Vascular diseases of the skin Flashcards
1
Q
What are the two antigens that hold the epidermis to the BM?
A
BPAG1 and BPAG2
2
Q
What is the pathophysiology of bullous pemphigoid?
A
Attack against the BPAG1 and BPAG2
3
Q
What are the three major locations for blisters?
A
- Subcorneal
- Intraepidermal
- Subepidermal
4
Q
What is the direct immunofluorescence tool used to diagnose skin disorders?
A
Abs against patient’s perilesional skin helps diagnose
5
Q
What is indirect immunofluorescence tool to diagnose skin disorders?
A
Circulating Ab in patient’s serum against normal skin
6
Q
What is the most common bullous autoimmune disease?
A
Bullous pemphigoid
7
Q
What is Bullous pemphigoid?
A
Type II hypersensitivity, Blistering skin disease
8
Q
What age range is generally affected with bullous pemphigoid?
A
60-80+
9
Q
Which gender is usually more affected with bullous pemphigoid?
A
Equal
10
Q
What is the pathophysiology of bullous pemphigoid?
A
Autoantibody against antigens BP180 (BPAG1) and BP230 (BPAG2)
11
Q
What are the skin findings with bullous pemphigoid?
A
Urticarial/papular lesions that progress to tense bullae
12
Q
What are the s/sx of bullous pemphigoid?
A
- Urticarial/papular lesions
- Itchy
- bullae arise in normal or erythematous skin
13
Q
What areas of the skin are not affected with bullous pemphigoid?
A
Mucosal surfaces
14
Q
What is the natural history of bullous pemphigoid?
A
Evolves over weeks/months to a generalized bullous eruption
15
Q
Tense bullae arising in tense or erythematous skin = ?
A
bullous pemphigoid
16
Q
What is the major difference in skin involvement between bullous pemphigoid and bullous pemphigus?
A
bullous pemphigoid does NOT involved mucosal surfaces
Pemphigus does
17
Q
What type of hypersensitivity reaction is bullous pemphigoid?
A
Type II
18
Q
What is erythrodermic bullous pemphigoid?
A
Body wide distribution of bullous pemphigoid
19
Q
What is spongiosus?
A
A lot of edema between cells of the epidermis
20
Q
What is the initial histological finding of bullous pemphigoid?
A
Inflammation of the epidermis, with eosinophils surrounding
21
Q
What is the direct immunofluorescence finding in bullous pemphigoid?
A
Linear deposition of IgG and C3 along the BM zone
22
Q
Linear deposition of IgG and C3 along the BM zone = ?
A
bullous pemphigoid
23
Q
What is the treatment for bullous pemphigoid?
A
Systemic steroids or other immunosuppressants
If local, then local steroids
24
Q
True or false: there is often a lot of skin scarring with bullous pemphigoid
A
False–no scarring
25
How do you follow a patient with bullous pemphigoid?
Serum antibodies to BP180 and BP230
26
What is cicatricial pemphigoid?
Rare variant of bullous pemphigoid of the elderly that affects mucosal surfaces, usually of the head and neck.
27
What is the natural h/o cicatricial pemphigoid?
Chronic and recurring course
28
True or false: there is often scarring with cicatricial pemphigoid
True
29
What is the treatment for cicatricial pemphigoid?
Immunosuppressives
30
What should always be done when a patient complains of pruritus anywhere?
Look at mucosal surfaces
31
What are the differences between cicatricial pemphigoid and bullous pemphigoid?
none
32
What is pemphigoid gestationis?
Rare, intensely pruritic dermatosis of pregnancy
33
What are the skin findings with pemphigoid gestationis?
vesicular eruptions on abdomen with sparing of mucous membranes
34
When in gestation does pemphigoid gestationis often occur?
4th-7th month
35
Which ethnicity is usually affected with pemphigoid gestationis?
Whites
36
True or false: pemphigoid gestationis can recur in subsequent pregnancies
True
37
What percent of babies born to mother with pemphigoid gestationis develop the skin disease? What is the prognosis?
5%
| Typically resolves 6 months postpartum
38
What is the management for pemphigoid gestationis?
Systemic prednisone to suppress blisters and relieve itching
39
Bullous lesions affecting the abdomen of a pregnant woman = ?
pemphigoid gestationis
40
Does pemphigoid gestationis involved the umbilicus?
Yes--if not, think of other things
41
What are the histological findings of pemphigoid gestationis?
Same as bullous pemphigoid
42
What is pruritic papules and plaques of pregnancy? How can you differentiate this from pemphigoid gestationis?
Appears in the striae, and is umbilical SPARING
| -no blisters
43
What is the histology like with PUPP?
Dermatitis-like
44
What are the DIF findings in PUPP?
Negative
45
What is pregnancy prurigo?
Non-blistering skin disorder that arises in pregnancy
46
What are the histological findings of pregnancy prurigo?
Dermatitis pathology
47
What are the DIF findings in pregnancy prurigo?
Negative
48
What is the age range and gender that is usually affected with dermatitis herpetiformis?
20-60 year old males
49
What are the skin findings of dermatitis herpetiformis?
- Papules
- Vesicles
- Occasional bullae on an erythematous, often urticarial base
50
Is dermatitis herpetiformis pruritic?
Yes
51
What is the treatment for dermatitis herpetiformis? (2)
- Gluten free diet
| - Dapsone
52
What disease is dermatitis herpetiformis commonly associated with?
Celiac disease
53
Which ethnicity is affected with dermatitis herpetiformis?
Caucasians
54
What area of the body is usually affected with dermatitis herpetiformis, that other skin diseases do not usually affect?
Extensor surfaces of elbow and knees
55
What is the pathogenesis of dermatitis herpetiformis?
Antibodies against tissue transglutaminase (TTG) bing to TTG in the gut, circulate to the skin, and activate PMNs
56
How can you follow the course of dermatitis herpetiformis?
Follow TTG antibodies
57
What are the histological findings of dermatitis herpetiformis?
Subtle subepidermal blisters, filled with PMNs
58
What are the DIF findings of dermatitis herpetiformis?
Granular IgA deposition in dermal papillae
59
What is adult linear bullous dermatosis?
An acquired, autoimmune blistering disease that may present with a clinical pattern of vesicles indistinguishable from dermatitis herpetiformis, or with vesicles and bullae in a bullous pemphigoid-like appearance
60
What is childhood linear bullous dermatosis?
An acquired, self-limited bullous disease that may begin by the time the patient is age 2 to 3 and usually remits by age 13
61
What areas of the body are affected with linear bullous dermatosis?
Trunk and mucous membranes
62
What are the skin findings of linear bullous dermatosis?
Group vesicles ("cluster of jewels")
63
Group vesicles ("cluster of jewels") on the skin = ?
linear bullous dermatosis
64
What is the drug that can cause linear bullous dermatosis?
Vancomycin
65
What are the histological findings of linear bullous dermatosis?
Subepidermal papillae filled with PMNs
66
What are the DIF findings of linear bullous dermatosis?
Linear deposition of IgA along the BM
67
Linear deposition of IgA along the BM on DIF = ?
linear bullous dermatosis
68
What is the most common type of porphyria?
Porphyria cutanea tarda (PCT)
69
What is the enzyme that is defective in PCT?
Uroporphyrinogen decarboxylase
70
What is the enzyme that is defective in AIP? inheritance pattern?
Porphobilinogen deaminase
| AD
71
What are the compounds that build up in PCT? What is the effect of this in the skin?
Uroporphyrins and coproporphyrins
Absorb light to cause a phototoxic effects
72
What hematological disease can predispose patients to PCT?
Hemochromatosis genes
73
What is the protein that is defective in hemochromatosis? How is it defective?
HFE
HFE works to increase the intestinal release of iron into the blood. When HFE is mutated, the intestines perpetually interpret a strong transferrin signal as if the body were deficient in iron. This leads to maximal iron absorption from ingested foods and iron overload in the tissues.
74
What are the environmental exposures that contribute to PCT?
EtOH and estrogens
75
What infectious agent is associated with PCT?
Hep C
76
What is the viral family of Hep C? Genetic makeup? Enveloped?
Flaviviridae
+ssRNA
Enveloped
77
What areas of the skin are usually affected with PCT?
Blisters on light exposed skin, particularly the hands
78
What are the skin findings of PCT?
Tense bullae on normal appearing background
79
Bullae on light exposed areas, (particularly the hands) on normal background of skin = ?
PCT
80
What are the urine findings of PCT?
Fluoresce red
81
What is the histology of PCT
Cell poor subepidermal blisters
82
What are the DIF findings ot PCT?
Superficial blood vessels with circumferential IgG deposition
83
What is the management for PCT?
Total avoidance of EtOH and estrogens
- Phlebotomy to reduce Fe
- Low dose chloroquine
84
What is the MOA of chloroquine?
Causes a toxic buildup of heme in RBCs to kill parasites
85
What is pseudoporphyria?
Photodistributed blisters resembling PCT, but in the absence of serum, urine, or other porphyrin abnormalities
86
In whom does pseudoporphyria usually occur?
Dialysis patients
87
What is the histology of pseudoporphyria?
same as PCT
88
What are the drugs that typically lead to pseudoporphyria?
NSAIDs
| Tetracyclines
89
What is bullous diabeticorum?
Weakness of the BM between the dermis and epidermis leads to bullae
90
True or false: there is often scarring with bullous diabeticorum
False
91
What part of the body is most often affected with bullous diabeticorum?
Legs and feet
92
What are the DIF findings of bullous diabeticorum?
Negative
93
What are the histological findings of bullous diabeticorum?
Often cell poor subepidermal blisters, but variable blisters
94
What is pemphigus vulgaris?
Formation of autoantibodies to the intercellular layers of the epidermis, leading to spontaneous blistering or fissures resulting from minor trauma
95
What is the age range typically affected with pemphigus vulgaris?
40-60
96
Which gender is more commonly affected with pemphigus vulgaris?
Equal
97
What ethnicities are most commonly affected with pemphigus vulgaris?
Jewish/mediterranean
98
What is the natural h/o pemphigus vulgaris?
Chronic course with acute exacerbations and remissions
Fatal if untreated
99
What percent of cases of pemphigus vulgaris have oral mucosal involvement?
50%
100
What are the skin findings of pemphigus vulgaris?
Progression from erythema to blisters to ulceration
| -Blisters are flaccid and arise on normal skin
101
How do you treat pemphigus vulgaris?
Immunosuppressives
102
What is the pathophysiology of pemphigus vulgaris?
Autoantibodies against desmoglein 1 and 3
103
What are desmosomes?
Between cells
104
Where are hemidesmosomes?
Between cell and BM
105
How can you follow the course of pemphigus vulgaris?
DSG1 and DSG3 antibody titers
106
Where are the blisters of pemphigus vulgaris located?
Often start in the oral mucosa
107
Are the blisters with pemphigus vulgaris pruritic or painful?
Not pruritic, but painful
108
What is Nikolsky sign?
Dislodging of epidermis by lateral fingers pressure near lesions, leading to erosions
109
Flaccid vesicles on normal skin = ?
pemphigus vulgaris
110
Tense vesicles on erythematous skin = ?
Bullous Pemphigoid
111
What are the histological findings of pemphigus vulgaris?
Interepidermal blister
112
What are the DIF findings of pemphigus vulgaris?
Cell surface IgG or C3 "chicken wire"
113
What infectious agent causes impetigo? How do they cause it?
- Staph aureus or strep pyogenes
| - toxin that cleaves desmoglein 1
114
What are the skin findings of impetigo?
honey crusted lesions
115
What are the histological findings of impetigo?
Superficial blister with focal acantholysis
116
What are the histological characteristics of friction blisters?
Cell poor blister below the stratum corneum
117
What is Hailey-Hailey disease (benign familial pemphigus)?
AD defect in ATP2C1, a Ca pump leads to impairment of inter-cellular stores of Ca, and decreased cellular cohesion
118
What is the age range that is typically affected with Hailey-Hailey disease (benign familial pemphigus)?
10-40
119
What are the skin findings of Hailey-Hailey disease (benign familial pemphigus)?
Vesicles and bullae replaced by crusting erosions resembling impetigo
120
Where are the vesicles/bullae found on the skin with Hailey-Hailey disease (benign familial pemphigus)?
Flexural and intertriginous areas (axillae, groin, neck)
121
What is the treatment for Hailey-Hailey disease (benign familial pemphigus)?
Tetracycline and topical steroids
122
What is the protein that is defective in Hailey-Hailey disease (benign familial pemphigus)?
ATP2C1
123
What are the histological findings of Hailey-Hailey disease (benign familial pemphigus)?
Acantholysis forming a dilapidated brick wall
124
What is acantholysis?
Loss of intercellular adhesions (e.g. desmosomes)
125
Acantholysis forming a dilapidated brick wall appearance on histology = ?
Hailey-Hailey disease (benign familial pemphigus)
126
What is Darier's disease?
AD defect in ATP2A2--a Ca pump
127
What is the age range that is affected with Darier's disease?
0-20
128
What are the skin findings of Darier's disease?
Multiple discrete scaling papules in seborrheic distribution
129
What is the treatment for Darier's disease? (3)
- Sunscreens
- Avoid rubbing
- Retinoids
130
Where on the skin are blisters with Darier's disease usually found?
```
Chest
Back
ears
Forehead
Scalp
Groin
```
131
Multiple discrete scaling papules in seborrheic distribution = ?
Darier's disease
132
What are the histological findings of darier's disease?
Total epidermal acantholysis
133
What is the age range that is usually affected with Grover's disease? Which gender?
Middle aged and older
| M more than F
134
What causes Grover's disease?
heavy sweating / heat, usually from bedridden patients
135
What are the skin findings of Grover's disease?
Abrupt onset of pruritic crops of reddish, small papules with vesicles and erosions
136
What areas of the body are affected with Grover's disease?
trunk and proximal extremities
137
Abrupt onset of pruritic crops of reddish, small papules with vesicles and erosions
= ?
Grover's disease
138
What is the treatment for Grover's disease?
Topical steroids or phototherapy
139
What are the histological findings of Grover's disease?
Acantholysis
140
Cell poor or cell rich (inflammatory): PCT
Cell poor
141
Cell poor or cell rich (inflammatory): TEN
Cell poor
142
Cell poor or cell rich (inflammatory): Bullous pemphigoid
Inflammatory
143
Cell poor or cell rich (inflammatory): dermatitis herpetifromis
Inflammatory
144
Cell poor or cell rich (inflammatory): linear IgA dermatosis
Inflammatory
145
Cell poor or cell rich (inflammatory): bullous SLE
Inflammatory
146
Subepidermal, Subcorneal, or Suprabasal: pemphigus foliaceus
Sub corneal
147
Subepidermal, Subcorneal, or Suprabasal: SSSS
Subcorneal
148
Subepidermal, Subcorneal, or Suprabasal: impetigo
Subcorneal
149
Subepidermal, Subcorneal, or Suprabasal: herpes
Suprabasal
150
Subepidermal, Subcorneal, or Suprabasal: pemphigus vulgaris
Suprabasal
151
Subepidermal, Subcorneal, or Suprabasal: pemphigus vegetans
Suprabasal
152
Subepidermal, Subcorneal, or Suprabasal: acantholytic disorders
Suprabasal
153
Subepidermal, Subcorneal, or Suprabasal: PCT
Subepidermal
154
Subepidermal, Subcorneal, or Suprabasal: linear IgA dermatosis
Subepidermal
155
Subepidermal, Subcorneal, or Suprabasal: TEN/SJS
Subepidermal
156
Subepidermal, Subcorneal, or Suprabasal: bullous pemphigoid
Subepidermal
