Galderma Chapter 1: Part 2

Front 1

Ornithine Decarboxylase is a marker for what and can be found in which layer of the epidermis?

Back 1

Found in basal cells
-Marker for proliferative activity

Front 2

Name 6 things that simulate the cells in the basal layer

Back 2

1) Trauma 4) Estrogens
2) UV 5) B-agonists
3) EGF 6) Tumor promotors

Front 3

What 3 things inhibit cells in the basal layer

Back 3

1) Protein deprivation in psoriatic skin
2) Retinoids
3) Steroids

Front 4

What 5 proteins are upregulated during hyper proliferative states?

Back 4

1) K6 4)a3b
2) K16 5) Integrin
3) Ki-67

Front 5

What disease is due to decreased lamellar granules?

Back 5

Flegel's Disease

Front 6

This disease is characterized by uniformly abnormal or absent lamellar granules

Back 6

Harlequin Ichthyosis

Front 7

What is the gene defect seen with Harlequin Ichthyosis?

Back 7

ABCA12

Front 8

This type of Ichthyosis is caused by steroid sulfatase missing from lamellar granules

Back 8

X-linked Ichthyosis

Front 9

Prenatal diagnosis of this disease will show, decreased estrogen levels and non-hydrolyzed sulfated steroids in maternal urine.

Back 9

X-linked Ichthyosis

Front 10

Naxos syndrome is associated with what desmosomal protein?

Back 10

Plakoglobin

Front 11

Desmoplakin I/II is associated with this disease

Back 11

Carvajal Syndrome

Front 12

Envoplakin is one of the antigens in this blistering disorder

Back 12

Paraneoplastic Pemphigus

Front 13

All forms of EB Simplex are due to which 3 antigens?

Back 13

1) K5
2) K14
3) Plectin

Front 14

EB Simplex-muscular dystrophy is related to a defect in what protein?

Back 14

Plectin

Front 15

Junctional EB, Herlitz type (lethal) is cause by defects in what protein?

Back 15

Laminin 5

Front 16

Junctional EB, Herlitz type (lethal) is cause by defect in this gene.

Back 16

LAMB3 gene

Front 17

Junctional EB, Pyloric atresia is cause by defects in this protein.

Back 17

a6b4 integrin

Front 18

DEB, Bart's Syndrome and EB acquisita are all caused by defects in this type of collagen.

Back 18

Collagen 7

Front 19

Another name for BPAg1 is...

Back 19

Desmoplakin

Front 20

Another name for BPAg2 is...

Back 20

Type 17 Collagen

Front 21

Desmoplakin I/II is associated with this disease

Back 21

Carvajal Syndrome

Front 22

Envoplakin is one of the antigens in this blistering disorder

Back 22

Paraneoplastic Pemphigus

Front 23

All forms of EB Simplex are due to which 3 antigens?

Back 23

1) K5
2) K14
3) Plectin

Front 24

EB Simplex-muscular dystrophy is related to a defect in what protein?

Back 24

Plectin

Front 25

Junctional EB, Herlitz type (lethal) is cause by defects in what protein?

Back 25

Laminin 5

Front 26

Junctional EB, Herlitz type (lethal) is cause by defect in this gene.

Back 26

LAMB3 gene

Front 27

Junctional EB, Pyloric atresia is cause by defects in this protein.

Back 27

a6b4 integrin

Front 28

DEB, Bart's Syndrome and EB acquisita are all caused by defects in this type of collagen.

Back 28

Collagen 7

Front 29

Another name for BPAg1 is...

Back 29

Desmoplakin

Front 30

Another name for BPAg2 is...

Back 30

Type 17 Collagen

Front 31

The NC16A domain of BPAg2 interacts with which 2 proteins extracellularly?

Back 31

1) a6b4 integrin
2) Laminin 5

Front 32

The Lamina Densa is made of which type of collagen?

Back 32

Type 4 Collagen

Front 33

The Sublamina Densa is made up of which type of collagen?

Back 33

Type 7 Collagen

Front 34

Keratinocytes produce what 2 growth factors for melanocytes?

Back 34

1) beta-FGF
2) TGF-alpha

Front 35

Keratinocytes produce these 3 inhibitory factors for melanocytes?

Back 35

1) IL-1
2) IL-6
3) TGF-beta

Front 36

Oculocutaneous Albinism (OCA) type 1 is caused by a defect in melanin synthesis due to a defect in what?

Back 36

Tyrosinase

Front 37

Oculocutaneous Albinism (OCA) type 2 is caused by a defect in melanin synthesis due to what gene?

Back 37

P-gene

Front 38

Oculocutaneous Albinism (OCA) type 3 is caused by a defect in melanin synthesis due to what gene?

Back 38

TRP-1 gene

Front 39

Langerhans cells will stain positive to what 5 stains?

Back 39

1) CD45
2) S-100
3) CD1a
4) Actin
5) Vimentin

Front 40

Langerhans cells are central to the pathogenic processes of what 4 diseases?

Back 40

1) Atopic Dermatitis
2) Psoriasis
3) Allergic Contact Dermatitis
4) Infections like Leishmaniasis

Front 41

Langerhans cells adhere to keratinocytes cells by what?

Back 41

E-Cadherin

Front 42

Langerhans cells produce what cytokine?

Back 42

IL-1

Front 43

What 4 disease processes involve Langerhans cells?

Back 43

1) Letterer-Siwe Disease
2) Hand-Schuller-Christian dz
3) Eosinophilic Granuloma
4) Hashimoto-Pritzker Disease

Front 44

True or False, Langerhans cell cannot be infected with HIV?

Back 44

False, Langerhans cell CAN be infected by HIV

Front 45

What 2 finding on EM characterize Langerhans cells?

Back 45

1) Kidney shaped nucleus
2) Birbeck granules

Front 46

Name the 5 cell types commonly found in the Dermis

Back 46

1) Fibroblasts
2) Mast Cells
3) Monocytes
4) Phagocytes
5) Glomus cells

Front 47

Collagen constitutes what % of the dry weight of skin?

Back 47

75%

Front 48

What vitamin is a required cofactor for production of collagen?

Back 48

Vitamin C
(C= Collagen)

Front 49

What group of syndromes is characterized by excessive stretchability and fragility of the skin with tendency toward easy scar formation with "fish-mouth" scars?

Back 49

Ehlers-Danlos Syndrome

Front 50

What disorder is caused by abnormal cross linking of collagen due to mutated beta-cystathione synthase?

Back 50

Homocystinuria

Front 51

This AR disorder is associated with collagen fibrillinogenesis

Back 51

Tenascin-X

Front 52

What 6 diseases are caused by defects in Elastin?

Back 52

1) Cutis Laxa
2) Marfan's Syndrome
3) Congenital Contractural Arachnodactyly
4) Pseudoxanthoma Elasticum
5) Buschke-Ollendorf Syndrome
6) Anetoderma

Front 53

Cutis Laxa is associated with a defect in what gene?

Back 53

Fibulin 5 gene

Front 54

Marfan's syndrome is associated with what elastic fiber disorder?

Back 54

Decreased Fibrillin 1

Front 55

Congenital Contractural Arachnodactyly is associated with a mutation in what?

Back 55

Fibrillin 2

Front 56

Pseudoxanthoma Elasticum is associated with calcium deposition caused by what gene defect?

Back 56

ABCC6 Gene defect

Front 57

Buschke-Ollendorf Syndrome is caused by what gene defect?

Back 57

LEMD3 gene defect

Front 58

Decreased desmosine is seen with which Elastin Disease?

Back 58

Anetoderma

Front 59

Name the 2 "CD"s that all phagocytic skin macrophages express in the dermis

Back 59

1) CD11c
2) CD6

Front 60

B2 integrin binds to what to stimulate phagocytosis in dermal macrophages?

Back 60

C3b+

Front 61

What on the surface of macrophages binds to CAM-2?

Back 61

CD11a which = LFA

Front 62

What on the surface of macrophages binds to LCAM?

Back 62

CD11b which = Mac-1

Front 63

Macrophages kill bad guys as part of the immune system by what 5 processes?

Back 63

1) Phagocytic
2) Antigen processing & presenting to naive T-cells
3) Microbiocidal
4) Tumoricidal
5) Secretory (cytokines)

Front 64

Macrophages are microbiocidal through the production and release of what 3 things?

Back 64

1) Lysozyme
2) Peroxide
3) Superoxide

Front 65

What are the 3 types of mast cells in the body?

Back 65

1) T Type (GI and resp. mucosa)
2) TC Type (skin, GI, submucosa
3) C type (skin, lymph nodes)

Front 66

Where is the greatest density of Mast cells found in the skin?

Back 66

Papillary Dermis

Front 67

What 5 diseases are associated with Mast Cells?

Back 67

1) Mastocytosis
2) Solitary Mastocytoma
3) Diffuse Erythrodermic Mastocytosis
4) TMEP
5) UP

Front 68

What type of stem cells are Mast cells derived from?

Back 68

Bone marrow-residing CD34+ stem cells

Front 69

Proliferation of Mast cells depends on what receptor?

Back 69

C-kit receptor and its ligand stem-cell factor (SCF)

Front 70

Mutations in c-kit may result in what 2 diseases?

Back 70

1) Mastocytosis
2) Piebaldism

Front 71

What 4 stains are positive for Mast Cells?

Back 71

1) C-Kit
2) CD34
3) Giemsa
4) Leder

Front 72

What cells produce and store inflammatory mediators in preformed secretory granules?

Back 72

Mast Cells

Front 73

Mast cells produce what IL?

Back 73

IL-8

Front 74

IL-8 is a strong chemotactic factor for what cell?

Back 74

Neutrophils

Front 75

What are the 3 preformed mediators stored in mast cells?

Back 75

1) Tryptase
2) Heparin
3) Histamine

Front 76

Degranulation of Mast cells produces what 4 results?

Back 76

1) Vascular smooth muscle contraction
2) Increased vascular permeability
3) Tissue Edema
4) Recruitment of inflammatory cells

Front 77

What type of reaction do mast cells participate in?

Back 77

1) Immediate-Type Hypersensitivity Reactions

Front 78

What are the 15 Mast cell mediators?

Back 78

Tryptase, Chymase, Cathepsin G, Histamin, Heparin, IL-4, IL-V, IL-3, IL-5, GM-CSF, TNF-alpha, CCL3, Leukotrienes C4D4EF, Platelet activating factor

Front 79

What 2 disease groups are associated with Glomus cells?

Back 79

1) Glomus Tumor
2) Glomangioma

Front 80

Name the 5 proteins in the Plakin family.

Back 80

1) Envoplakin
2) Periplakin
3) Desmoplakin
4) BPAg1
5) Plectin

Front 81

EB Simplex-Dowling Meara is associated with which 2 keratins?

Back 81

1) K5
2) K14

Front 82

Plectin is associated with what inherited disease and what autoimmune disease?

Back 82

Inherited: EB Simplex- Muscular Dystrophy type
Autoimmune: Paraneoplastic Pemphigus

Front 83

BPAg2 is associated what inherited dz and what 2 autoimmune dz.'s?

Back 83

Inherited: GABEB
Autoimmune: EB, CP

Front 84

a6b4 integrin is associated with what inherited dz and what autoimmune dz?

Back 84

Inherited: JEB w/ pyloric atreasia
Autoimmune: b4 integrin ocular CP

Front 85

Laminin 5 is associated with what inherited dz and what autoimmune dz?

Back 85

Inherited: Junctional EB
Autoimmune: CP

Front 86

Collagen 7 is associated with what inherited dz and what 2 autoimmune dz?

Back 86

Inherited: Dystrophic EB- collagenous domain
Autoimmune:
1) EBA -non collagenous domain
2) Bullous LE

Front 87

Granulation tissue primarily contains what type of collagen?

Back 87

Type 3 collagen

Front 88

What do the remaining hair follicles develop during gestations?

Back 88

4-5 months gestation, they develop in a cephalic to caudal direction

Front 89

ORS makes what type of keratin?

Back 89

Trichilemmal keratin

Front 90

Which of the 3 parts of the IRS keratinizes first?

Back 90

Henle's Layer

Front 91

What are the 3 parts of the IRS? Outside --> Inside?

Back 91

Henle-->Huxley-->Cuticle

Front 92

What are the 3 parts of the hair shaft Outside --> Inside?

Back 92

Cuticle-->Cortex-->Medulla

Front 93

What type of hair disorder is seen with chemo?

Back 93

Anagen Effluvium

Front 94

What hair disorder is characterized by abrupt cessation of mitotic activity in rapidly dividing hair matrix cells?

Back 94

Anagen Effluvium

Front 95

Mutation in this gene causes hair to be 30-50% longer than usual.

Back 95

FGF5 gene

Front 96

Mutations in the Hairless gene and Vitamin D receptor causes what?

Back 96

Atrichia

Front 97

These 3 molecules are important for transition from Telogen --> Anagen

Back 97

1) Sonic Hedgehog gene (SHH)
2) Patched gene (receptor for SHH)
3) Beta-Catenin

Front 98

Name 7 things that can cause Telogen Effluvium.

Back 98

1) Stress
2) Endocrine problems
3) Nutritional deficiency
4) Hypervitaminosis A
5) Delivery of baby
6) Fever
7) Drugs

Front 99

Name 7 diseases that are associated with defects of the hair shaft.

Back 99

1) Monilethrix
2) Netherton's Syndrome
3) Pili annulati
4) Menkes Disease
5) Uncombable Hair Syndrome
6) Hereditary Mucoepithelial Dysplasia
7) Nanos Syndrome

Front 100

What disorder is causes the "Beaded Hair" with alternating thick and thin areas?

Back 100

Monilethrix

Front 101

Monilethrix is caused by a defect in this hair keratin.

Back 101

Keratin hHb6

Front 102

This syndrome is presents with trichorhexis invaginatum or "Bamboo Hair"

Back 102

Netherton's Syndrome

Front 103

Defect in what gene causes "Bamboo Hair"?

Back 103

Serine-Protease Inhibitor Gene or SPINK5 gene

Front 104

What disorder causes "Banded Hair"?

Back 104

Pili Annulati

Front 105

This disease causes twisted hair or "Pili Torti".

Back 105

Menkes Disease
(Menkes "Kinky" Hair syndrome)

Front 106

Menkes Disease is caused by a defect in what?

Back 106

Defect in Copper Transporter

Front 107

What do kind of hair do you see with Uncombable hair syndrome?

Back 107

Triangle-shaped hair

Front 108

What 3 symptoms characterize Hereditary Mucoepithelial Dysplasia?

Back 108

1) Red Gums
2) KP
3) Episodic hair loss

Front 109

What disorder causes red gums?

Back 109

Hereditary Mucoepithelial Dysplasia

Front 110

Nanos Syndrome is caused by a mutation in what protein?

Back 110

Plakoglobin

Front 111

What disease is characterized by Wooly hair, hyperkeratosis of the palms & soles, and cardiac arrhythmias?

Back 111

Nanos Syndrome

Front 112

What adnexal gland is under androgenic hormonal control?

Back 112

Sebaceous Glands

Front 113

Name the 5, free sebaceous glands (not associated with hair) found on the body.

Back 113

1) Montgomery's areolar tubercles
2) Fordyce's spots
3) Meibomian Glands
4) Glands of Zeis
5) Tyson's glands

Front 114

What and where are the Glands of Moll?

Back 114

Free standing Apocrine glands on the eyelid margin

Front 115

What is the name for free standing sebaceous glands on the nipple and areola?

Back 115

Montgomery's areolar tubercles

Front 116

Where do you find Fordyce's spots? What are they?

Back 116

Free standing sebaceous glands on the vermillion border of the lips and on the buccal mucosa

Front 117

Where are Meibomian glands located?

Back 117

Deep on the eyelids, embedded in the tarsal plate

Front 118

What are Meibomian glands?

Back 118

Free-standing modified sebaceous glands that from the most exterior (lipid) layer of the tear film, preventing evaporation

Front 119

What is a Chalazion?

Back 119

Inflammation of the Meibomian gland.

Front 120

Where are the Glands of Zeis located?

Back 120

Superficial eyelid margin

Front 121

What are the Glands of Zeis and what function do they serve?

Back 121

Free-standing sebaceous glands, that also contribute to the exterior lipid layer of the tear film

Front 122

What is a Hordeolum?

Back 122

Inflammation of the Glands of Zeis.

Front 123

What do we call the Apocrine glands on the eyelid margin?

Back 123

Glands of Moll

Front 124

What type of secretion do the Glands of Moll exhibit?

Back 124

Decapitation secretion. (apocrine)

Front 125

What is the name for free-standing sebaceous glands on the genitalia?

Back 125

Tyson's Glands

Front 126

Where are Tyson's Glands located?

Back 126

Free-standing sebaceous glands on the genitalia

Front 127

Apocrine glands are innervated by what?

Back 127

Sympathetic fibers

Front 128

Eccrine glands are found everywhere except what 6 areas?

Back 128

1) Vermillion border of the lips
2) Nail beds
3) Labia Minora
4) Glans Penis
5) Clitoris
6) External auditory canal

Front 129

What type of gland is on the vermillion border of the lips?

Back 129

Sebaceous glands, Fordyce spots

Front 130

What 2 types of glands are on the genitalia?

Back 130

Apocrine glands AND
Modified Sebaceous glands (Tyson's Glands)

Front 131

What type of gland is found ON the breast (nipple & areola)?

Back 131

Sebaceous glands, (Montgomery's)

Front 132

What type of gland are the mammary glands, found IN the breast?

Back 132

Apocrine glands (Mammary Glands)

Front 133

What 2 types of glands are around the eyelids?

Back 133

1) Apocrine glands (Moll's)
2) Sebaceous glands
-Meibomian (deep-chalazion)
-Zeis (SF-Hordeolum)

Front 134

What disease is caused by occlusion of apocrine ducts?

Back 134

Fox-Fordyce Disease

Front 135

What disease is characterized by Chromhidrosis (pigmented sweat)?

Back 135

Fox-Fordyce Disease

Front 136

What chemical causes chromhidrosis seen in Fox-Fordyce disease?

Back 136

Lipofuscin

Front 137

What are the 2 types of free nerve endings found in the skin?

Back 137

1) Penicillate Fibers
2) Papillary Nerve endings

Front 138

Which type of free nerve endings are found sub-epidermally in hair-bearing skin?

Back 138

Penicillate Fibers

Front 139

Penicillate fibers detect what 3 things?

Back 139

1) Pain
2) Touch
3) Temperature

Front 140

Where can you find papillary free nerve endings in the skin?

Back 140

At the orifice of the hair follicle

Front 141

What type of free nerve ending is particularly sensitive to cold?

Back 141

Papillary nerve endings

Front 142

Name the 3 corpuscular receptors in the skin.

Back 142

1) Meissner's Corpuscle
2) Pacinian (Vater-Pacini) Corpuscle
3) Krause End bulbs

Front 143

Which corpuscular receptor detects touch and light pressure?

Back 143

Meissner's Corpuscle

Front 144

Which corpuscular receptor is in weight-bearing sites of the body?

Back 144

Pacinian (Vater-Pacini) Corpuscle

Front 145

Meissner's corpuscles are found where?

Back 145

Dermal papilla of digital skin

Front 146

Where can you find Pacinian corpuscles in the skin?

Back 146

In the deep dermis and SQ of weight-bearing sites

Front 147

Which corpuscular receptor detects vibrational stimuli?

Back 147

Pacinian Corpuscles

Front 148

What corpuscular receptor is found in the genitals and lips?

Back 148

Krause End bulbs

Front 149

What group of blistering disorders have a positive DIF in the intercellular space (fish-net pattern)?

Back 149

All types of Pemphigus

Front 150

What immunoglobulin is deposited in the intercellular space in Pemphigus?

Back 150

IgG

Front 151

All types of pemphigus deposit IgG in the intercellular space except 1.

Back 151

Except IgA Pemphigus

Front 152

What 2 types of pemphigus have a positive DIF in the intercellular space AND BMZ?

Back 152

1) Paraneoplastic pemphigus
2) Drug-induced pemphigus

Front 153

What group of blistering disorders have a positive DIF to just the BMZ?

Back 153

Pemphigoid

Front 154

What immunoglobulin is deposited in the BMZ with the pemphigoid group?

Back 154

C3 & IgG
C3 is more intense

Front 155

What 5 disorders will have DIF positive with IgG and C3 in the BMZ?

Back 155

1) BP
2) HG
3) CP
4) EBA
5) Bullous LE

Front 156

DIF in the BMZ with IgG > C3 favors what group of diseases?

Back 156

Pemphigoid

Front 157

DIF in the BMZ with IgG>C3 favors what 2 diseases?

Back 157

1) EBA
2) Bullous LE

Front 158

What disorder will have linear deposition of IgA at BMZ?

Back 158

Linear IgA bullous dermatosis

Front 159

On salt-split skin BP will show deposits on what side of the split?

Back 159

Epidermal side (roof)

Front 160

On salt-split skin EBA will show deposits on what side of the split?

Back 160

Dermal side (floor)

Front 161

What 3 diseases will have deposits on the DERMAL side or Floor on a salt-split skin test?

Back 161

1) EBA
2) Bullous LE
3) CP

Front 162

What disease will show IgG and IgA within dermal blood vessels and the BMZ?

Back 162

Porphyrias
PCT and pseudoporphyria

Front 163

What disease will have positive DIF in the BMZ and in the Papillary Dermis?

Back 163

Dermatitis Herpetiformis

Front 164

What disease has a DIF showing IgA and C3 in the papillary dermis and along the BMZ?

Back 164

Dermatitis Herpetiformis

Front 165

In DH, 100% of biopsies taken from normal appearing skin will show what?

Back 165

IgA deposition

Front 166

Circulating IgG anti-intercellular antibodies in the serum are associated with what 4 diseases?

Back 166

1) PV
2) PF
3) PNP
4) Drug-induced Pemphigus

Front 167

What disease will show circulating IgA Anti-intercellular antibodies?

Back 167

IgA Pemphigus

Front 168

What 2 diseases will show circulating IgG Anti-BMZ antibodies?

Back 168

BP and EBA

Front 169

What disease will show circulating IgA Anti-BMZ antibodies?

Back 169

Adult and childhood forms of Linear IgA disease

Front 170

DH patients can have circulating antibodies to what 3 proteins?

Back 170

1) Anti-gliadin
2) Anti-reticular
3) Anti-endomysial

Front 171

In Discoid LE, where is the best place to biopsy?

Back 171

Oldest, untreated lesion on non-sun-exposed skin

Front 172

DIF showing IgG and IgM along the DE junction leads what diagnosis?

Back 172

Discoid Lupus

Front 173

DIF showing IgG and IgM along the DE junction and basal keratinocytes leads to what diagnosis?

Back 173

SCLE

Front 174

Cytoid bodies on DIF should make you think of what CT disease?

Back 174

Lupus