invovlement in porphyria?

When the skin is involved, as it is in all non-acute and some acute types,
symptoms from sensitivity to sunlight may include a wide variety of problems
such as rashes, blisters, changes in skin pigment, changes in facial hair, and
fragile skin that injures more easily."

SOURCE:
Evaluating Porphyrin Metabolism
Dr. Grace Ziem, MD
1996
+++++++++++++++
How does hyperpigmentationtake place in porphyria?


Melanins are synthesized in melanosomes from the amino
acid tyrosine into dopa and dopaquinone.

The enzyme tyrosinase is required in these early steps.

After the tyrosinase steps, the pathways to produce
black/brown and amber/red pigments diverge and involve
many other enzymes.

MSH treatment accelerates melanin synthesis and causes
the skin to visibly darken.

SOURCE:
Pigementation change
Richard Jacobsen, MD PHD
Hematology
++++++++++++++++++

How does hypertrichosis present in PCT?

Hypertrichosis is often observed, most florid over temporal and malar facial
areas, but also sometimes present on arms and legs.

SOURCE:
Porphyria Cutaneous Tarda
Maureen B Poh-Fitzpatrick, MD,
Professor Emerita and Special Lecturer,
Department of Dermatology,
Columbia University College of Physicians and Surgeons,
Clinical Professor of Medicine,
Division of Dermatology,
University of Tennessee College of Medicine
eMedicine Journal
+++++++++++++
Is hyperpigementation associated with PCT?

Hyperpigmentation may occur.

SOURCE:
Familial and sporadic porphyria cutanea
de Verneuil, H.; Aitken, G.; Nordmann, Y. :
Human Genetics
44: 145-151, 1978.
++++++++++++++++

Hyperpigementation can appear on the face or the arms.

SOURCE:
Robert Johnson M.D.
Internal Medicine
+++++++++++++
Is pigmentation asociated with PCT?

The lesions of PCT can leave depigmented and pigmented scars.

SOURCE:
Acute porphyrias:
Diagnosis and management.
Solberg, L.A. et. al.
Mayo Clinical Proceedings
1994;69:991-995.
+++++++++++++++++++
Is pigmentation change associated with PCT?

Pigmentation may be manifested in PCT patients.

SOURCE:
Acute porphyrias:
Diagnosis and management.
Solberg, L.A. et. al.
Mayo Clinical Proceedings
1994;69:991-995.
+++++++++++++++++++++
In time milia, areas of pigmentation and de-pigmentation can occur in PCT

SOURCE:
The Porphyrias
The Metabolic Basis of Inherited DIsease
Kappas, Sassa et. al.
1996
++++++++++++++++++++


Where does hyperpigmentation occur in PCT patients?

Pigmentary changes include melasma-like hyperpigmentation of the face,and an
erythematous suffusion or plethora of the central face, neck, upper chest and
shoulders may be present.

SOURCE:
Porphyria Cutaneous Tarda
Maureen B Poh-Fitzpatrick, MD,
Professor Emerita and Special Lecturer,
Department of Dermatology,
Columbia University College of Physicians and Surgeons,
Clinical Professor of Medicine,
Division of Dermatology,
University of Tennessee College of Medicine
eMedicine Journal
+++++++++++++++++
What color are PCT porphyrins?

PCT porphyrins are reddish pigments that accumulate in the liver and are
disseminated in plasma to other organs.

SOURCE:
Porphyria Cutaneous Tarda
Maureen B Poh-Fitzpatrick, MD,
Department of Dermatology
University of Tennessee College of Medicine
++++++++++++++++++++


Is there pigmentation change in HEP?


Pigmentation changes are common in HEP.


SOURCE:
The Porphyrias
The Less Common Porphyrias
Endocrine and Metabolic Disorders
Merck Manual of Diagnosis
+++++++++++++++++
Is pigmentation commonly associated with EPP?

Pigmentation can follow chronic sun exposure in EPP..

SOURCE:
Dr. Robert Johnson M.D.
Retired Clinician
++++++++++++++++++

What is Melanin?

How is it associated with porphyria?


Melanin is the pigment material of the skin.

In PCT and other cutaneous forms of porphyria the pigmentation
will change when the skin is exposed to direct sunlight.

SOURCE:
Vitamins and Minerals:
Healthy Diet & Safe Supplementation
Denise Mortimore
Element
2001
++++++++++++++++++

Some hyper pigmentation can be treated with topical creams such as
alpha-hydroxy quinone, tretinoin, topical steroids or azelic acid.

If the porphyria patient does not improve with these medicines other modalities
should be used.

Such modalities include (1) chemical peeling with tri-chloro acetic acid or
glycolic acid; (2) Electrical stimulation of the skin; (3)Iontophoresis; or (4) Laser
surgery.

SOURCE:
Valarie Backstrom MNS RN
Dermatology
+++++++++++

HYPERPIGMENTATION

Pigments are organic coloring molecules within the body.

They may be found in the bile, blood, urine, choroid (eye), or skin and hair.

Blood, bile and urinary pigments are derived from hemoglobin or the products
of hemoglobin catabolism.

The endogenous pigments of the choroid, skin and hair are called melanins.

SOURCE:
Pigementation change
Richard Jacobsen, MD PHD
Hematology
++++++++++++++++++++++++

What causes hyperpigmentation in porphyria patients?

External agents can also alter skin color.

Some internal compounds--such as the byproducts of hemoglobin
metabolism--may color the skin.

SOURCE:
Pigementation change
Richard Jacobsen, MD PHD
Hematology
+++++++++++++++++++++++

What is hyperpigmentation?

Darkening of the skin.

SOURCE:
Stedman's Medical Encylcopedia
+++++++++++++++++++++++++

Darkening skin patches similar to bruises appear as part of skin invovlement in
porphyria.

SOURCE:
Robert Johnson M.D.
Internal Medicine
+++++++++++++++++++++++
Hyperpigmentation is experienced by 8% of porphyria patients.

SOURCE:
United Health Services
Medical Education Department
1999
++++++++++

When the skin is involved, as it is in all non-acute and some acute types,
symptoms from sensitivity to sunlight may include a wide variety of problems
such as rashes, blisters, changes in skin pigment, changes in facial hair, and
fragile skin that injures more easily."

SOURCE:
Evaluating Porphyrin Metabolism
Dr. Grace Ziem, MD
1996
++++++++++++++++++++++++++

What is Melanin?

How is it associated with porphyria?

Melanin is the pigment material of the skin.

In PCT and other cutaneous forms of porphyria the pigmentationwill change
when the skin is exposed to direct sunlight.

SOURCE:
Vitamins and Minerals:
Healthy Diet & Safe Supplementation
Denise Mortimore
Element
2001
++++++++++++++++++++++++


Are changes in pigementation associated with PCT?

In PCT time milia, areas of pigmentation and de-pigmentation may occur.

SOURCE:
Medic Handbook
1995
+++++++++++++++++++]
Pigmentation may be manifested in PCT patients.

SOURCE:
Acute porphyrias:
Diagnosis and management.
Solberg, L.A. et. al.
Mayo Clinical Proceedings
1994;69:991-995.
++++++++++++++++++++

The lesions of PCT can leave depigmented and pigmented scars.

SOURCE:
Acute porphyrias:
Diagnosis and management.
Solberg, L.A. et. al.
Mayo Clinical Proceedings
1994;69:991-995.
++++++++++++++++++++

Where does hyperpigmentation occur in PCT patients?

Pigmentary changes include melasma-like hyperpigmentation of the face,and an
erythematous suffusion or plethora of the central face, neck, upper chest and
shoulders may be present.

SOURCE:
Porphyria Cutaneous Tarda
Maureen B Poh-Fitzpatrick, MD,
Professor Emerita and Special Lecturer,
Department of Dermatology,
Columbia University College of Physicians and Surgeons,
Clinical Professor of Medicine,
Division of Dermatology,
University of Tennessee College of Medicine
eMedicine Journal
++++++++++++++++++++++++++
What color are PCT porphyrins?

PCT porphyrins are reddish pigments that accumulate in the liver and are
disseminated in plasma to other organs.

SOURCE:
Porphyria Cutaneous Tarda
Maureen B Poh-Fitzpatrick, MD
Department of Dermatology
University of Tennessee College of Medicine
++++++++++++++++++++++++


Is there pigmentation change in HEP?

Pigmentation changes are common in HEP.

SOURCE:
The Porphyrias
The Less Common Porphyrias
Endocrine and Metabolic Disorders
Merck Manual of Diagnosis
+++++++++++++++++++++++++

Is pigmentation commonly associated with EPP?

Pigmentation can follow chronic sun exposure in EPP..

SOURCE:
Dr. Robert Johnson M.D.
Internal Medicine
+++++++++++++++++++++++++

When the skin is involved, as it is in all non-acute and some acute types,
symptoms from sensitivity to sunlight may include a wide variety of problems
such as rashes, blisters, changes in skin pigment, changes in facial hair, and
fragile skin that injures more easily.

SOURCE:
Evaluating Porphyrin Metabolism
Dr. Grace Ziem, MD
1996
++++++++++++++++++

External agents can also alter skin color.

Some internal compounds--such as the byproducts of hemoglobin
metabolism--may color the skin.

SOURCE:
Pigementation change
Richard Jacobsen, MD PHD
Hematology
++++++++++++

Hyperpigmentation is the darkening of the skin.

Such darkening can often appear in patches.

Some forms of cutaneous porphyria can produce hyperpigmentation.

SOURCE:
United Medical Services
Medical Terminology
1996
++++++++++

Hyperpigmentation is abnormally dark skin.

It is skin that has turned darker than normal.

SOURCE:
Melanie Abrams, FNP
Dermatology
+++++++++

Normal skin contains cells called melanocytes that produce the brown
skin-coloring pigment melanin.

There are several conditions in which melanocytes are either abnormal or
abnormally distributed.

Most skin conditions that cause discoloration are harmless.

SOURCE:
Michael Lehrer, M.D.
Department of Dermatology
University of Pennsylvania Medical Center
Philadelphia, PA.
++++++++++

Sometimes mistaken for a suntan, bronzing of the skin often develops gradually
starting at pressure points such as the elbows, knuckles, and knees and
spreading from there.

It is also seen in the creases of the soles of the feet and the palms of the hands.

The bronze color can range from light to dark (in fair skinned people) with the
intensity often a function of the underlying cause.

SOURCE:
Michael Lehrer, M.D.
Department of Dermatology
University of Pennsylvania Medical Center
Philadelphia, PA.
++++++++++

Some medications can cause hyperpigmentation.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++


Excessive exposure to the sun can cause hyperpigmentation.

SOURCE:
Melanie Abrams, FNP
Dermatology
+++++++++



Nonprescription depigmenting creams are available for lightening the skin.

If used, follow instructions carefully and don't use one for more than 3 weeks at
a time.

Darker skin requires greater care when using these preparations.

Cosmetics may also help in covering a discoloration.

SOURCE:
Michael Lehrer, M.D.
Department of Dermatology
University of Pennsylvania Medical Center
Philadelphia, PA.
++++++++++

Avoid too much sun exposure.

Always use sunscreens or blockers.

SOURCE:
Robert Johnson MD
Internal Medicine
+++++++++++

Hyperpigmentation may persist even after treatment, so emotional support is
recommended.

SOURCE:
Michael Lehrer, M.D.
Department of Dermatology
University of Pennsylvania Medical Center
Philadelphia, PA.
++++++++++

Hyperpigmentation is an aberration in which dark spots appear on the skin,
which often makes it cosmetically undesirable.

This benign condition is attributed to an overproduction of melanin, a
dark-colored pigment in the skin.

The relative amounts of melanin, as well as other skin pigments, genetically
determine an individual's skin color.

Thus, people with innately dark skin have more melanin than people with lighter
skin color.

SOURCE:
Ellis DA, Tan AK,
How we do it: management of facial hyperpigmentation,
Journal of Otolaryngology,
26, 286 (1997 Aug.)
++++++++++++


Sun exposure, especially ultraviolet radiation, is responsible for
hyperpigmentation in porphyria patients.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++

PHOTOSENSITIVITY

Some porphyria patients develop a rash because their skin is sensitive to
sunlight.

Such a reaction is known as photosensitivity.

SOURCE:
Valarie Backstrom MNS RN
Dermatology
+++++++++++

Photosensitivity is the major manifestation of some cutaneous porphyrias.

SOURCE:
The Porphyrias
Meyer, Urs A.
Harrison&#8217;s Principles of Internal Medicine,
12th ed. Mc GrawHill, 1991.
++++++++++++

For porphyria patients who are photosensitive, it is vital to select a Very High
Protection (SPF 45+), Water-Resistant and Broad Spectrum product.

SOURCE:
Miriam Morrison FNP
Dermatology & Allergy
++++++++++++

Certain types of commercial lighting may cause skin rash in porphyria patients.

SOURCE:
Robert Johnson MD
+++++++++++
Photosensitive porphyria patients should apply sunscreen liberally first thing in
the morning to all uncovered skin.

SOURCE:
Miriam Morrison FNP
Dermatology & Allergy
++++++++++++


Some porphyria patients may not associate their skin complaint with the light.

It is not always the bright summer sun which is responsible for such reactions.

Often cutaneous porphyria patients react to winter daylight.

Some porphyria patients who are very sensitive may even be affected by
fluorescent lamps indoors.

SOURCE:
Valarie Backstrom MNS RN
Dermatology
+++++++++++

UVR is divided into UVB (short wavelength rays which burn and tan) and UVA
(longer wavelength tanning rays).

Patients can be sensitive to one kind of sunlight (i.e. only to UVB, UVA or visible
light) or to a wider range of radiation.

The most common photosensitivity is to UVA.

Many porphyria patients find themselves being quite sensitive to all lighting.

SOURCE:
Miriam Morrison FNP
Dermatology & Allergy
++++++++++++

If you are photosensitive, it is vital to select a Very High Protection (SPF 45+),
Water-Resistant and Broad Spectrum product

SOURCE:
Valarie Backstrom MNS RN
Dermatology
+++++++++++

Sunlight contains both ordinary visible light and shorter invisible light rays called
ultraviolet radiation (UVR).

UVR can produce tanning but also causes burning.

In some people it can cause skin cancer.

In porphyria patients it can cause blistering, scarring and itching.

SOURCE:
Miriam Morrison FNP
Dermatology & Allergy
++++++++++++


Cutaneous porphyria patients should apply the sunscreen liberally first thing in
the morning to all uncovered skin.

SOURCE:

Valarie Backstrom MNS RN
Dermatology
+++++++++++

Porphyria patients should protect their lips with dark colored lipstick or a
UVR-absorbing lipsalve.

This is especially important for CEP porphyrics.

SOURCE:
Miriam Morrison FNP
Dermatology & Allergy
++++++++++++


Because they are photosensitive a porphyria patient often develops swelling,
redness, and blisters when exposed to the sun.

SOURCE:
The encyclopedia of baby and child care
Smith, L. H.
1980
New York, Warner Books.
++++++++++++

For the most severely light sensitive porphyria patients, normal activities may be
severely curtailed.

This is especially true for CEP patients.

SOURCE:

Marjorie Lambert FNP

Dermatology

+++++++++++

Cutaneous porphyria patients should protect their lips with dark colored lipstick
or a UVR-absorbing lipsalve.

Remember not to use lipstick with a perfume or alcohol base.

SOURCE:
Valarie Backstrom MNS RN
Dermatology
+++++++++++

When porphyria patients are outdoors, reapply sunscreens every two hours or
more often.

SOURCE:
Valarie Backstrom MNS RN
Dermatology
+++++++++++

Some porphyria patients find night work and sleep during the day, others put up
with the rash.

Nearly always, medications in the form of ointments or tablets, can help to a
variable extent.

Protective clothing is important if one may be exposed to any direct sunlight.

SOURCE:

Marjorie Lambert FNP

Dermatology

+++++++++++

Porphyria patients should always reapply suncreens after sweating heavily,
bathing, towelling dry or rubbing your skin.

SOURCE:
Valarie Backstrom MNS RN
Dermatology
+++++++++++

Photosensitivity can be confirmed by Phototests.

A phototest uses artificial light from various different sources which is shone on
small areas of the skin to see whether the rash can be reproduced, or if sunburn
occurs more easily than expected.

SOURCE:
Miriam Morrison FNP
Dermatology & Allergy
++++++++++++

UVR is present in significant quantities between September and April.

There is enough UVR to cause a rash on photosensitive skin between 10 a.m.
and 5 p.m. even on a cloudy day.

SOURCE:
Marjorie Lambert FNP
Dermatology
+++++++++++


The skin of most porphyria patients must not be exposed to the sun.

SOURCE:
The encyclopedia of baby and child care
Smith, L. H.
1980
New York, Warner Books.
++++++++++++

Bright surfaces, like snow, concrete and sand, reflect UVR and can nearly
double the amount that gets to the skin.

SOURCE:
Marjorie Lambert FNP
Dermatology
++++++++++++

Photosensitivity is the major manifestation in cutaneous porphyria patients.

SOURCE:
The Porphyrias
Meyer, Urs A.
Harrison's Principles of Internal Medicine,
12th ed. Mc GrawHill, 1991.
+++++++++++

Photosensitivity induced by contact with certain items can be tested by
Photopatch Tests.

Adhesive patches containing known photosensitizing materials are applied to the
upper back, removed after two days, and light is shone on the area.

The reaction is observed two days later.

SOURCE:
Miriam Morrison FNP
Dermatology & Allergy
++++++++++++

Porphyria patients with photosensivity cutaneous reactions should confine
excursions out of doors to early in the morning or late in the evening.

SOURCE:
Marjorie Lambert FNP
Dermatology
+++++++++++

Most photosensitive porphyria patients often find it difficult to find a sunscreen
they can tolerate. Suncreen ingredients can and often are contraindicated for
porphyria patients.

SOURCE:
Valarie Backstrom MNS RN
Dermatology
+++++++++++

Porphyria patients need to remember that sun protection is needed whatever the
weather. It is needed even if you sit in the shade.


SOURCE:
Marjorie Lambert FNP
Dermatology
++++++++++

Contact allergy or photoallergy to the sunscreening chemicals themselves can
occur, although this is uncommon, particularly PABA, benzophenone or butyl
methoxy dibenzoylmethane.

SOURCE:
Valarie Backstrom MNS RN
Dermatology
+++++++++++

Photoreactions can happen in porphyria patients.

SOURCE:
Miriam Morrison FNP
Dermatology & Allergy
++++++++++++

Patch and photopatch tests will identify which suncreens are safe for a porphyria
patient.

SOURCE:
Valarie Backstrom MNS RN
Dermatology
+++++++++++

Porphyria patients need to remember that sun protection is needed whatever the
weather. It is needed even if you sit in the shade.


SOURCE:
Marjorie Lambert FNP
Dermatology
++++++++++

UVA can pass through window glass.

Porphyria patients need to protect themselves when sitting inside
a building next to a window or traveling in a car as the UVA can
cause severe cutaneous problems for those with photosensitivity.

SOURCE:
Marjorie Lambert FNP
Dermatology
+++++++++++

Chemicals that produce a photoreaction (reaction with exposure to UV light) are
called photoreactive agents or, more commonly, photosensitizers.

After exposure to UV radiation either from natural sunlight or an artificial source
such as tanning booths or even those "purple-lighted" mosquito zappers, these
photosensitizers cause chemical changes that increase a person's sensitivity to
light, causing the person to become photosensitized.

Medications, food additives, and other products that contain photoreactive
agents are called photosensitizing products.



SOURCE:
Miriam Morrison FNP
Dermatology & Allergy
++++++++++++

Acute photosensitivity reactions in PCT are not common.

SOURCE:
Medic Handbook
1995
++++++++++++

Skin contact with various chemicals may result in a toxic or allergic reaction on
sun-exposed skin.

This is often called photocontact dermatitis.

SOURCE:
Marjorie Lambert FNP
Dermatology
++++++++++++

Use reflectant sunscreens if you are a PCT patient.

SOURCE:
Robert Johnson MD
++++++++++++

Photocontact dermatitis most often arises from tar products, fragrances and
sometimes sunscreens.

SOURCE:
Marjorie Lambert FNP
Dermatology
++++++++++

The FDA has reported that photoreactive agents have been found in
deodorants, antibacterial soaps, artificial sweeteners, fluorescent brightening
agents for cellulose, nylon and wool fibers, naphthalene (mothballs), petroleum
products, and in cadmium sulfide, a chemical injected into the skin during
tattooing.



SOURCE:
Miriam Morrison FNP
Dermatology & Allergy
++++++++++++

Zinc and titanium dioxide sunblocks should be applied to the most sensitive
areas of PCT patients.

Such sunscreens work by reflecting the ultraviolet radiation, and can be messy.

SOURCE:
Valarie Backstrom MNS RN
Dermatology
++++++++++

Blisters and brown streaks may occur from touching certain plants followed by
sun exposure.

This is known as phytophotodermatitis.

Some cutaneous porphyria patients have severe photocontact dematitus
sensitivity.

SOURCE:
Marjorie Lambert FNP
Dermatology
+++++++++++

UVR-absorbing film can be applied to windows at home or in the car
of porphyria patients who are sun sensitive.

SOURCE:
Leslie Griffin, FNP
Dermatology
+++++++++++

The most common causes are vegetables that contain photosensitising psoralen
chemicals.

SOURCE:
Marjorie Lambert FNP
Dermatology
++++++++++++

Photoreactive agents, such as Azulfidine, can cause both acute and chronic
effects.

Acute effects, from short-term exposure, include exaggerated sunburn-like skin
conditions, eye burn, mild allergic reactions, hives, abnormal reddening of the
skin, and eczema-like rashes with itching, swelling, blistering, oozing, and
scaling of the skin.

Chronic effects from long-term exposure include premature skin aging, stronger
allergic reactions, cataracts, blood vessel damage, a weakened immune system,
and skin cancer.

SOURCE:
Miriam Morrison FNP
Dermatology & Allergy
++++++++++++

Bonwyke DermaGard is one brand of sun oprotection film that can be used in
windows of a home or car.

SOURCE:
Leslie Griffin, FNP
Dermatology
+++++++++++

Widely used medications containing photoreactive agents include
antihistamines, used in cold and allergy medicines; nonsteroidal
anti-inflammatory drugs (NSAIDs), used to control pain and inflammation in
arthritis; and antibiotics, including the tetracyclines and the sulfonamides, or
"sulfa" drugs.


SOURCE:
Miriam Morrison FNP
Dermatology & Allergy
++++++++++++

There are many clear sunscreens that absorb UVR.

Photosensitive porphyria patients need to use sunscreen which can block out
more than 99% of the UVB and 90% of the UVA if applied correctly.

It should be noted that even done correctly, even the best however, may not
totally prevent photosensitivity rashes in very sensitive individuals, so minimize
time in the sun.

complete avoidance of the sun is necessary for some forms of porphyria.

SOURCE:
Miriam Morrison FNP
Dermatology & Allergy
++++++++++++


Photosensitizing medications can result in unexpected sunburn.

Such medications can only cause a dry, bumpy or blistering rash on exposed
skin (face, neck, arms, backs of hands and often lower legs and feet).

The rash may or may not be itchy.

They can also result in onycholysis (nail plate lifting off the nail bed).

The most common medications causing photosensitivity are diuretics,
tetracycline antibiotics and anti-inflammatory agents.

SOURCE:
Marjorie Lambert FNP
Dermatology
+++++++++++

Masks for porphyria patients sensitive to sun can be made to cover the face for
trips outside too (clear ones are available).

Surveys have found that only the most disabled patients are prepared to wear
these masks

SOURCE:
Leslie Griffin, FNP
Dermatology
+++++++++++

Unguarded fluorescent daylight lamps can occasionally provoke a rash in PCT
patients.

SOURCE:
Leslie Griffin, FNP
Dermatology
+++++++++++

UV lighting should be avoided by porphyria patients.

SOURCE:
Robert Johnson MD
++++++++++


There are two basic ways for cutaneous porphyria patients to protect their skin
from the damaging effects of UVR.



The first is to block out all light with an opaque material such as clothing.


Dark colored and densely woven fabric is the most effective.

Wear shirts with high collar and long sleeves, trousers or a long skirt, socks and
shoes, a wide-brimmed hat and if possible gloves.

Some clothes are now labelled with UPF, the sun protection factor for fabrics.

Be sure to choose those with a UPF of 40+.

The other option is to use sunscreens.





SOURCE:
Miriam Morrison FNP
Dermatology & Allergy
++++++++++++

Ordinary tungsten light bulbs are usually considered safe for PCT patients.

Viewing of televisions is considered safe for PCT patients.

SOURCE:
Leslie Griffin, FNP
Dermatology
+++++++++++

Barrier creams and avoidance of excess sunlight is thought to prevent
photosensitivity type reactions in variegate and hereditary coproporphyria.

Such barrier creams are advisable in the winter due to exposure to light glare
from snow.

SOURCE:
The Porphyrias
Alana Adams RPH
Welsh Drug Information Center
Cardiff, Wales, U.K.
++++++++++++++ +++++

Photosensitivity results from the interaction of sunlight with conjunctiva and,
possibly, in red blood cells in the superficial circulation.

The porphyrins absorb light in the 400-nm wavelengths, become photoexcited,
and cause local tissue damage, probably through production of free radicals.

SOURCE:
Psoriasis, ichthyosis, and porphyria.
Steiner, G. and R. C. Arffa
Int Ophthalmology Clin
1997; 37(2): 41-61.

++++++++++++

PHOTOSENSITIVE DRUGS

The following medications have beenindicated as as being photosensivity.

Be sure to take precautions to avoiding exposure to sunlight or UV lighting.

Antidiabetics -oral medications


Generic names (Common brand names):
acetohexamide (Dymelor, Dimelin, Dimelor, Gamadiabet, Ordimel, Toyobexin)
chlorpropamide (Glucamide, Diabinese, Abemide, Arodoc, Chlorabetic,
Chlordiabet, Chlormide, Chlorprosil, Copamide, Deavynfar, Diabemide, Diabenil,
Diabexan, Diamide, Diatanpin, Dibetes, Gliconorm, Glycermin, Glymese,
Hypomide, Insilange, Insulase, Meldian, Mellitos, Milligon, Norgluc, Normoglic,
Novopropamide, Orodiabin, Propamide, Tanpinin)
glipizide (Glucotrol, Glibenese, Glidiab, Glucotrol XI, Glynase, Mindiab, Minidab,
Minidiab)
glyburide (DiaBeta, Micronase, Glynase PresTab, Antibet, Azuglucon, Bastiverit,
Betanase, Calabren, D.B.T., Daonil, Debtan, Dibelet, Euglocon, Euglucan,
Gilbesyn, Gilemal, Glamide, Gliban, Glibenil, Glibesyn, Glibetic, Glimet,
Glucobene, Glucohexal, Glucolon, Glucoven, Glukovital, Glyben, Glynor,
Hemi-Daonil, Libanil, Melix, Migulcan, Semi-Daonil, Semi-Euglucon, Sugril,
Yuglucon)
tolazamide (Tolamide, Ronase, Tolinase, Diabewas, Diadutos, Norglycin,
Tolanase, Tolisan)
tolbutamide (Oramide, Orinase, Abemin, Aglicem, Aglycid, Ansulin, Arcosal,
Artosin, Diabecid-R, Diaben, Diatol, Dolipol, Fordex, Glucosulfa, Glyconon,
Guabeta, Mobenol, Noglucor, Novobutamide, Orabet, Orsinon, Rastinon,
Raston, Tolbusal, Tolbutamida Valdecases, Tolsiran)

* This list is not necessarily inclusive of all brand names.


INFORM YOUR HEALTH CARE PROVIDER
a.. if any of the following medical conditions are present
b.. liver disease
c.. porphyria (a condition in which the body utilizes porphyrins poorly, resulting
in paralysis, abdominal colic, skin lesions, mental disturbances, and murky or
discolored urine)
SUN EXPOSURE

These medications may cause photosensitivity.

Be careful when out in the sun, because the patient may sunburn faster.

It is best to use a good sun block (including one for the lips) and a wide brim hat.

If burning becomes a problem, stay out of the sun.

SOURCE:
Robert Johnson MD
Internal Medicine
++++++++++++++++++++

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PORPHYRIA FACTS is a medical education website dedicated to helping you focus your research on the inherited metabolic diseases known as the "Porphyrias".

PORPHYRIA FACTS is for individuals seeking information on Porphyria. The specific focus is on education, and research in the porphyrias.

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