Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Dermatology > photodermatology and the porphyrias > Flashcards

photodermatology and the porphyrias Flashcards

You may prefer our related Brainscape-certified flashcards:
Dermatology Board Prep flashcards
1
Q

what is Photosensitivity

A

Photosensitivity is heightened skin sensitivity or an unusual reaction when your skin is exposed to UV radiation from sunlight or a tanning bed

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

what can cause Photosensitivity

A 
medications 
skin conditions 
other medical conditions 
skin care product 
genetics
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

what are the 2 types of Photosensitivity

A

Phototoxic reaction

Photoallergic reaction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Describe Phototoxic reaction

A

Phototoxic reaction

  • most common
  • caused mostly by drugs activated by sun light
  • can look or feel like a sun burn or a rash
  • triggered by skincare ingredience
  • happen in minutes or hours
  • limited to the skin
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Describe Photoallergic reaction

A
  • happens when UV light interacts with the ingredient in medication and other products applied directly on the skin.
  • The body’s immune system recognizes changes caused by sun exposure as a foreign threat. The body produces antibodies and attacks, causing a reaction. A photoallergic reaction can leave you with a rash, blisters, red bumps or even oozing lesions one to three days after application and exposure to the sun.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what is poephyria

A

it is a group of disorders that result from a buildup of natural chemicals that produce porphyrin in your body

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

what are porphyrins essential for

A

the function of hemoglobin

*they bind to it

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

what are the 2 types of porphyria

A
  • acute, which mainly affects the nervous system,

* cutaneous, which mainly affects the skin.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

true or false Porphyria is usually inherited

A

True, one or both parents pass along an abnormal gene to their child.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

true or false Porphyria can be cured

A

false

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

what is the most common type of cutaneous Porphyria

A

Porphyria cutanea tarda (PCT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

what are the symptoms of cutaneous Porphyria

A

As a result of sun exposure, you may experience:
• Sensitivity to the sun and sometimes artificial light, causing burning pain
• Sudden painful skin redness (erythema) and swelling (edema)
• Blisters on exposed skin, usually the hands, arms and face
• Fragile thin skin with changes in skin color (pigment)
• Itching
• Excessive hair growth in affected areas
• Red or brown urine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

what are the 2 types of inheritance pattern

A

Autosomal dominant :A defective gene from one of your parents
Autosomal recessive: Defective genes from both parents

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

true or false if you have the genes for porphyria you will present with symptoms

A

false : some people have latent porphyria (this is the case for most carriers)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

true or false Porphyria cutanea tarda (PCT) is mostly inherated

A

False
Porphyria cutanea tarda (PCT) typically is acquired rather than inherited, although the enzyme deficiency may be inherited. Certain triggers that impact enzyme production — such as too much iron in the body, liver disease, estrogen medication, smoking or excessive alcohol use — can cause symptoms.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

true or false when exposed to triggers your body’s demand for heme production increases

A

True

This overwhelms the deficient enzyme, setting in motion a process that causes a buildup of porphyrins.

17
Q

what are the triggers

A
  • Exposure to sunlight
  • Certain medications, including hormone drugs
  • Recreational drugs
  • Dieting or fasting
  • Smoking
  • Physical stress, such as infections or other illnesses
  • Emotional stress
  • Alcohol use
  • Menstrual hormones ― acute porphyria attacks are rare before puberty and after menopause in women
18
Q

what are the complications

A

• Cutaneous porphyrias can result in permanent skin damage. Also, the skin blisters can become infected. When your skin heals after cutaneous porphyria, it may have an abnormal appearance and coloring, be fragile, or leave scars.

19
Q

Prevention

A

you cant prevent it
avoid triggers
get family members to get genetic testing

20
Q

diagnosis

A

Tests include a combination of blood, urine or stool testing.
Genetic testing and counseling may be recommended in the family of a person with porphyria.

21
Q

treatment

A
  • Periodically drawing blood (phlebotomy) to reduce the iron in your body, which decreases porphyrins.
  • Taking a drug used to treat malaria — hydroxychloroquine (Plaquenil) or, less often, chloroquine (Aralen) — to absorb excess porphyrins and help your body get rid of them more quickly than usual. These medications are generally used only in people who can’t tolerate a phlebotomy.
  • A dietary supplement to replace vitamin D deficiency caused by avoidance of sunlight.
behavioural avoidance 
clothing 
sunscreen 
phototherapy PUV 
Chromophore removal 
inhibition of mediators/ action release 
inhibition of inflammatory response
22
Q

what is Fitzpartic sun-reaction skin phototypes

A

SPT I to IV

sometime V and VI are added

1 don’t tan
2 tan with difficulty brown after multiple exposure
3 tan very easily brown after first exposure
4 always tans

5 brown colour skin
6 black colour

23
Q

true or false eyelids are not involved in photosensitive reactions

A

TRUE

24
Q

describe porphyrins

A

chemicals that absorbe light

25
Q

describe chromophores

A

the chemicals that absorbe radiation

26
Q

list 4 diffrent porphyrias

A

Phototoxic skin porphyrias (such as erythropoietic protoporphyria)
Blistering and fragility skin porphyrias
Acute attack porphyrias (some with no skin involvement; some also cause blistering and fragility)
Severe congenital porphyrias (such as congenital erythropoetic porphyria)

27
Q

Is porphyria cutanea tarda a hepatic or erythopoetic porphyra ?

A

Heptic (problems mainly in the lver rather then bone marrow.

28
Q

What does uroporphyrinogen decarboxylase do?

A

This enzyme is involved in the production of a molecule called heme.

29
Q

how does PCT impact the function of uroporphyrinogen decarboxylase

A

PCT reduced activity of uroporphyrinogen decarboxylase disrupts heme production and allows byproducts of the process to accumulate in the body, triggering the signs and symptoms of porphyria cutanea tarda.

30
Q

signs of PCT

A 
Blisters on back of hands 
Errotions
Millia (appera when the dermal epidermal junction is damaged)
Bulla (uniocular, multiocular)
Hyperpigmentation
	Hypertrichosis
	Solar urticaria
	Morphoea
31
Q

what colour urin test indicates pct

A

purple

wave length is absorbed and reemited

32
Q

what are the causes of PCT

A

Alcohol
Viral hepatitis
Oestrogens
Haemochromatosis

33
Q

what enzyme has reduced activity in erythropoietic porphyria

A

Erythropoietic protoporphyria (EPP) is one of the cutaneous porphyrias. EPP is due to an inherited deficiency of the enzyme ferrochelatase. Reduced activity of this enzyme causes a build-up of protoporphyrin in the skin resulting in photosensitivity.

34
Q

true or false erythropoietic porphyria has sever symptoms but no signs

A

True
there is pain but nothing on the skin.

sometimes there are signs however

35
Q

what is the diagnosis method for erythropoietic porphyria

A

plasma scan

Urine is not helpul as they are not abnormal

36
Q

true or false The longer the wavelength the deeper it penetrates through the skin without being absorbed

A

True

37
Q

The main groups of porphyrias are:

A

Phototoxic skin porphyrias (such as erythropoietic protoporphyria)

Blistering and fragility skin porphyrias

Acute attack porphyrias (some with no skin involvement; some also cause blistering and fragility)

Severe congenital porphyrias (such as congenital erythropoetic porphyria)

38
Q

true or false Manifestation in the skin mainly relates to where the porphyrins mainly accumulate

A

true

39
Q

Which enzyme has reduced activity in porphyria cutanea tarda ?

A

Uroporphyrinogen nuro oxide synthase

Dermatology (9 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions