Allergy, Immunology, Derm Flashcards
Cold Abscesses
Hyper IgE Syndrome
Hyper IgE Syndrome Genetic Mutation
STAT3
Hyper IgE Syndrome - Features
eczema, scoliosis, hyper-extensibility
cold abscesses, retained primary teeth, pneumatoceles
Type 3 Hypersensitivty Reaction
Immune-Complex
Type I Hypersensitivity Reaction
immediate rxn: anaphylaxis, hives/urticaria, allergic rhinitis/asthma
Type 2 Hypersensitvity Reaction
Cytotoxic, IgG or IgM mediated
Examples of Type 2 Hypersensitivity Reactions
AIHA, thrombocytopenia, leukopenia
Good Pasture Syndrome
Myasthenia Gravis
Examples of Type 3 Hypersensitivity
Serum Sickness, Arthrus Reaction
Autoimmune diseases
Type 4 Hypersensitvity Reactions
Delayed-type hypersensitvity
i.e: PPD, some asthma phenotypes
Duncan Syndrome
X-linked lymphoproliferative disease
several or fatal reactions with EBV
Leukocyte Adhesion Deficiency - Symptoms
delayed umbilical cord separation
omphalitis
severe gingivitis
skin lesions: cigarette paper scarring
LAD Type 1 diagnosis
CD18 by flow cytometry
Hyper IgM Syndrome
normal/high IgM, low IgA, IgE, IgG
recurrent infections, remember PCP
CD40 ligand
Serum Sickness, timeline for presentation
6-12 days after exposure
Type III hypersensitivity
DRESS syndrome, timeline for presentation
2-8 weeks after exposure
Antibody found in secretions
IgA
When do infants produce their own IgG?
2-3 months of age
Transient Hypogammaglobulinemia of Infancy
normal/variant production, will usually produce normal IgG levels by 3-4 years
no treatment unless recurrent severe infections (give IVIG)
Non-IgE reactions, common agents
NSAIDS, contrast, ASA
Chronic granulomatous disease - Features
X-linked
Recurrent abscesses
S aureus, Serratia, Burkholda, Aspergillus
Chronic Granulomatous Disease - Testing/Defect
DHR / dihydrorhodamine oxidation
cannot generate the respiratory burst
Atopic Dermatitis =
itch that rashes
infants: face, extensor surfaces
kids: flexor surfaces
Ataxia-Telangiectasia: inheritance and defect
AR
ATM mutation, DNA breaks
Ataxia-Telangiectasia features
ataxia, ocular teleangiectasis
sinopulmonary disease, bronchiectasis
Level elevated in Ataxia-Telangiectasia
alpha-1-fetoprotein
Ataxia Telangiectasis complication
maligiancy: lymphoma, lymphoblastic leukemia
Bloom Syndrome
similar to ataxia teleangiectasis but WITHOUT ataxia
immunodeficiency
DNA ligase 1 disorder
What immunodeficiency associated with prolonged bleeding from circumcision
Wiskott-Alrich Syndrome
Types of Hereditary Angioedema
low C4 (substrate for C1-INH) type 1: low C1-INH type 2: normal CI-INH
Food Allergy Mmenmoic in Kids
WEMPS
Wheat, Egg, Milk and Soy, Peanuts, Seafood
SCID
eczema, FTT, diarrhea
lymphopenia
absent thymus
SCID - blood products
give irridiated
non-irridiated contains T lymphocytes which can cause GVHD
X-linked (Bruton) Agammaglobulinemia
recurrent infections, esp enterovirus
small or absent lymph nodes or tonils
Chediak-Higashi Syndrome
partial albinism
ataxia
giant granules in neutrophils
Common Medications for SJS
sulfonamides
treatment of linear IgA bullous dermatosis
dapsone
X-linked immunodeficiencys
Bruton
chronic granulomatous def
DRESS can be associated with re-activation of
HHV-6
DiGeorge- abnormality of what cells
T cells
Treatment of Group C or G Streptococcal Pharyngitis?
Yes, PCN for symptoms (not ARF)
Erythasma
reddish to brown plaques in axillae, groin in obese individuals
corynebacterium, rx w/ macrolide
X linked recessive icthyosis associated with
GU abnormalities
Severe seborrheic dermatitis, consider
langerhans
port wine stains of LE, think
Klippel-Treneauy Syndrome
Nevus of Ota associated with risk of
ocular and cutaneous melanoma
TNPM has what cells?
Neutrophils
Large Facial Hemangiomas, associated syndrome
PHACES
posterior fossa abn, hemangioma, arterial abnormalities, cardiac, eye, and sternal defects
Incontineta Pigmenti
X-linked dominant
blistering that follows line of blaschko
delayed eruption of teeth
Cells Associated with Erythema Toxicum Neonatum
Eosinophils
IgA Linear Bullous Dermatosis Clinical Course
Rx with dapsone
usually resolves by puberty
PLEVA
browning/reddish bumps, often misdiagnosed as varicella
Rx with erythromycin
Abnormal DHR test
Chronic Granulomatous Disease
Absent serum CH50
Complete Deficiency
Giant granules in neutrophils
Chiedak-Higashi