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J Derm > Porphyrias > Flashcards

Porphyrias Flashcards

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Dermatology Board Prep flashcards
1
Q

what is the staging tool for normal skin tones

A

Fitzpatrick (I-VI, white-black) taking into consideration tanning/burning ability

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2
Q

generally speaking, what is a porphyria

A

a disease where porphyrins build up in the skin and nerves = abnormal cutaneous sensitivity

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3
Q

what are the 3 main types of porphyria

A 
porphyria cutanea tarda (PCT) type 1
erythropoietic protoporphyria (EPP) 
acute intermittent porphyria
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4
Q

what is the general presentation of porphyrias (3)

A

blistering
skin fragility
acute phototoxicity (sensitivity to light)

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5
Q

how is acute phototoxicity relieved

A

damp towel

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6
Q

what does PCT stand for

A

porphyria cutanea tarda

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7
Q

what causes porphyria cutanea tarda (PCT) type 1

A

uroporphyrinogen decarboxylase enzyme deficiency

starts with ‘porphyria’ so has ‘porphyrin’ in the name

LOTS OF THINGS CAUSE THIS eg alcohol, viral hepatitis

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8
Q

how does porphyria cutanea tarda (PCT) type 1 present (6)

A

blisters
fragility
hyperpigmentation
hypertrichosis (abnormal hair growth all over body)
solar uticaria (induced by light)
morphoea (patches of hardened skin on face and hands)

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9
Q

investigations for polyphyria cutanea tarda (PCT) type 1

A

woods lamp for diagnosis

find out cause

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10
Q

treatment of porphyria cutanea tarda (PCT) type 1

A

relieve skin

fix underlying problem

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11
Q

what does EPP stand for

A

erythropoietic protoporphyria

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12
Q

aetiology of erythropoietic protoporphyria (EPP)

A

ferrochelatase deficiency

think bc erythro = ferro

may be a liver problem (other causes too)

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13
Q

presentation of erythropoietic protoporphyria (EPP) (3)

A

easily sunburnt
itchy skin
phototoxicity

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14
Q

can erythropoietic protoporphyria (EPP) occur in kids

A

yes

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15
Q

investigations for erythropoietic protoporphyria (EPP)

A 
RBC porphyrins (blood tests)
LFTs
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16
Q

treatment of erythropoieic protoporphyria (EPP)

follow up of erythropoietic protophyria (EPP)

A

only if bad
avoid sunlight, wear dark colours, wear sunscreen etc
Dundee creams (zinc oxide)
antioxidants (not iron)

6 monthly LFTs and RBC porphyrins

17
Q

what is acute intermittent porphyria (what is wrong in the body)

A

haemoglobin deficiency

18
Q

which enzyme is deficient in acute intermittent porphyria

A

porphobilinogen (PBG) deaminase enzyme deficiency

19
Q

how does acute intermittent porphyria present (4) - NOT a derm condition

A

poorly localised abdo pain
psychotic symptoms - anxiety, depression, hallucinations
seizures
urinary symptoms

20
Q

is acute intermittent porphyria genetic or environmental

A

genetic

21
Q

investigations for acute intermittent porphyria

A

high serum/urine porphobilinogen

low RBC porphobilinogen deaminase

J Derm (23 decks)

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