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derm (mine) > porphyrias > Flashcards

porphyrias Flashcards

1
Q

what is porphyrias

A

group of diseases in which substances called porphyrins build up negatively affecting the skin or nervous system

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2
Q

what is the most common porphyrin

A

haem

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3
Q

what are the general symptoms of porphyrias

A

acute phytotoxicity
blistering
skin fragility

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4
Q

what is the treatment of porphyrias

A 
behavioural avoidance
clothing
sunscreen
hardening the skin (PUVA)
chromophore removal
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5
Q

what is Normal cutaneous photosensitivity

A

sunburn

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6
Q

what is used to classify response to sunlight

A

Fitzpatrick scale

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7
Q

where would white, freckled skin be on the Fitzpatrick scale

A

type 1

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8
Q

where would deep black skin be on the Fitzpatrick scale

A

type 6

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9
Q

what causes Erythopoietic protoporhyria (EPP)

A

deficiency in ferrochelatase

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10
Q

what type of mutation causes Erythopoietic protoporhyria (EPP)

A

X-linked dominant

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11
Q

what are the symptoms of Erythopoietic protoporhyria (EPP)

A 
acute photosensitivity 
swelling
pain
mild anaemia
hepatobiliary disease (gallstones, liver failure)
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12
Q

what can repeated exposure to the sun in Erythopoietic protoporhyria (EPP) cause

A

scarring
hyper or hypo-pigmentation
lichenification

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13
Q

what is the treatment of Erythopoietic protoporhyria (EPP)

A

spectrophotometer
quantitative RBC porphyrins
fluorocytes
transaminases

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14
Q

what is the treatment of Erythopoietic protoporhyria (EPP)

A 
genetic counselling
LFTs and RBC porphyrins every 6 months
photo-protection measures
UVB phototherapy
anti-oxidants
iron avoidance
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15
Q

what is Porphyria cutanea tarda (PCT)

A

the most common type of porphyria

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16
Q

what causes Porphyria cutanea tarda (PCT)

A

mutation in uropophyrinogen decarboxylase. responsible for the 5th step in harm production

17
Q

what are the symptoms of Porphyria cutanea tarda (PCT)

A 
onychosis
blistering/erosion of exposed skin 
hyperpigmentation 
hypertrichosis 
solar urticarial 
hardened patched skin (morphea)
liver problems
18
Q

what are the investigations for Porphyria cutanea tarda (PCT)

A

woods lamp

spectrophotometer

19
Q

what is the treatment of Porphyria cutanea tarda (PCT)

A

establish and treat underlying cause

relieve skin discomfort

20
Q

what causes acute intermittent porphyria

A

deficiency of porphobilinogen deaminase

21
Q

what are the symptoms of acute intermittent porphyria

A 
severe and poorly localised abdominal pain 
urinary issues (pain, retention, incontinence)
psychiatric signs
22
Q

what are the investigations of acute intermittent porphyria

A

urine and serum will show raised pophobilinogen

RBC assay

23
Q

what is the treatment of acute intermittent porphyria

A

haemtin and haeme arginate

derm (mine) (11 decks)

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