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176 Cards in this Set

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What is PPAR gamma?
Peroxisome proliferator activated receptor
Member of nuclear hormone receptor superfamily
Thiazolidinediones are PPAR gamma ligands used to control diabetes
May be useful in treatment of metastatic melanoma
May increase risk for heart failure
Initiates gene transcription by forming heterodimer with RXR
What is ingenol mebutate?
Derived from Euphorbia peplus
3 day course has shown equal efficacy for AK's as efudex & aldara
Causes cellular necrosis
What condition has elevated IL22 levels produced by IL22 T cells?
atopic dermatitis

Both psoriasis & AD have increased IL22; in psoriasis IL22 produced by IL17 cells
Efficacy of vaccinations during and after treatment with etanercept
Pneumococcal vaccines: normal Ab response since B-cell mediated
T-cell dependent Ab: tetanus booster; response may not be sufficient, suppressed immune response for at least 6 wks after treatment
No live vaccines: live attenuated zoter or live attenuated flumist
IL18
Produced by macrophages, dendritic cells, keratinocytes
mediator of autoimmunity
Higher serum levels in patients with LE, increase w/ disease activity
Major causative agent of non-bullous CIE in Japan?
ABCA12
Gene mutated in acral peeling skin syndrome?
transglutaminase 5
Gene defect in Leguis syndrome?
SPRED 1
Genetically distinct but similar to NF1
CALM, axillary freckling, macrocephaly
More benign; look for LD, ADHD, speech delay
MC association with CMTC?
body asymmetry (hypotrophy or hypertrophy)
What is posterior reversible encepholopathy syndrome?
HA, visual disurbances, focal neuro deficits, altered mental status
Often assoc wtih HTN, immunosuppression, eclampsia
Can complicate GAS infection in children
Appears 1-3 wks after pharyngitis, 3-6 weeks after impetigo
M types associated with acute post strep GN following pyoderma?
2, 49, 55, 57, 60
(1, 12 when follows pharyngitis)
Initial trigger of inflammation in LPP due to?
abnormal functioning of PPAR gamma
Side effect of thiazolidinediones
Peripheral edema--can lead to CHF
Weight gain
Induces cytochrome P450
Risk factors associated with poor prognosis in primary anaplalstic large cell lymphoma?
age>60
absence of spontaneous remission
mltifocal skin lesions
extensive single-limb involvement & localization on head & neck
Association b/t vitamin D and NMSC
Higher vitamin D, lower NMSC
Risk factors associated with SCC in HS?
HPV-16, smoking, anogenital location in men
NSAID that may have antitumor effects?
Celecoxib
BCC formation linked with COX activity
Trend toward reduced tumor burden in celecoxib treated Gorlin's group
MCC of death in TEN/SJS?
Infection
2 factors in TEN/SJS associated with increased risk for bloodstream infections?
age > 40, BSA>30%
Best skin prep to avoid surgical site infections?
Chlorhexidine gluconate and alcohol had lower overall rate of infections c/w poviodone-iodine
Chromosomal loci identified by FISH to differentiate malignant and benign melanocytic lesions
Nevoid melanoma had clonal abnormalities in chromosomes 6 and 11, most commonly a gain in 6p25
Promising new immunologic therapy for melanoma
autologous melan-a/mart-1 specific cytotoxic t cell clones
Most noteworthy change in 2009 melanoma staging?
Now includes mitotic rate; most significant correlation with survival was identified at threshold of >1/mm3

In one large study, mitotic rate second only to tumor thickness in predicting survival
In 2009 melanoma staging, what pattern of IHC staining can alone define stage 3
detection of melanoma cells in LN by IHC staining alone is now sufficient for stage 3 status
Worrisome features that differentiate atypical spitzoid melanocytic tumors from classic Spitz
minimal to no maturation
absent or few Kamino bodies
mitotic figures present at the base

More than half occurred on extremities, median mitotic rate 3/mm3
Course of animal-type or epitehlioid melanocytomas
Heavily pigmented melanocytic lesion ddx includes epithelioid blue nevus and animal-type malnoma
Indolent course in younger patients with lower risk of SLN positivity, low likelihood of distant mets
Gene defect in severe neonatal cutis laxa associated with impaired pulmonary, GI, GU, MS, and dermal development
LTBP4: latent form of tranforming factor beta binding protein
MC autoimmune ds in pts with HCV
thyroid ds
High prevalence of antibodies agst which infection is seen in pts with B cell NHL
HCV
Gold standard for evaluating venous anatomy
Color duplex ultrasound scanning
Androgenic steroid with fibrinolytic properties; useful in lipodermatosclerosis
stanazolol
potential cause of toxic alopecia in the workspace
borax
Cause of diffuse melanosis from melanoma
Increased alpha-MSH, hepatocyte GF, and endothelin-1
Lab associations with impetigo herpetiformis
hypocalcemia or low serum vitamin D
HPV association with verrucous carcinoma of the foot
HPV 16
Glomangiomas express what?
vimentin
smooth muscle actin
Association with interstitial granulomatous dermatitis with cords and arthritis
ANA
Mutation and TOC in cerebrotendinous xanthomatosis
sterol-27-hydroxylase gene
TOC: chenodeoxycholic acid
Secondary causes of oxalosis
ehtylene glycol poisoining
IV glycerol
ESRD
Most sensitive and specific T-cell deletion marker for diagnosis of MF
deletion of CD7
Earliest known transcription marker for melanocytes?
MITF (microphthalmia transcription factor)
Pigmented papules and nodules on legs in onchocerciasis
Sowda
Main causative organism in gm negative toe web infection
pseudomonas
What molecule targeted by tacrolimus is highly expressed in keloids?
Gli-1
T-cell migration into epidermis
CLA on T cells binds E or P selectin on endothelial cells; LFA-1 on T-cells binds to ICAM1 to allow adhesion; integrins modulate transmigration
MC symptom of cardiac SLE
pericarditis
Symptoms of adult T-cell lymphoma/leukemia
"HOTS"
hypercalcemia
osteolytic bone lesions
T-cell leukemia
splenomegaly
CD3+/CD4+/CD7-
caused by HTLV1
Gold standard for detecting autoAb in chronic urticaria
basophil histamine release assay
MC location for solitary reticulohistiocytoma
head/neck
Diagnostic tests for leish
Montenegro test, kinetoplast PCR, culture with Nicolle-Novy-Macneal medium
Only proven tx for trichotillomania in controlled studies
clomipramine

Newer anecdotal reports of N-acetylcysteine
Medium of excimer laser
xenon-chrloride
308nm
apparent association with chronic pigmented purpura
odontogenic infections
Potent vasodilator in normal skin
PGE2
Best criteria to diagnose Sezary syndrome
> 1000 Sezary cells/mm3 on Sezary prep
Best treatment for chronic paronychia
topical steroids
Retinoid receptors targeted by tretinoin and adapalene
Tretinoin: RAR alpha, beta, gamma
Adapalene: RAR beta, gamma
MC SE of spironolactone
menstrual irregularity
MC hair manifestation in IP
vertex alopecia
MC extracutaneous manifestation of IP
partial anodontia (also the MC dental abnormality)
Gene defect in anhidrotic ectodermal dysplasia with immunodeficiency
NEMO
Cofactor for gamma-glutamyl carboxylase
Vitamin K (phytomenadione)
MCC of tinea corporis gladiatorum
T tonsurans (40%), T rubrum (40%)
Head/neck/arms MC location
What is Riga Fede disease
benign ulcerative granulomatous process occurs as result of repetitive trauma on oral mucosa by front teeth
Sign of developmental anomaly/neuro disorder
MC location for desmoplastic melanoma
sun exposed areas of head/neck
>50% are amelanotic
Exaggerated mental crease
PXE
Edema of upper eyelids may be presenting sign of what?
graves disease
Treatment for mucocutaneous L. braziliencis
IV sodium stibogluconate
Cutaneous manifestation of multiple myeloma
follicular spicules and multiple ulcers
Location for primary mucinous carcinoma
Usually around eyelids
MCC of fixed drug eruption
sulfonamides
MCC of fixed drug eruption on genital mucosa
cotrimoxazole
MCC of fixed drug eruption on the lips
naproxen/oxicams
MC location for fixed drug eruption from dipyrone
trunk and extremities
Describe how CO2 is carried?
7% in plasma

23% in hemoglobin

70% in form of bicarbonate ions
MC smallpox vaccination complication
autoinoculation
Independent serologic markers seen in amyopathic dermatomyositis
anti-p155, anti-Se
Cutaneous finding associated with increased incidence of malignancy in DM
vasculitis
Infection associated with PLEVA
Strep pharyngitis
Tx with UVB and e-mycin
Study to differentiate ACD from ICD
reflectance confocal microscopy
MOA for low dose UVA1 in acrosclerosis
Induction of collagenase, reduction of collagen production
Keloids show increased levels of epidermal expression of what?
VEGF
SE of gemcitabine
scleroderma-like changes
HPV associated with disseminated large cell acanthoma
HPV6
Treatment for glossodynia
Olanzapine
What is exacerbated by interferon alpha treatment of HCV?
psoriasis
COX2 inhibitor that can cause purpuric eruption, F, malaise
Valdecoxib
Neurologic cause of anogenital pruritus
degenerative changes in lower spine, treated effectively with paravertebral blocks
Other causes: contact, LP, fungus, perianal fissure
Hoarseness due to excess granulation tissue on vocal cord can be SE of what med?
isotretinoin
Macromelanosomes are seen in what ds
NF-1
Endocrine abnormality in NF-1
autoimmune thyroiditis
Most specific eye finding in NF-1
Lisch nodule (as opposed to optic glioma)
Gene defects in congenital generalized lipodystrophy
AGPAT2, seipin (BSCL2)
treat with leptin replacement
MC systemic cause of complex aphthosis
IBD
Polymorphism in what enzyme is associated with androgenetic alopcia
ornithine decarboxylase
MC lymphoproliferative d/o associated with sarcoid
Hodgkin's
Ds associated with HCV patients on ribavirin and IFN alpha
sarcoidosis
IFN alpha likely culprit
Increase of what receptor is seen in HTLV/adut T cell leukemia/lymphoma
Increased IL2 receptor expression (CD25)
Vaccinations using aluminum hydroxide as an adjuvant may cause what?
cutaneous lymphoid hyperplasia
4 common skin findings in Proteus
epidermal nevi (develop in 1st month of life), vascular malformations, subcutaneous lipomas, cerebriform CT nevi
Gene involved in AD transmission of DSAP
SSH1, ARPC2: both play a role in assembly and disassembly of actin filaments
Most reliable method for diagnosis of cutaneous leisch
PCR
Persistent cutis marmorata associated with what 3 disorders?
trisomy 18
Downs
Cornelia de Lange
Two drugs to cause livedo
amantidine, minocycline
MC associated disease with EBA
Crohns
Useful markers of disease activity in PAN
serum beta-FGF, VEGF
Triad of Felty's syndrome
arthritis, leukopenia, splenomagaly
Also see therapy resistant leg ulcerations
Develops in 1% of RA patients
MC nail finding in RA
onychorrhexis
Antiviral protein expressed in pityriasis lichenoides, VZV, and cytotoxic CD8 T cell lymphoma of skin
MxA
Association with ptergium inversum unguis
Stroke
Halo nevi are typical finding in which syndrome
Turner's
Fava-Neto reaction to diagnose what?
Paracoccidioidomycosis
Immunologic reaction to polysaccharide antigens of paracocci cell
Elevated levels of what in men with mild to severe acne
serum 17-hydroxyprogesterone levels
Low levels of what are associated with risk of melanoma progression/poor prognosis
coenzyme Q
also low in pancreatic, breast, lung CA
MC skin d/o in HIV patients
folliculitis
Window glass blocks which UV wavelength?
Blocks UVB
Transmits UVA and visible light
Protein expressed in human scalp anagen but weak in catagen
heat shock protein-27
HLA associated with vulvovaginal gingival syndrome
Severe variant of lichen planus
HLA DQB1*0201 0201 present in 80%
Most sensitive and cost effective method of diagnosing onychomycosis
KOH with chlorazol black
PAS stain was most sensitive but least cost effective
Virus associated with malignancy in immunosuppressed
EBV
Associated with hypermethylation of p16
Associations with childhood lipodystrophy
Autoimmunity (MC: DM or JRA)
Rare but characteristic SE of glatiramer acetate (used for MS)
Localized panniculitis
MOA: stimulates production of myelin basic protein
Syndromes associated with pheochromocytoma
NF1
VHL
MEN2
Carbamazepine-induced SJS/TEN in Han Chinese
HLA-B1502
Combination of HLA B5701, HLA DR7, and HLA DQ3 is 100% predictive of hypersensitivity reaction to?
abacavir
Retinoic acid-induced capillary leak syndrome associated with which d/o
acute promyelocytic leukemia
psoriasis
PRP
MC abnormality with CMTC
limb atrophy
Good prognostic factor in primary cutaneous B cell lymphoma
bcl6
Poor prognostic factors in primary cutaneous B cell lymphoma
bcl2, mum1, oct2
Increased expression of what in Spitz nevus vs melanoma
p16, p27
MC oral finding in TS
dental pits
In kids, antiphospholipid syndrome most commonly secondary to what?
SLE
MC skin finding in antiphospholipid syndrome
livedo reticularis
Keratin decreased in circumscribed acral hypokeratosis
K2e
Common dermoscopic finding of DF
linear irregular crypts
homogenous bluish pigmentation (c/w hemosiderotic variants)
First line empiric tx for M. Marinum
Clarithromycin

JAAD Sep 07
Photodermatosis signicantly higher in AfAm c/w Caucasians
PMLE

C/w Caucasians porphyria and solar urticaria rarely occur in AA
MC site for HSV1 latency and HSV2 latency
HSV1: trigeminal nerve
HSV2: sacral ganglia
Exam of choice for HSV encephalitis
PCR
Alternative therapy for calciphylaxis
Bisphosponates
Causes of carotenoderma
Hypothyroidism, DM (impaired conversion of beta-carotene into retinol)
Kidney dysfunction decreased excretion
Liver dysfunction: impaired conversion of beta-carotene into retinol
Causes of xanthoderma besides carotenoderma
Multiple myeloma
quinacrine, phenol, methylenedianiline, dipyridamole ecess, dihydroxyacetone
Infections associated with IL12 and IL23 deficiency
Salmonella, Mycobacterium
Addition of what med to valayclovir may decrease incidence of post-herpetic neuralgia
gabapentin
MC topical antiobiotic to cause anaphylaxis
bacitracin
MCC SJS/TEN
allopurinol
Biologic dressing for wounds with light to moderate exudates
hydrocolloids, foams (malodorous)
Biologic dressing for dry/painful wounds
hydrogel
Biologic dressing for wounds with moderate to heavy exudates, bleeding wounds
alginates, hydrofibers
What is Galli-Galli disease?
rare acantholytic variant of Dowling-Degos disease with reticulate hyperpigmenation on flexures and scaly erythematous papules on flexures and trunk
AD, K5 mutation
Gene defect in infantile systemic hyalinosis
AR, capillary morphogenesis protein 2 (CMG2)
Joint contractrues, osteopenia, short starture, protein losing enteropathy, gingival hypertrophy, increased infections
Perianal nodules, pearly papules on face
Clinical features of Merkel cell CA (AEIOU)
asymptomatic, expanding rapidly, immune suppression, older than 50, UV-exposed area in fair skinned person
Marker for advanced MF
CA27.29
80% of peds pts receiving this med (in addition to etoposide and cyclophosphamide) develop erythema that progresses to desquamation and hyperpigmentation
thiotepa
MC location for primary cutaneous adenoid cystic carcinoma
scalp
Reservoir for Leishmania mexicana in Texas
burrowing wood rat (Neotoma micropus)
Fetal risk with PR in pregnancy
Premature delivery
Neonatal hypotonia
Possibly fetal death, esp in 1st 15 wks
Peak UV absorption for ecamsule
345nm
Peak UV absorption for avobenzone
360nm (true UVA filter)
Clinical features in H-syndrome
cutaneous hyperpigmented, hypertrichotic, indurated patches associated with hearing loss, short stature, cardiac anomalies, HSM, scrotal masses, hypogonadism
HPV subtypes found in some nevus sebaceus
6, 16, 33
MCC drug-induced DM
hydroxyurea
other common causes: statins, cyclophosphamide
Sunscreens with higher level of UVA protection c/w other sunscreens
avobenzone, octocrylene
Cardinal feature of juvenile PRP
palmoplantar hyperkeratosis
MCC sepsis in TEN
S. aureus, Pseudomonas
MC malignancy associated with hypertrichosis lanuginosa acquisita in men vs women
Men: lung #1, colorectal #2
Women: colorectal #1, breast or lung #2
Causes of acquired ichthyosis
MC malignancy: Hodgkin's lymphoma
Exhaustive list including NHL, MM, meds (statins, cimetidine, clofaz), inflammatory ds (SLE, DM), nutritional ds
preferred test for HSV encephalitis
PCR
Treatment for febrile ulceronecrotic Mucha Habermann disease
Methotrexate
Catharsis
Emotional release. hypothesis maintains that "releasing" aggressive energy (through action or fantasy) relieves aggressive urges.
At what level does trichilemmal keratinization begin?
isthmus (no granular layer formed)
Disorders associated with trichorrhexis nodosa
1) arginosuccinic aciduria: arginonsuccinate lyase deficiency, low arginine, high citrulline
2) citrullinemia: argininosuccinic acid synthetase deficiency in type 1, SLC25A13 mutation in type 2;
Gene mutation associated with non photosensitizing variant of TTD?
C7orf11
Gene mutation in Bjornstad syndrome
BCS1L Gene: encodes an ATPases necessary for assembly of complex III in the mitochondria
Pili torti (scalp only), sensorineural deafness by age 4
Gene defect in woolly hair and skin fragility syndrome not associated with cardiac abnormalities?
desmoplakin
Solid tumor MC associated with Costello syndrome
rhabdomyosarcoma
HRAS mutation
Sparse, curly hair
Growth deficiency
MR
Coarse facies
Outgoing Personality