Allergy, Immunology, Derm

Question 1

Cold Abscesses

Answer 1

Hyper IgE Syndrome

Question 2

Hyper IgE Syndrome Genetic Mutation

Answer 2

STAT3

Question 3

Hyper IgE Syndrome - Features

Answer 3

eczema, scoliosis, hyper-extensibility

cold abscesses, retained primary teeth, pneumatoceles

Question 4

Type 3 Hypersensitivty Reaction

Answer 4

Immune-Complex

Question 5

Type I Hypersensitivity Reaction

Answer 5

immediate rxn: anaphylaxis, hives/urticaria, allergic rhinitis/asthma

Question 6

Type 2 Hypersensitvity Reaction

Answer 6

Cytotoxic, IgG or IgM mediated

Question 7

Examples of Type 2 Hypersensitivity Reactions

Answer 7

AIHA, thrombocytopenia, leukopenia
Good Pasture Syndrome
Myasthenia Gravis

Question 8

Examples of Type 3 Hypersensitivity

Answer 8

Serum Sickness, Arthrus Reaction

Autoimmune diseases

Question 9

Type 4 Hypersensitvity Reactions

Answer 9

Delayed-type hypersensitvity

i.e: PPD, some asthma phenotypes

Question 10

Duncan Syndrome

Answer 10

X-linked lymphoproliferative disease

several or fatal reactions with EBV

Question 11

Leukocyte Adhesion Deficiency - Symptoms

Answer 11

delayed umbilical cord separation
omphalitis
severe gingivitis
skin lesions: cigarette paper scarring

Question 12

LAD Type 1 diagnosis

Answer 12

CD18 by flow cytometry

Question 13

Hyper IgM Syndrome

Answer 13

normal/high IgM, low IgA, IgE, IgG
recurrent infections, remember PCP
CD40 ligand

Question 14

Serum Sickness, timeline for presentation

Answer 14

6-12 days after exposure

Type III hypersensitivity

Question 15

DRESS syndrome, timeline for presentation

Answer 15

2-8 weeks after exposure

Question 16

Antibody found in secretions

Answer 16

IgA

Question 17

When do infants produce their own IgG?

Answer 17

2-3 months of age

Question 18

Transient Hypogammaglobulinemia of Infancy

Answer 18

normal/variant production, will usually produce normal IgG levels by 3-4 years
no treatment unless recurrent severe infections (give IVIG)

Question 19

Non-IgE reactions, common agents

Answer 19

NSAIDS, contrast, ASA

Question 20

Chronic granulomatous disease - Features

Answer 20

X-linked
Recurrent abscesses
S aureus, Serratia, Burkholda, Aspergillus

Question 21

Chronic Granulomatous Disease - Testing/Defect

Answer 21

DHR / dihydrorhodamine oxidation

cannot generate the respiratory burst

Question 22

Atopic Dermatitis =

Answer 22

itch that rashes

infants: face, extensor surfaces
kids: flexor surfaces

Question 23

Ataxia-Telangiectasia: inheritance and defect

Answer 23

AR

ATM mutation, DNA breaks

Question 24

Ataxia-Telangiectasia features

Answer 24

ataxia, ocular teleangiectasis

sinopulmonary disease, bronchiectasis

Question 25

Level elevated in Ataxia-Telangiectasia

Answer 25

alpha-1-fetoprotein

Question 26

Ataxia Telangiectasis complication

Answer 26

maligiancy: lymphoma, lymphoblastic leukemia

Question 27

Bloom Syndrome

Answer 27

similar to ataxia teleangiectasis but WITHOUT ataxia immunodeficiency DNA ligase 1 disorder

Question 28

What immunodeficiency associated with prolonged bleeding from circumcision

Answer 28

Wiskott-Alrich Syndrome

Question 29

Types of Hereditary Angioedema

Answer 29

``` low C4 (substrate for C1-INH) type 1: low C1-INH type 2: normal CI-INH ```

Question 30

Food Allergy Mmenmoic in Kids

Answer 30

WEMPS | Wheat, Egg, Milk and Soy, Peanuts, Seafood

Question 31

SCID

Answer 31

eczema, FTT, diarrhea lymphopenia absent thymus

Question 32

SCID - blood products

Answer 32

give irridiated | non-irridiated contains T lymphocytes which can cause GVHD

Question 33

X-linked (Bruton) Agammaglobulinemia

Answer 33

recurrent infections, esp enterovirus | small or absent lymph nodes or tonils

Question 34

Chediak-Higashi Syndrome

Answer 34

partial albinism ataxia giant granules in neutrophils

Question 35

Common Medications for SJS

Answer 35

sulfonamides

Question 36

treatment of linear IgA bullous dermatosis

Answer 36

dapsone

Question 37

X-linked immunodeficiencys

Answer 37

Bruton | chronic granulomatous def

Question 38

DRESS can be associated with re-activation of

Answer 38

HHV-6

Question 39

DiGeorge- abnormality of what cells

Answer 39

T cells

Question 40

Treatment of Group C or G Streptococcal Pharyngitis?

Answer 40

Yes, PCN for symptoms (not ARF)

Question 41

Erythasma

Answer 41

reddish to brown plaques in axillae, groin in obese individuals corynebacterium, rx w/ macrolide

Question 42

X linked recessive icthyosis associated with

Answer 42

GU abnormalities

Question 43

Severe seborrheic dermatitis, consider

Answer 43

langerhans

Question 44

port wine stains of LE, think

Answer 44

Klippel-Treneauy Syndrome

Question 45

Nevus of Ota associated with risk of

Answer 45

ocular and cutaneous melanoma

Question 46

TNPM has what cells?

Answer 46

Neutrophils

Question 47

Large Facial Hemangiomas, associated syndrome

Answer 47

PHACES | posterior fossa abn, hemangioma, arterial abnormalities, cardiac, eye, and sternal defects

Question 48

Incontineta Pigmenti

Answer 48

X-linked dominant blistering that follows line of blaschko delayed eruption of teeth

Question 49

Cells Associated with Erythema Toxicum Neonatum

Answer 49

Eosinophils

Question 50

IgA Linear Bullous Dermatosis Clinical Course

Answer 50

Rx with dapsone | usually resolves by puberty

Question 51

PLEVA

Answer 51

browning/reddish bumps, often misdiagnosed as varicella | Rx with erythromycin

Question 52

Abnormal DHR test

Answer 52

Chronic Granulomatous Disease

Question 53

Absent serum CH50

Answer 53

Complete Deficiency

Question 54

Giant granules in neutrophils

Answer 54

Chiedak-Higashi