Ch 87 - Photodermatologic DOs Flashcards

Question

type of UV in PMLE?

Answer 1

``` macular plaque vesicular bullous lichenoid EM-like prurigo itch w/o visible signs ```

Answer 2

mins-hours of sun (rarely days) -> lasts few days | pruritic papules/vesicles/plaques no scarring

Answer 3

papulovesicles on helices (esp boys) | +- rare fever, malaise, h/a, N

Answer 4

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

Answer 5

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)

Answer 6

``` 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 ```

Answer 7

cheliitis | conjuctivitis

Answer 8

HLA-DR4 (as in pemph gest )

Answer 9

PMLE BUT | Ø dermal edema

Answer 10

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

Answer 11

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

Answer 12

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

Answer 13

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

Answer 14

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

Answer 15

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

Answer 16

older outdoor workers M |  pre-existing ACD to compositae, plant antigens, fragrances or topical meds or sunscreens (photoACD

Answer 17

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

Answer 18

``` compositae plant antigens fragrances topical meds sunscreens ```

Answer 19

pretty persistnet, 25% resolve in 10yrs

Answer 20

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

Answer 21

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

Answer 22

pretty much all idiopathic photoderms: PMLE, AP,etc

Answer 23

ACD | photo ACD

Answer 24

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

Answer 25

AKs, BCCs, SCCs > melanomas | internal malignancy: 10-20x risk

Answer 26

290-340nm = UVB+UVA2

Answer 27

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

Answer 28

photosensitivity xerosis pigmentary changes cancers

Answer 29

ocular (think OCA) CNS hearing internal malignancy

Answer 30

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

Answer 31

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

Answer 32

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

Answer 33

+++ 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)

Answer 34

XP Cockayne cerebro-oculo-facio-skeletal trichothiodystrophy

Answer 35

ERCC6 – 2/3, ar ERCC8 – 1/3, ar note: all photosensitive disorders are ar

Answer 36

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

Answer 37

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

Answer 38

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

Answer 39

sensitive: UvA, UvB | symptomatic + photoprotection

Answer 40

same as cockayne ERCC6 ERCC8 ?mild Cockayne

Answer 41

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

Answer 42

``` 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)

Answer 43

``` photosensitivity intellectual delay brittle hair ichthyosis: collodion baby decreased fertility short stature ``` + characteristic facies: receding chin, prominent ears, eosinophilia, sideroblastic anemia

Answer 44

tiger-tail banding (low sulfur content), | trichoschisis (hair splits), trichorrhexis nodosa (bilateral splits), ribboning

Answer 45

trichotiodystrophy | ERCC2/ERCC3; ar

Answer 46

ar BLM (RECQL3) > DNA helicase Ashkenazi Jews average= 26yo ReCeiveQuaLityBLOOMS at least 3

Answer 47

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 ```

Answer 48

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

Answer 49

``` 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 ```

Answer 50

ar RECQL4 N lifespan RECeive QuaLity ROthes (4)

Answer 51

``` 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

Answer 52

bloom syndrome RECeiveQuaLity blooms, at least 3 RECQL3

Answer 53

RECeive QuaLity ROTHes,at least 4 | RECQL4 - Rothmund Thompson

Answer 54

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

Answer 55

* Darier-disease-like * Hailey-Hailey like * Pemphigus-like * Acantholysis and spongiosis (eosinophils)

Answer 56

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