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Bologna 80-100 > Ch 87 - Photodermatologic DOs > Flashcards

Ch 87 - Photodermatologic DOs Flashcards

1
Q

Rank UVA/B/C in terms of contribution to sunburn from worst to least?

A

UVC (absorbed by ozone) > UVB > UVA

MA; burned to CCCrisB

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2
Q

7 phototoxic meds?

A 
HANDCrAFTS
hydrochlorothiazide 
amio (pick hyperpigmenting drug)
naproxen
diltiazem (also AGEP), doxy
cipro
ALA, M-ALA, 5-methoxypsoralen
furosemide
thiazide, triazole (voriconazole)
st.john's wart, sulfonyureas

BOTH in phototox and photoallergic; quinidine, sulfonamides

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3
Q

7 photoallergic meds?

A 
Queen puts oxybenzone daily, showers in chlorex, puts musky fragrances on  and loves her DOGs
quinidine/quinine/quinolones (cipro/levoflox)
oxybenzone
chlorhex
fragrances (musk, sandlewood)
diclofenac
oxybenzone
griseofulven 
sulfonamides (also photoxoxic)
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4
Q

2 meds that are photoallergic and phototoxic?

A

sulfonamides and quinidine

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5
Q

4 plant families that cause phytophotoderm?

A

Ape eating cake, sitting on tree roots and demanding more

apicaceae - celery, parsley, parnsnip, fennel, wild rubarb, hogweed
rutacaceae - lemons, limes, oranges, grapefruits, lei flowers
moracea - fig tree
clustacea - st. john’s wort

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6
Q

8 plants that can cause phytophotoderm?

A

celery, parsley, parnsnip, fennel - apicaceae
lemon, lime, orange, grapefruit, lei - rutacea
fig tree - moracea
st. john’s wort - clustacea

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7
Q

5 acute changes in skin post photoexposure?

A 
erythema 
pigment darkeining/delayed tanning
epidermal hyperplasia
langerhan cell function changes, supressor T cells
vitamin D synx

+- photo-onycholysis
+- pseudoporphyria

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8
Q

defn of minimal erythema dose?

A

lowest UVR dose capable of inducing skin erythema

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9
Q

what wavelength causes epidermal hyperplasia?

A

UVB > UVC

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10
Q

wavelength of UVB?

UVA?

A

B: 290-315
A: 315-400 (UVA1: 315-340; 1: 340-400)

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11
Q

wavelength involved in D3 synthesis?

A

UVB @300 nm

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12
Q

most erythmogenic UV?

A

UVB @300 nm (1000x more than UVA)

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13
Q

7 chronic skin changes post UV?

A 
solar elastosis
rhytidines
solar lentigines
ephelides
Fabre Racouchot
erosive pustular dermatosis of scalp
colloid milium
poikiloderma of Civatte
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14
Q

UV that penetrates glass?

A

UVA (basically only thing A does)

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15
Q

what level of skin does UVA penetrate to? UVB?

A

UVA - deep dermis

UCB - epidermis only, thickens

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16
Q

5 investigations for photosensitivity

A 
H&E
ANA (95%+ in SLE) 
SSA/SSB
serum porphyrins
photopatch testing
phototesting to UVA/B/visible
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17
Q

List 5 idiopathic dermatoses, possibly immune mediated?

A 
PMLE
actinic prurigo
hydroa vacciniforme
chronic actinic dermatitis
solar urticaria
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18
Q

4 classes of photodermatoses?

A

idiopathic, possibly immunologically mediated
defective DNA repair/chromosome instability
photo-aggravated dermatoses
chemical and drug induced photosensitivity

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19
Q

7 photoaggravated dermatoses?

A 
Seb Derm #1
Rosacea
Acne
AD
PsO
CTCL
SLE
DM
LP
Darier
HHD
Grovers

what needs phototherapy or flares with sun?

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20
Q

3 clinical elements of poikiloderma?

A

telangiectasia + atrophy + hyperpigmentation

Civatte:
lateral neck & chest, spares perifollicular skin & submental,
light-skinned with cumulative sun exposure

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21
Q

Name of milium linked to sun exposure?

A

colloid milium

neck, face, dorsal hands

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22
Q

finding on scalp from +++ sun exposure?

A

erosive pustular dermatitis of the scalp

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23
Q

tx for erosive pustular derm of scalp?

A

potent topical CS, tacroliums, calcipotriene, oral isotretinoin

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24
Q

path difference in solar lentigo vs ephelide?

A

solar lentigo: rete elongation (think more time so has time to elongate) +- more melanocytes
both increased basal pigmentation

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25
Q

type of UV in PMLE?

A

UVA> UVB

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26
Q

5 morpho types of PMLE (NIB, but on OSCE)

A 
macular
plaque
vesicular
bullous
lichenoid
EM-like
prurigo
itch w/o visible signs
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27
Q

clinical presentation of PMLE?

A

mins-hours of sun (rarely days) -> lasts few days

pruritic papules/vesicles/plaques no scarring

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28
Q

clin of Juvenile spring eruption?

A

papulovesicles on helices (esp boys)

+- rare fever, malaise, h/a, N

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29
Q

H&E of PMLE (5)?

A

epidermal sponge
+++dermal edema
superficial+ deep perivascular and periappendageal lymphohistiocytic dermal infiltrate +- neuts and eos

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30
Q

5 tx for PMLE?

A

photoprotection: broad spectrum sunscreen, hat, clothes
hardening: nbUVB 2-3x/w in spring
start at 50% MED,  by 10-15%
± PO CS – 0.5-1 mg/kg/d x 1/52 w/ photo, once hardened may need 15-20m 12-2 pm @ sun (w/o sunscreen) for rest of season
also <0.5 mg/kg pred 5-7 days on vacay
HCQ

CsA, AZA, MTX, nicotinamide, helioplex, etc (from exam review)

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31
Q

UV in actinic prurigo? presentation?

A 
UVA and UVB
erythematous pruritic papules/nodules 
 face, distal limbs
exposed and * non-exposed *
± lichenification 
± scarring (pitted)
± cheilitis lip (classically lower lip >>> upper, can be the only sx
± conjunctivitis
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32
Q

two unique features of acitnic prurigo

A

cheliitis

conjuctivitis

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33
Q

HLA type in actinic prurigo?

A

HLA-DR4 (as in pemph gest )

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34
Q

H&E of actinic prurigo?

A

PMLE BUT

Ø dermal edema

35
Q

Tx for actinic prurigo?

A

photoprotection
TCS and CI
nbUVB
resistant: thalidomide 50-100 mg QHS x 2-3 weeks (remission), then taper to as low as possible (50g Q2-3d)
careful about teratogenicity and peripheral neuropathy
PO CS, AZA, CsA

36
Q

Actinic prurigo, features to dx from PMLE?

A 
native american
HLE-DR4
sun exposed and NON-exposed
cheilitis, conjunctivitis
may need thalidomide
no dermal edema on H
37
Q

What virus is hydroa vacciniforme a/w?

A

EBV-associated: more severe, spreads to sunprotected
± facial swelling, ulcerated skin lesions, exaggerated rxns to mosquito bites, fevers, leukopenia, thrombocytopenia, transaminitis, HSM, increased NK lymphocytes

38
Q

hydroa vacciniforme clin?

A

symmetric clusters, pruritic/stinging photodistributed macules  papulovesicles/bullae ± hemorrhagic  umbilicates w/hemorrhagic crusts ->varioliform scars (weeks)

39
Q

hydroa tx?

A 
photoprotection (very resistant)
anecdotal: 
bb/nbUVB, PUVA
B-carotene
HCQ, AZA, thalidomide, CsA, fish oil
40
Q

H&E for hydroa vacciniforme?

A

epidermal spongiosis  pathognomonic prominent reticular keratinocyte degeneration “dead keratinocytes floating in water – hydroa” formation of intraepidermal vesicles with fibrin and acute inflammatory cells, confluent epidermal necrosis
EBV RNA + in lymphoid infiltrate

41
Q

popln classically affected in chronic actinic dermatitis?

A

older outdoor workers M

 pre-existing ACD to compositae, plant antigens, fragrances or topical meds or sunscreens (photoACD

42
Q

tx chronic actinic derm?

A

photoprotection
avoid allergens
computer/video screens safe
films that block UVR
topical and intermittent PO CS w/ emollients needed, +- TO CI.
refractory: very low dose PUVA w/initial high dose PO and TO CS, CsA, Aza, MMF

43
Q

cross reactants in chronic actinic

A 
compositae
plant antigens
fragrances
topical meds
sunscreens
44
Q

solar urticaria - prediction?

A

pretty persistnet, 25% resolve in 10yrs

45
Q

solar urticaria - clin?

A

min : whealing ± pruritius/burning > pain : resolve 1-2hr
ONLY sun-exposed areas; occasionally
± anaphylactoid: malaise, nausea, bronchospasm, syncope

fixed SU – always at same site(s)

drug-induced – chlorpromazine, tetracyclines, tar

46
Q

tx for solar urticaria?

A

photoprotection (usu insufficient)
PO Anti-Hist high dose (Cetirizine 40-80) – 1 hour before exposure (50%, effective), graduated UVA/PUVA
resistant: IVIg, omalizumab (anti-IgE), plasmapheresis

47
Q

2 conditions a/w solar urticaria?

A

pretty much all idiopathic photoderms: PMLE, AP,etc

48
Q

2 conditions a/w chronic actinic dermatitis?

A

ACD

photo ACD

49
Q

5 genes for Xeroderma Pigmentosum? What is the primary defect in the disorder?

A

XPA- XPG, ar

impairment in global genomic nucleotide excision repair (GG-NER) – impairment in the removal of DNA damage from any part of genome -> damaged DNA accumulates -> mutations and cancer

50
Q

5 cancer risks of Xeroderma Pigmentosum?

A

AKs, BCCs, SCCs > melanomas

internal malignancy: 10-20x risk

51
Q

type of light are XP patients sensitive to?

A

290-340nm = UVB+UVA2

52
Q

clinical of XP?

A

lentigines by 2yo + xerosis + pigmentary changes => xeroderma pigmentosum

Ocular: photophobia, keratitis, corneal opacification, vascularization
SCC, melanoma, loss of eyelashes
Neuro (20-30% - esp. D old: BAD Girl): hyporeflexia, Sz, deafness (A, D Ø variant)

Internal malignancy: 10-20x risk

53
Q

cutaneous findings of xeroderma pigmentosum (list 4)?

A

photosensitivity
xerosis
pigmentary changes
cancers

54
Q

3 other organs that may be involved in XP?

A

ocular (think OCA)
CNS
hearing
internal malignancy

55
Q

What is DeSanctis-Cacchione Syndrome?

A

severe form of XP (non-specific to type) microcephaly,  intellectual,  growth,  sexual, deafness, ataxia, limb weakness

56
Q

What is best way to screen for CNS involvement in XP?

A

screen via deep tendon reflexes and audiometry to r/o neuro involvement

57
Q

What is the risk of internal organ malignancy in XP? Average patient lifespan? Causes of death?

A

Internal malignancy: 10-20x risk
ave lifespan: 37 yrs
death: skin ca, neuro , internal Ca

58
Q

Tx in Xeroderma Pigmentosum (list 5)?

A

+++ photoprotection  Vit D, Ca supplement
v. regular follow-up
cryo, EDC, excision, 5FU, Imiquimod, surgical excision
PO retinoids (isotretinoin)
Safe to use XR or radiation (Fitz)

59
Q

List 4 sun sensitive (genetic) disorders in DNA Repair?

A

XP
Cockayne
cerebro-oculo-facio-skeletal
trichothiodystrophy

60
Q

genes involved in Cockayne Syndrome? Is it linked with cutaneous cancers (if so, which forms)?

A

ERCC6 – 2/3, ar
ERCC8 – 1/3, ar

note: all photosensitive disorders are ar

61
Q

actual defect in Cockayne syndrome?

A

TC-NER - > nucleotide excision ok, but accelerated aging
ERCC6 – 2/3
ERCC8 – 1/3

62
Q

3 types of Cockayne? Which one has normal life span?

A

CS1- 80% birth -2 yo, short life
CS2 -birth, short life
CS3 -late onset; N lifespan

63
Q

clinical presentation of Cockayne? (Lord of Rings) List 3 non-skin systems affected?

A

NO skin Cas on sun-exposed skin
photosensitivity, pigmented macules
progressive premature aging, thinning hair, dental caries, alopecia, clubbing of nails, acral edema, sunken eyes, prominent ears
cachectic dwarf: thin body, stooped posture, hypogonadism, joint contractures, short stature

CNS: basal ganglia calcification, osteoporosis, retinal degeneration
microcephaly, intellectual impairment, deafness

Ocular: sunken eyes, “salt and pepper” retinitis pigmentosa, cataracts

Ø increased risk of internal malignancy

COCkAyNE – 8 letters = ERCC8, Cachectic dwarfism, Ocular (salt/pepper Retin pigmentosa), Cataracts/Caries, Avoid Sun, Age (progressive premature), Neuropathy (peripheral), Ears (Mickey Mouse), Eyes (sunken)
ave life = 12 yrs

64
Q

Range of Light to avoid? Tx in Cockayne Syndrome?

A

sensitive: UvA, UvB

symptomatic + photoprotection

65
Q

What is the clinical presentation and genes in UV sensitive syndrome?

A

same as cockayne
ERCC6
ERCC8
?mild Cockayne

66
Q

How is UV-sensitive syndrome different from Cockayne?

A

?mild Cockayne – overlap with I and II
photosensitivity, solar lentigines
unclear what light action spectrum is

67
Q

gene involved in Trichothiodystrophy?

A 
ar; NER 
PIBIDS
ERCC2/XPD (95%) = TTD1
ERCC3/XPB (rare) = TTD2
6 types total
1-3 photosensitive, 4-6 NOT photosensitive

MA: still ERC, need 2 for sulfur bonds, or 3 (rare)

68
Q

What does PIBIDS stand for?

A 
photosensitivity
intellectual delay
brittle hair
ichthyosis: collodion baby
decreased fertility
short stature

+ characteristic facies: receding chin, prominent ears, eosinophilia, sideroblastic anemia

69
Q

3 possible hair findings in Trichotiodystrophy?

A

tiger-tail banding (low sulfur content),

trichoschisis (hair splits), trichorrhexis nodosa (bilateral splits), ribboning

70
Q

tx for Trichotiodystrophy?

A

none

71
Q

what disorder combines hairshaft abn and photosensitivity?

A

trichotiodystrophy

ERCC2/ERCC3; ar

72
Q

Trichotiodystrophy is photosensitive disorder, does it have increased risk of malignancies?

A

no

73
Q

Gene for Bloom syndrome? What does it encode? inheritance pattern?

A

ar
BLM (RECQL3) > DNA helicase
Ashkenazi Jews
average= 26yo

ReCeiveQuaLityBLOOMS at least 3

74
Q

Clinical presentation of Bloom (List 3 cutaneous and 4 extracutan)

A

malar (+dorsal) erythema and telangiectasia
CALMs/areas of hypopigmentation
elongated face + malar hypoplasia + prominent nose
recurrent infections esp otic and pulm
> susceptibility to CA
photosensitivity spectrum unk

growth delay/short stature
N intelligence
DM
immune def (IgM/A>IgG): chronic URT/GI
reduced fertility (men sterile)
>: leukemia, lymphoma, GI adenocarcinoma
DM
75
Q

? BLooMMMM

A

BLooMM – Butterfly telangiectasia, Leukemia/lymphoma, iMmune deficiency, decreased IgM, diabetes Mellitus

76
Q

associated disorders with BLOOM ? (CAs and infections)

A 
growth delay/short stature
N intelligence
DM
immune def (IgM/A>IgG): chronic URT/GI
reduced fertility (men sterile)
>>: leukemia, lymphoma, GI adenocarcinoma
>DM
Quadrira
dial configuration in lymphocytes and fibroblasts = diagnostic
77
Q

Gene for Rothmund-Thomson Syndrome? inheritance pattern?

A

ar
RECQL4
N lifespan

RECeive QuaLity ROthes (4)

78
Q

Clinical presentation of Rothmund-Thomson Syndrome? list 5

A 
photodistributed erythema, edema and vesicles – cheeks/face during first few months of life -> poikiloderma of dorsal hands/forearms/buttocks 
sparse hair 
hypoplastic nails
acral keratoses > SCC <5% acral
>> susceptibility to CA

Rothmund Thompson – reduced thumbs

79
Q

Which photosensitive disorder is associated with telangiectasias?

A

bloom syndrome
RECeiveQuaLity blooms, at least 3
RECQL3

80
Q

Which photosensitive disorder is associated with poikiloderma

A

RECeive QuaLity ROTHes,at least 4

RECQL4 - Rothmund Thompson

81
Q

Cancers in Rothmund Thompson?

A

N immune, intelligence, lifespan
short stature/skeletal abnormalities = hypoplastic thumbs/radii/ulnae
chronic diarrhea/vomiting
pituitary hypogonadism +- saddle nose
dental abnormalities, Juvenile cataracts
malignancy: osteosarcoma 10-30%, SCC< 5% acral

82
Q

4 histo types of Grovers?

A
  • Darier-disease-like
  • Hailey-Hailey like
  • Pemphigus-like
  • Acantholysis and spongiosis (eosinophils)
83
Q

5 Tx for Grovers?

A

TCS, pramoxine(itch), calcineurin inhibitors, vit D analogues, oral antihistamines, avoidance of exacerbating factors
Occasionally: oral retinoids, PUVA, UVA1 (“some success”)

Bologna 80-100 (3 decks)

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