Dermatopathology: Blistering disorders Flashcards
1
Q
IHC marker for keratinocytes
A
Cytokeratin
2
Q
Cytokeratin is an IHC marker for this
A
Keratinocytes
3
Q
2 IHC markers for melanocytes
A
S100, HMB45
4
Q
2 IHC markers for Langerhans cells
A
S100, CD1a
5
Q
IHC marker for nerves
A
S100
6
Q
2 IHC markers for vessels
A
Actin-muscle, CD34-endothelium
7
Q
Flat, non-elevated, circumscribed, <5mm
A
Macule
8
Q
Flat, non-elevated, circumscribed, >5mm
A
Patch
9
Q
What is the difference between a macule and a patch?
A
Both are flat, non-elevated, and circumscribed
Macule is <5mm
Patch is >5mm
10
Q
Elevated lesion, <5mm
A
Papule
11
Q
What is the size of a macule?
A
<5mm
12
Q
What is the size of a patch?
A
> 5mm
13
Q
Elevated lesion, >5mm
A
Nodule
14
Q
What is the difference between a papule and nodule?
A
Both are elevated lesions
Papule is <5mm
Nodule is >5mm
15
Q
Elevated, flat-topped lesion, usually >5mm
A
Plaque
16
Q
Fluid-filled cavity, <5mm
A
Vesicle
17
Q
Fluid-filled cavity, >5mm
A
Bulla
18
Q
What is the difference between vesicle and bulla?
A
Both are blisters; fluid-filled cavity
Vesicle is <5mm
Bulla is >5mm
19
Q
Purulent vesicle or bulla
A
Pustule
20
Q
Dry, flaky area of impaired cornification on skin surface
A
Scale
21
Q
Dried collection of blood, serum, or pus on skin surface
A
Crust
22
Q
Thickening of skin with increased skin markings due to chronic rubbing/scratching
A
Lichenification
23
Q
Thinned area of skin
A
Atrophy
24
Q
Skin elevation caused by dermal edema
Often itchy, usually resolves quickly
A
Wheal
25
Separation of nail from nail bed
Onycholysis
26
Describes skin lesion that appears in lines of trauma (e.g. scratch lines)
Koebner phenomenon
27
Small distal dilated blood vessels that appear close to skin surface
Telangiectasia
28
A traumatic lesion causing epithelial disruption and bleeding (a deep scratch)
Excoriation
29
Increased thickness of stratum corneum
Hyperkeratosis
30
Hyperkeratosis is an increased thickness of this layer
Stratum corneum
31
Hyperkeratosis with retained keratinocyte nuclei
Parakeratosis
32
Hyperkeratosis with no retention of keratinocyte nuclei
Orthokeratosis
33
Hyperplasia of stratum granulosa
Prominent keratohyalin granules
Hypergranulosis
34
Increased thickness of epidermis
Acanthosis
35
Intercellular edema in epidermis
Desmosomes easily seen due to fluid between epithelial cells
(intercellular bridges often indicate squamous cell involvement)
Spongiosis
36
Loss of intercellular keratinocyte adhesion
Acantholysis
37
Formation of spaces between cells, usually seen in basal cell-basement membrane zone
Vacuolization
38
Vacuolization is usually seen in this zone of the skin
Basal cell-basement membrane zone
39
Abnormal (premature) individual cell keratinization
Dyskeratosis
40
Linear pattern of melanocytic proliferation at dermal-epidermal junction
Lentiginous
41
Lentiginous is a linear pattern of melanocytic proliferation at this
Dermal-epidermal junction
42
Lentiginous is a linear pattern of proliferation of these cells at dermal-epidermal junction
Melanocytes
43
Infiltration of epidermis by inflammatory cells
Exocytosis
44
Complete loss of epidermis and exposure of dermis/subcutis
Ulceration
45
Incomplete loss of epithelium
Erosion
46
What attach suprabasal keratinocytes to each other?
Desmosomes
47
Protein in desmosomes that is more superficial in the epidermis
Desmoglein 1
48
Protein in desmosomes that is found deeper in the epidermis
Desmoglein 3
49
Problem with this structure results in intraepidermal splits
Desmosomes
50
Structure that attaches basal keratinocytes to basement membrane
Hemidesmosomes
51
Problem with this structure results in subepidermal splits
Hemidesmosomes
52
Anchoring filaments that provide stability to the dermal-epidermal adhesion
Collagen VII
53
Blister filled with acute inflammation
Pustule
54
Describes blistering disorders due to loss of keratinocyte intercellular attachment
Pemphigus
55
Most common form of Pemphigus
Pemphigus Vulgaris
56
What age does Pemphigus Vulgaris present?
40-60 year old
57
Bullous disorder with this typical patient:
40-60 year old
Male = female
May be life threatening
Pemphigus Vulgaris
58
What causes Pemphigus Vulgaris?
IgG Autoantibodies against desmoglein 3
59
Pemphigus Vulgaris is caused by IgG autoantibodies against this
Desmoglein 3
60
What type of split is present in Pemphigus vulgaris?
Suprabasal
61
Condition characterized by row of "tombstones"
Pemphigus Vulgaris
(due to basal cells remaining attached)
62
Condition where IgG produces "fishnet" pattern in direct immunofluorescence
Pemphigus Vulgaris
63
Type of bullae in Pemphigus Vulgaris
Flaccid; easily ruptured; painful
64
Phenomenon where a blister expands when pressure is applied
Nikolsky sign
65
Nikolsky sign in Pemphigus Vulgaris
Positive
66
Common initial site of Pemphigus Vulgaris
Oral mucosa
67
Pemphigus Vulgaris affects these sites
Mucosal sites and skin
(oral mucosa, esophagus, skin - head, trunk)
68
Who is more likely to have Pemphigus Vulgaris, male or female?
Equal
69
Bullous disorder caused by IgG autoantibodies against desmoglein 3
Pemphigus Vulgaris
70
Type of Pemphigus that is an endemic in Brazil
Pemphigus Foliaceus
71
Pemphigus Foliaceus involves antibodies against this
Desmoglein 1
72
Type of split in Pemphigus Foliaceus
Subcorneal
73
Is there mucosal involvement in Pemphigus Foliaceus?
No
There is no desmoglein 1 in mucosa
74
Is there mucosal involvement in Pemphigus Vulgaris?
Yes
Oral mucosa is common initial site
75
Pemphigus disorder involving erosions rather than bullae
Pemphigus Foliaceus
76
What causes Pemphigus Foliaceus?
Antibodies against desmoglein 1
77
Bullous disorder caused by antibodies against desmoglein 1
Pemphigus Foliaceus
78
What causes Paraneoplastic Pemphigus?
IgG against pemphigus and pemphigoid antigens
79
Type of Pemphigus caused by IgG against pemphigus and pemphigoid antigens
Paraneoplastic Pemphigus
80
Bullous disorder with same appearance as Pemphigus Vulgaris, but may have band of lymphocytes in dermis
Paraneoplastic Pemphigus
81
Paraneoplastic Pemphigus is most common with this neoplasm
Lymphomas
82
Bullous disorder most common with lymphomas
Paraneoplastic Pemphigus
83
Paraneoplastic Pemphigus can have the same appearance as Pemphigus Vulgaris, but may have a band of these
Lymphocytes in the dermis
84
Blistering disease due to autoantibodies against BPA of hemidesmosomes
Bullous Pemphigus
85
What causes Bullous Pemphigus?
IgG autoantibodies against BPA (Bullous Pemphigus Antigen) of hemidesmosomes
86
Is acantholysis present in Bullous Pemphigus?
No
87
Is acantholysis present in Pemphigus Vulgaris?
Yes
88
The pathogenesis of Bullous Pemphigus involves IgG against BPA of this structure
Hemidesmosomes
89
Split in Bullous Pemphigus
Subepidermal
with eosinophils
90
Bullous Pemphigus involves a subepidermal split with these cells
Eosinophils
91
What shows on direct immunofluorescence in Bullous Pemphigus?
Complement
Linear IgG along dermal-epidermal junction (DEJ)
92
Linear IgG along dermal-epidermal junction is seen on DIF in this blistering disease
Bullous Pemphigus
93
Blistering disease associated with celiac disease
Dermatitis Herpetiformis
94
Dermatitis Herpetiformis is associated with this
Celiac disease
95
Age at presentation in Dermatitis Herpetiformis
Peak teens to 20s
96
Who is more likely to have Dermatitis Herpetiformis, male or female?
Males
(2:1 male to female ratio)
97
Dermatitis Herpetiformis is more common in this population
Scandinavian
98
Blistering disease more common in Scandinavian populations
Dermatitis Herpetiformis
99
Bullous disorder that is an endemic in Brazil
Pemphigus Foliaceus
100
When wheat is digested, this is produced that is deamidated, binds to transglutaminase, and becomes a neoantigen
Gliadin
101
Form of transglutaminase in the intestine
Tissue translutaminase (TTG, TG2)
102
Form of transglutaminase in the skin
Epidermal transglutaminase (eTG, TG3)
103
In Dermatitis Herpetiformis, anti-eTG IgA deposits here
Tips of dermal papilla
104
What causes Dermatitis Herpetiformis?
Anti-eTG IgA deposits at tips of dermal papilla
(complement is fixed, leading to patches of vesicles on erythematous base)
105
Split in Dermatitis Herpetiformis
Subepidermal at tips of dermal papilla
Split is filled with neutrophils (pustules)
106
What is seen on DIF in Dermatitis Herpetiformis?
Granular IgA at dermal papillary tips
107
Granular IgA at dermal papillary tips is seen on DIF in this bullous disorder
Dermatitis Herpetiformis
108
Blistering disorder involving a subepidermal split at tips of dermal papilla
Dermatitis Herpetiformis
109
Blistering disorder involving a subepidermal split filled with neutrophils (pustules)
Dermatitis Herpetiformis
110
Blistering disorder when clusters of vesicles/pustules are formed on an erythematous base
Very pruritic
Dermatitis Herpetiformis
111
Locations on the body where clusters of vesicles/pustules are found in Dermatitis Herpetiformis
elbows, knees, buttocks
112
What causes Epidermolysis Bullosa?
Defective proteins at dermis-epidermal junction results in mechanical fragility
113
Bullous disorder caused by defective proteins at DEJ, resulting in mechanical fragility
Epidermolysis Bullosa
114
Bullous disorder where friction blisters form easily
Epidermolysis Bullosa
115
Form of Epidermolysis Bullosa that is mild with no scarring
EB simplex
116
2 gene mutations in EB simplex
Keratin 14 and 5
117
Most severe form of Epidermolysis Bullosa, forms scars
Dystrophic EB
118
Dystrophic EB is due to defective this
Anchoring filaments (type VII collagen)
119
Split in Epidermolysis Bullosa
Most forms have subepidermal split
120
Bullous disorder characterized by friction blisters
Epidermolysis Bullosa
121
Autoimmune form of Epidermolysis Bullosa
Epidermolysis bullosa acquisita
122
Onset of Epidermolysis bullosa acquisita
Adult onset
123
Bullous disorder where friction blisters are produced at sites of trauma (fingers, feet, oral)
Epidermolysis bullosa acquisita
124
What causes Epidermolysis bullosa acquisita?
IgG autoantibodies against type VII collagen
125
Bullous disorder caused by IgG autoantigens against type VII collagen
Epidermolysis bullosa acquisita
126
Split in Epidermolysis bullosa acquisita
Subepidermal
127
DIF appearance of Epidermolysis bullosa acquisita
Linear IgG along basement membrane
128
Linear IgG along basement membrane is seen on DIF in this bullous disorder
Epidermolysis bullosa acquisita
129
In Dermatitis herpetiformis, the subepidermal split at the tips of dermal papilla are filled with these cells
Neutrophils
130
Bullous disorder with a subepidermal split filled with neutrophils, forming pustules
Dermatitis herpetiformis
131
Bullous disorder with a subepidermal blister with eosinophils
Bullous pemphigus
132
Bullous pemphigus involves a subepidermal blister with these
Eosinophils
