Week 7

Question 1

Your patient is a 3 year-old who presents with cervical lymphadenopathy, conjunctival erythema, cracked lips, and swollen hands and feet. Echo shows dilated coronary arteries. What is at the top of your DDx? Name one other buzz word symptom for this disease.

Answer 1

Kawasaki disease. Strawberry tongue is another possible finding.

Question 2

How is Kawasaki disease treated?

Answer 2

IVIG and aspirin (only exception for giving kids aspirin)

Question 3

What size vessels does Kawasaki disease affect (small, med, large)?

Answer 3

Medium

Question 4

What type of topic therapy vehicle is best used to treat xerotic skin? Explain.

Answer 4

Ointments - oil-based, so it provides a layer of occlusion to keep moisture from escaping the skin.

Question 5

What is the fancy word for molecules that absorb photons in the skin?

Answer 5

Chromophores

Question 6

Describe the metabolic pathway for Vitamin D3 synthesis.

Answer 6

7-dehydrocholesterol –> cholecalciferol (in the skin, needs UV) –> 25-dehydroxycholecalciferol in the liver –> 1, 25-dihydroxycholecalciferol in the kidney (active D3)

Question 7

Describe the biology of a sunburn with regards to UVA and UVB.

Answer 7

UVA: penetrates deeper but is less energetic. Biphasic; responsible for immediate sunburn via direct effect on vessels in the dermis –> vasodilation, then peaks again 6 to 24 hours later. Also results in immediate tanning due to direct photo-oxidation of existing melanin.

UVB: more energetic, only penetrates the epidermis. Kills keratinocytes –> inflammatory response. Also triggers synthesis of more melanin. Sunburn from UVB peaks at 12-24 hours after exposure; new melanin synthesis happens 2-3 days after exposure.

Question 8

What gene mutation is associated with basal cell carcinoma?

Answer 8

Ptch gene mutation

Question 9

What gene mutation is associated with squamous cell carcinoma?

Answer 9

p53

Question 10

What gene mutations are associated with melanomas?

Answer 10

CDK2NA and BRAF

Question 11

Your patient is a baby that presents with a bluish-black lumbosacral area. It kinda looks like a bruise. You consider calling CPS, but remember one condition that changes your mind. What are you thinking about?

Answer 11

Dermal melanocytosis - melanocytes are arrested in the cell cycle before they can migrate to other tissues.

Question 12

Your patient has recurrent pyogenic infections, diffuse hypopigmentation, and silvery hair. What is at the top of your DDx?

Answer 12

Griscelli syndrome. Recurrent infections are due to impaired vesicular transport - inability to form phagolysosomes. It also affects melanosome transfer - that’s why there is hypopigmentation and silver hair.

Question 13

Your patient has suffered some burns that are now hyperpigmented. What is this called and why does this happen?

Answer 13

Post-inflammatory hyperpigmentation - from messed up melanosome transfer after the burn injury –> macrophages eat up the melanosomes and stay in the dermis.

Question 14

What is melasma?

Answer 14

Hyperpigmentation due to increased melanin synthesis from increased estrogen and progesterone most commonly seen during pregnancy.

Question 15

What disease is characterized by long-term deposition of colloidal silver salts in the skin, leading to a slate-grey pigmentation?

Answer 15

Argyria

Question 16

Which antibiotic can be deposited in the skin, leading to black-blue discoloration on the shins, in scars, in the thyroid, bones, teeth, and sclera?

Answer 16

Minocycline

Question 17

Name four places on the body that melanocytes migrate to.

Answer 17

1. Skin
2. Iris
3. Leptomeninges
4. Ear

Question 18

Name a topical steroid in strenth class 1, 2, 4, 6, and 7.

Answer 18

1. Clobetasol
2. Flucinonide
3. Triamcinolone
4. Desonide
5. Hydrocortisone

Question 19

What is the difference between absorption spectra and action spectra?

Answer 19

Absorption spectra is the wavelengths absorbed by a chromophore, action spectra is the wavelengths that cause a biologic effect.

Question 20

A pulse dye laser is used to get rid of a capillary malformation on a kid’s face. What chromophore is absorbing the radiation from this laser?

Answer 20

Hemoglobin

Question 21

What molecules are damaged in the process of photoaging aka solar elastosis?

Answer 21

Collagen and elastin in the dermis.

Question 22

What type of UV radiation is emitted by tanning booths?

Answer 22

UVA

Question 23

What is the genetic/molecular defect in patients with xeroderma pigmentosum?

Answer 23

Inability to do nucleotide excision repair to fix pyrimidine dimers from UV radiation

Question 24

How does SLE cause photosensitivity?

Answer 24

Many SLE patients have an early complement protein deficiency, so when keratinocytes die from UVB, the debris floats around for a while –> PRRs bind debris and cause inflammation –> flare up of symptoms.

Question 25

What is Hermansky-Pudlak syndrome?

Answer 25

Autosomal recessive disorder of melanosome transfer. Mutations in HPS gene.

Clinically: hypopigmentation and bleeding (granules in platelets don’t function properly)

Question 26

What is Chediak Higashi syndrome?

Answer 26

Disorder of lysosomal trafficking and phagocytosis. Patients get recurrent bacterial infections and hypopigmentation.

Question 27

In order for a CD8+ cell to kill a tumor cell, it needs to be activated against the tumor cell via ___________ from a DC cell.

Answer 27

cross-presentation

Question 28

Name three things tumor cells secrete/express that inhibit the immune system.

Answer 28

1. TGF-B
2. PD-L1 (inhibitory co-stimulatory molecule that binds to PD-L receptors on T cells)
3. Fas ligand

Question 29

Do some tumors make high levels of TGF-B that recruits Tregs that turn down the immune response?

Answer 29

Yeah

Question 30

Some tumors can turn infiltrating macrophages into _________ macrophages that subsequently promote tumor growth in three ways, resulting in a positive feedback loop. What are those three ways?

Answer 30

M2 macrophage/tumor-associated macrophages:

1. Produce VEGF –> angiogenesis
2. Make MMPs –> ECM invasion
3. Make GFs to promote tumor growth

As the tumor grows, more macrophages come in and help the tumor grow more (positive feedback)

Question 31

What are myeloid-derived cells and what do they do for tumors?

Answer 31

Immature myeloid cells that normally dampen inflammation. They’re found in high numbers in tumors and induce development of Tregs, skew polarization of T cells to Th2, and suppress anti-tumor innate and T cell responses

Question 32

What is a chimeric antigen receptor?

Answer 32

Modified T cell receptors that have been developed to activate T cells in the absence of MHC or co-stimulation - used to educate T cells against tumor antigens in vitro

Question 33

How does tumor vaccination work?

Answer 33

Put a tumor plasmid in a bunch of DCs, put the DCs back in the patient to turn on CD8+ T cells.

Question 34

Which cytokine is given to cancer patients for cytokine therapy?

Answer 34

IL-2

Question 35

Checkpoint blockade therapy is currently approved for use against metastatic ________. What are the specific targets used in checkpoint blockade?

Answer 35

approved for melanoma

CTLA-4 and PD-L1 are targets for blockade

Question 36

Are there serious AEs associated with checkpoint blockade?

Answer 36

For sho (dermatitis, hepatitis, colitis, endocrinopathies)

Question 37

The malar rash seen in SLE patients spares the _______ ______.

Answer 37

nasolabial folds

Question 38

What might the urine of a patient with lupus nephritis look like?

Answer 38

Tea-colored

Question 39

Name the most common causes of mortality in patients with lupus. Name the most common causes of mortality in patients from lupus.

Answer 39

With lupus: infections and premature cardiac disease.

From lupus: Renal and CNS complications.

Question 40

Name six clinical findings that differentiate RA from osteoarthritis.

Answer 40

1. RA usually spares the DIP (OA does not)
2. Ulnar deviation and swan neck deformity in fingers are classic for RA
3. Heberden and Bouchard’s nodes are seen in the DIP and PIP joints in OA patients.
4. Rheumatoid factor suggests RA
5. Presence of rheumatoid nodules
6. RA often affects joints bilaterally, while OA can be unilateral and is usually larger, weight-bearing joints.