Path Flashcards
1
Q
What is the deficiency in Haemophilia A?
A
Factor VIII
2
Q
What is the deficiency in Haemophilia B?
A
Factor IX
3
Q
What does the APTT, PT and bleeding time do in Haemophilia A?
A
APTT increases
PT normal
bleed time normal
4
Q
Management in Haemophilia A?
A
Avoid NSAIDs and IM injections
Desmopressin (releases FVIII from vessel walls)
IV recombinant/plasma FVIII
5
Q
Management in Haemophilia B?
A
IV Factor IX
6
Q
Type 1 vWD is a qualitative or quantitative deficiency?
A
partial quantitative
7
Q
Type 2 vWD is a qualitative or quantitative deficiency?
A
qualitative
8
Q
Type 3 vWD is a qualitative or quantitative deficiency?
A
Total deficiency!!
9
Q
What does the APTT do in vWD?
A
APTT increases
10
Q
What does the INR do in vWD?
A
INR is normal.
INR is the PT, i.e. the extrinsic pathway, which is unaffected in vWD
11
Q
What can you give in vWD?
A
Desmopressin (increases vWF release)
vWF/FVIII concentrates
12
Q
Causes of disseminated intravascular coagulation?
A
“PTT GONe Mad”
Pancreatitis ???
Trauma
Transfusion (ABO incompatibility)
Gram negatives (i.e. sepsis)
Obstetric Complications (e.g. pre-eclampsia, amniotic fluid embolism or placental abruption)
Nephrotic syndrome???
Malignancy
13
Q
What happens to the following in DIC: PT FDP Platelets Fibrinogen
A
PT (INR) is prolonged
FDP (Fibrin degradation products) are raised. D-Dimer is a FDP so that can be raised too.
Platelets are low
Fibrinogen is low
14
Q
Management of DIC?
A
Treat underlying cause
Consider transfusions, FFP, platelets, cryoprecipitate.
15
Q
What does vitamin K activate?
A
Factors II, VII, IX, X
Protein C + S
16
Q
Causes of DVT?
A
“SICC PT”
Surgery
Immobilisation
Cancer
Contraception
Pregnancy
Trauma
17
Q
What to consider with heparin in antithrombin deficiency?
A
thrombin binds antithrombin to form thrombin-antithrombin complex (TAT) = anticoagulant.
Heparin enhances this binding. Need higher dose of heparin in people with AT deficiency.
18
Q
What clotting factors does protein C inhibit?
A
V and VIII
19
Q
What are the different types of Protein C deficiency?
A
Type 1 = Quantitative (Quan rhymes with 1!)
Type 2 = Qualitative
20
Q
Management of Protein C deficiency?
A
prophylaxis in familial/previous clotters = LMWH, heparin or warfarin
Homozygous protein C defect = supplemental protein C concentrates. Liver transplant is supposedly curative.
21
Q
What’s the inheritance pattern of Factor V Leiden?
A
Autosomal dominant with incomplete penetrance (i.e. not everyone who has the mutation will develop the disease)
22
Q
What happens to APTT in FV Leiden and why?
A
APTT is low
Leiden variant is unable to be degraded by activated Protein C (which usually inhibits FV activity) therefore there is increased clotting.
23
Q
Mechanism of heparin?
A
Potentiates Antithrombin III (AT) and inactivates thrombin as well as coagulation FIX, X and XI.
24
Q
How is LMWH usually adminstered?
A
Subcut OD
25
How is unfractionated heparin usually administered?
IV, loading dose then infusion.
26
Side effects of heparin?
Bleeding
Heparin-induced thrombocytopenia
Osteoporosis
27
What is the antidote for heparin overdose?
Protamine sulfate
28
Mechanism of Warfarin?
Vitamin K antagonist which leads to reduced activation of Factors II, VII, IX and X. Plus proteins C and S.
29
What to use in warfarin reversal?
IV vitamin K
30
Typical target INR for a person on Warfarin?
2.0-3.0
31
Typical INR of a healthy person?
0.8-1.2
32
What investigation to order to definitively diagnose ALL, what are you looking for?
Bone marrow biopsy
Use Wright or Giemsa stain.
Looking for >20-30% lymphoblasts.
33
Management of ALL?
Steroid (prednisolone/dexamethasone) with chemo (vincristine, asparaginase)
CNS prophylaxis too
Hydration plus allopurinol - prevent formation of uric acid and hence tumour lysis syndrome
Antibiotic prophylaxis
Transfusion, if indicated
34
What is the role of consolidation therapy in ALL?
To eliminate clinically undetectable residual leukaemia, hence preventing relapse and the development of drug-resistant cells.
35
What is the goal of maintenance therapy in ALL?
eliminate minimal residual disease (MRD)
36
Complications of ALL and the chemo?
Quite a few, some include:
"PaNTInG": Pancytopenia; Neutropenia; Tumour lysis syndrome; Infertility; Gastrointestinal Toxicity.
37
What is the induction chemotherapy of choice for acute promyelotic leukaemia?
oral tetinoin (aka all-trans-terinoic acid - ATRA)
38
What is the definitive diagnostic method for AML and what are you looking for?
Bone marrow biopsy/aspiration
>20% blast cells
Blast cells with granulated and Auer rod content
39
What is philadelphia chromosome?
Fusion oncogene, BCR-ABL, resulting from t(9;22)
40
In terms of blast cells in peripheral blood/BM, how is the accelerated phase defined?
10-20% blast cells
41
In terms of blast cells in peripheral blood/BM, how is the blast phase defined?
>20% blast cells
42
What are the three phases of CML?
"CAB" like the Fresh Prince took a cab to Philadelphia
Chronic
Accelerated
Blast
43
What cell markers are you looking for in CLL?
CD5, CD19 and CD23 +ve
| CD5 is usually found on T cells but can be expressed on B cells in CLL.
44
What might you see on a blood film in CLL?
Smudge/smear cells
45
What are good prognostic factors in CLL?
Low Zap-70 expression
13q14 deletion
hypermutated Ig gene
46
What are bad prognostic factors in CLL?
11q23 deletion
CD38 +ve
raised LDH
47
The Binet staging system is used for what condition?
CLL
48
The Rai staging system is used for what condition?
CLL
49
What autoantibodies are associated with SLE?
```
Anti-dsDNA
Anti-Sm
Anti-nRNP
Anti-cardiolipin
Anti-Nuclear Antigens
```
50
What autoantibodies are associated with RA?
Anti-CCP
RF
Anti-Nuclear antigens
51
What autoantibodies are associated with Sjogren's syndrome
Anti-Ro
| Anti-La
52
What autoantibodies are associated with Wegener's granulomatosis?
c-ANCA
53
What autoantibodies are associated with Microscopic polyangitis?
p-ANCA
54
What autoantibodies are associated with Diffuse cutaneous systemic sclerosis?
Anti-Scl 70 aka anti-topoisomerase I
55
What autoantibodies are associated with Polymyositis?
Anti-Jo1
56
What autoantibodies are associated with CREST syndrome?
Anti-centromere
57
What autoantibodies are associated with drug induced lupus?
Anti-histone
58
ACR criteria for diagnosing SLE? (mnemonic!)
```
Need 4 out of the 11:
"A RASH POINts MD"
Arthritis
Renal disease (proteinuria, cast cells)
ANA positive
Serositis (pleurisy/pericarditis)
Haematological disorders (haemolytic anaemia, leucopenia, lymphopenia, thrombocytopenia)
Photosensitivity
Oral ulcers
Immunological disorder (Anti-Sm, Anti-dsDNA, Anti-ANA, Anti-cardiolipin, AntinRNP)
Neurological (seizures/psychosis in absence of other diagnosis)
Malar rash
Discoid rash
```
59
What are the antibodies associated with limited cutaneous systemic sclerosis?
ANA
| Anti-centromere/nucleolar patterns
60
What does CREST stand for?
```
Calcinosis
Raynaud's
Esophageal symptoms
Sclerodactyly
Telangiectasia
```
61
What are patients with Sjogren's syndrome at risk of developing?
Non-Hodgkin's Lymphoma
62
Clinical features of henoch schonlein purpura?
Nephropathy (IgA)
Arthritis
GIT disturbances
Extensor surface (arm/legs) rash
63
What 'size' of vasculitis is Takayasu's arteritis?
Large - it affects places like the aorta
64
What 'size' of vasculitis is Giant-cell arteritis?
Large - places like the temporal artery
65
What 'size' of vasculitis is Polyarteritis nodosa?
Medium - renal and lung involvement
66
What 'size' of vasculitis is Kawasaki's disease?
Medium
67
What 'size' of vasculitis is Buerger's disease?
Medium - arteries of extremities
68
What 'size' of vasculitis is Wegener's granulomatosis?
Small
69
What 'size' of vasculitis is Churg-Strauss syndrome?
Small
70
What 'size' of vasculitis is Microscopic polyangiitis?
Small
71
What 'size' of vasculitis is Henoch Schonlein Purpura?
Small
72
What vascular signs might a person with Takayasu's arteritis have on examination?
Absent pulse with bruits and claudication
73
What histopathological findings might you find with Temporal arteritis?
Granulomatous transmural inflammation
Giant cells
Skip lesions
74
What infection is polyarteritis nodosa associated with?
Hep B
75
What might you find on angiography in a patient with polyarteritis nodosa?
microaneurysms
76
What is strongly associated with Buerger's disease?
Smoking
77
What are typical signs and symptoms of Buerger's disease?
Pain and claudication upon walking
| Inflammation and thrombosis affects vessels of hands and feet
78
What appearances might you see on angiograms in patients with Buerger's disease?
corkscrew appearances
| tree root or spider leg appearances
79
What is the triad of findings in a patient with Wegener's granulomatosis?
Upper respiratory tract: sinusitis, epistaxis, saddle nose
Lower respiratory tract: cavitation, pulmonary haemorrhage
Kidneys: crescentic glomerulonephritis
80
What immunological finding would increase your suspicion of Wegener's granulomatosis?
cANCA anti-proteinase3 positive
81
What findings make up Churg Strauss?
Asthma
Allergic rhinitis
Eosinophilia
82
What immunological finding would increase your suspicion of Churg Strauss?
pANCA anti-myeloperoxidase positive
83
What syndrome can you see in microscopic polyangitis?
Pulmonary renal syndrome:
- Pulmonary haemorrhage
- Glomerulonephritis
84
What immunological finding would increase your suspicion of microscopic polyangitis?
pANCA anti-myeloperoxidase positive
85
What age and gender does Hodgkin's lymphoma tend to affect?
M>F
| bimodal age incidence 20-29 and >60 year olds
86
What are the risk factors for Hodgkin's lymphoma?
history of EBV infection
| Family history
87
What are the symptoms of Hodgkin's lymphoma?
Painless lymphadenopathy +/- obstructive symptoms
B symptoms: Fever; lethargy; weight loss; night sweats
Pain in nodes after alcohol
88
What is Pel-Ebstein fever?
A cyclical fever which intensifies and remits over a typically 2 week period, it is associated with Hodgkin's lymphoma.
89
What specific investigations would you order for Hodgkin's lymphoma?
PET/CT scan - establish baseline extent
| lymph node biopsy - characteristic Reed-Sternberg cells
90
How is Hodgkin's lymphoma staged?
Stage 1: one LN region (LN region can include spleen)
Stage 2: two or more LN regions on the same side of the diaphragm
Stage 3: two or more LN regions on opposite sides of the diaphragm
Stage 4: extranodal sites (liver, BM)
A: No constitutional symptoms
B: Constitutional symptoms
91
What treatment can you give for patient's with Hodgkin's lymphoma?
ABVD chemotherapy
- Adriamycin (aka doxorubicin)
- Bleomycin
- Vinblastine
- Dacarbazine
92
Which lymphoma type produces pain after alcohol?
Hodgkin's lymphoma
93
What are the hallmark cells of Anaplastic large cell lymphoma associated with?
CD30
Clusterin
Characteristic golgi staining
Alk-1 protein expression
94
What are the risk factors for adult T cell lymphoma?
Caribbean and Japanese
| HTLV-1 infection
95
What is a risk factor for enteropathy-associated T cell lymphoma?
longstanding coeliac disease
96
What is a risk factor for cutaneous T cell lymphoma?
Mycosis fungoides
97
What are the three main clinical variants of Burkitt's lymphoma? What patient population do they typically affect and where is the site of lymphoma usually?
Endemic - children living in malaria endemic regions. Jaw/facial bone, ileum/cecum, ovaries, kidney, breast.
Sporadic - non-Malaria endemic regions. Jaw less commonly involved, ileocaecal region more so.
Immuno-deficiency - associated with HIV or post-transplant.
98
What typical histological appearance can be seen with Burkitt's lymphoma?
"Starry sky" appearance
99
What age group does diffuse large B cell lymphoma tend to affect?
middle aged and >60
100
What is Richter's transformation?
Conversion of CLL to diffuse large B-cell lymphoma
101
What histological finding can be seen in diffuse large B-cell lymphoma?
"sheets of large lymphoid cells"
102
What genetic factor is mantle cell lymphoma associated with and what is the implication of that genetic factor?
t(11;14) - overexpression of cyclin D1
103
What genetic factor is follicular lymphoma associated with and what is the implication of that genetic factor?
t(14;18) - overexpression of the bcl-2 gene which is therefore anti-apoptotic
104
What histological findings might you see in follicular lymphoma?
"follicular pattern or nodular appearance"
105
What are two risk factors for mucosal associated lymphoid tissue lymphoma?
H. pylori --> gastric MALT lymphoma
Sjogren's syndrome --> parotid lymphoma
106
What is the affected cell type in multiple myeloma?
plasma cells
107
What is paraprotein?
a monoclonal immunoglobulin
108
What are the clinical features of multiple myeloma?
"CRAB"
Caclium high - stones, bones, moans, groans and thirst
Renal failure - plus amyloidosis + nephrotic syndrome
Anaemia + pancytopenia
Bones - pain, osteoporosis, osteolytic lesions, fractures
+hyperviscosity
109
What specific investigations would you order for multiple myeloma?
serum electrophoresis - looking for narrow band
Blood film - rouleaux (RBC stacking)
Urine - Bence Jones protein
ESR - v high
110
What staging system is used for multiple myeloma?
Durie-Salmon
111
What are risk factors for multiple myeloma?
monoclonal gammopathy of undetermined significance (MGUS)
| Obesity
112
What are the values of plasma cells in BM for Multiple Myeloma, Smouldering myeloma and for MGUS?
MM: >10% in BM
MGUS: 10%
113
What would the serum paraprotein levels be for smouldering myeloma and for MGUS?
Smouldering: >30g/L
MGUS:
114
What is the pathological factor that contributes to Waldenstrom's macroglobulinaemia?
IgM that infiltrates LNs and BM
115
What are the symptoms of Waldenstrom's macroglobunlinaemia?
```
weakness
fatigues
weight loss
vision changes
confusion
```
116
How would you treat Waldenstrom's macroglobunlinaemia?
plasmapheresis for hyperviscosity
| chemotherapy
117
What are the 4 C's of Wegener's Granulomatosis?
- cANCA
- corticosteroids
- cyclophosphamide
- crescentic glomerulonephritis
Think of Wegener's as Wecener's!
118
How do Wegener's and Microscopic polyangiitis differ in the regions of the body affected?
Wegener's - upper respiratory tract down to lungs with kidneys
MPA - No upper resp tract but still involves lungs and kidneys
119
Using ANCAs - how can we tell the difference between Wegener's and microscopic polyangiitis?
Wegener's - cANCA
| MPA - pANCA
120
What is different between biopsies in patients with Wegener's and microscopic polyangiitis?
No granulomas formed in MPA unlike Wegener's.
121
How can chronic bronchitis be defined clinically?
Cough and sputum on most days for 3 months over 2 years
122
What are the histological features of chronic bronchitis?
Dilatation of the airways
Goblet cell hyperplasia
Mucous gland hypertrophy
123
Dilatation of the airways, goblet cell hyperplasia and mucous gland hypertrophy are histological features of what condition?
Chronic bronchitis
124
What are Curschmann spirals and what condition do they indicate?
spiral shaped mucus plugs from subepithelial mucous glands - seen in asthma (but also other lung disease)
125
What condition are Charcot-Leyden crystals seen in?
eosinophilic conditions like allergies or asthma
126
What are the characteristic histopathologic findings seen in idiopathic pulmonary fibrosis?
- Interstitial fibrosis in a "patchwork pattern"
- honeycomb change
- fibroblast foci
127
What is the pathogenesis of "honeycomb change"
Hyperplasia of type II pnemocytes causing cyst formation
128
Name the four stages of lobar pneumonia.
1. Congestion - first 24 hours, vascular engorgement, intra-alveolar fluid, neutrophils and bacteria.
2. Red hepatization or consolidation - red cells extravasate into alveolar spaces. Exudate consolidates alveolar parenchyma.
3. Grey hepatization - red cells disintegrate
4. Resolution - exudate cleared
129
What are risk factors for squamous cell carcinoma?
being Male
| smoking
130
What region of the lung does squamous cell carinoma tend to affect?
Proximal bronchi
131
Describe the progression of squamous cell carinoma of the lung beginning with epithelium.
1. Epithelium
2. Hyperplasia
3. Squamous metaplasia
4. angiosquamous dysplasia (B.M. thickening)
5. carcinoma in situ
6. invasive carcinoma
132
What histological features might you see with squamous cell carcinoma?
```
Keratinsation
intercellular prickles (desmosomes)
```
133
A lung biopsy shows keratinisation with intercellular prickles. What could this be?
Squamous cell carcinoma
134
What region of the lung do adenocarcinomas tend to affect?
The peripheral regions
135
how do SCCs and adenocarcinomas compare in terms of when they metastasise in the lung?
```
SCCs = late
adenocarcinomas = early
```
136
What histological appearance do lung adenocarcinomas tend to have?
Glandular differentiation with mucin production
137
What is the most common gene mutation in lung adenocarcinomas?
EGFR
138
What is the typical progression of lung adenocarcinomas, starting from 'atypical adenomatous hyperplasia'.
1. Atypical adenomatous hyperplasia
2. non-mucinous bronchoalveolar carcinoma (BAC)
3. mixed-pattern adenocarcinoma
139
What are the main different types of non-small cell lung cancers?
Adenocarcinoma
Squamous Cell Carcinoma
Large cell carcinoma
140
What are the cytological appearances of the cells in Large Cell Carcinoma?
Large cell size
Higher cytoplasmic to nuclear size ratio
Lack of salt-and-pepper chromatin
141
What histological appearance do large cell carcinomas have?
LCC is a diagnosis of exclusion so they do not have the appearance of other non-SCLCs, i.e. no glandular or squamous differentiation
142
What region of the lung does small cell carcinoma tend to affect?
Central airways, proximal bronchi
143
What cell type do small cell lung cancers arise from?
neuroendocrine
144
What are two paraneoplastic syndromes associated with small cell lung cancer?
SIADH
| Lambert-Eaton myasthenic syndrome (proximal leg+arm weakness relieved through use)
145
What genetic mutations are common in small cell carcinomas?
p53
| RB1
146
p53 and RB1 genetic mutations, common in what type of lung cancer?
small cell lung cancer
147
How do you interpret the following result from a dexamethasone test:
Low ACTH
Cortisol not suppressed by high or low dose dex
Cushing's syndrome (anything but pituitary)
148
```
How do you interpret the following result from a dexamethasone test:
Elevated ACTH (in hundreds)
Cortisol not suppressed by high or low dose dex
```
Ectopic ACTH production, e.g. adrenal/chest/abdo tumour
149
How do you interpret the following result from a dexamethasone test:
Normal or slightly elevated ACTH
Cortisol not suppressed by low dose dex
Cortisol suppressed by high dose dex
Cushing's disease because pituitary still retains some feedback control
150
How can the causes of Addison's disease be categorised?
Adrenal dysgenesis - genetic abnormalities
Impaired steroidogenesis - genetic/enzyme problems
Adrenal destruction - autoimmune/TB
151
What effect does aldosterone have on electrolytes and fluid?
Increase Na and water retention
| Deplete plasma K
152
What is the mechanism behind the metabolic acidosis in Addison's Disease?
Addison's --> low aldosterone --> reduced reabsorption of Na in distal tubule therefore increased H+ retention in serum
153
What test for Addison's disease?
synACTHen test
154
Describe the two tests involved in the synACTHen test
1. Short test: measure cortisol before and after admin IM 250mcg synacthen. 1 hour later cortisol levels should reach designated threshold level ~700nmol/l. If normal, it excludes primary adrenal failure. If abnormal, need to do long test ....
2. Long test: 1mg IM synacthen. Measure cortisol at 1, 4, 8 and 24 hours. Normal should reach 1000nmol/l by 4 hours. In primary adrenal failure = reduced at all stages. In secondary corticoadrenal insufficiency it is delayed but normal response seen.
155
What are the high uptake hyperthyroid conditions?
Graves
Toxic multinodular goitre (uneven uptake)
Toxic adenoma (hot nodule)
156
What are the low uptake hyperthyroid conditions?
```
Subacute DeQuervains thyroiditis (think, 'sub' = below)
Postpartum thryoiditis (think, they'll be tired after labour so too tired to take up)
```
157
A 'hot nodule' on radioactive iodine uptake test indicates what condition?
toxic adenoma
158
What are the autoimmune variants of hypothyroidism?
Primary atrophic hypothyroidism
| Hashimotos thyroiditis
159
What is the normal range for the anion gap?
14-18mmol/l
160
What is the mnemonic for elevated anion gap metabolic acidosis?
"KULT"
- Ketoacidosis
- Uraeamia (Renal Failure)
- Lactic acidosis
- Toxins (ethylene glycol, methanol, paraldehyde, salicylate)
161
Asparate aminotransferase is found in which part of the liver?
Hepatocytes
162
Alanine aminotransferase is found in which part of the liver?
Hepatocytes
163
Alkaline phosphatase is found in which part of the liver?
Bile canaliculi
164
AST:ALT = 1 is associated with what?
ischaemia (CCF and ischaemic necrosis/hepatitis)
165
AST:ALT >2.5 is associated with what?
Alcoholic hepatitis
166
AST:ALT
Hepatocellular damage
Paracetamol overdose with hepatocellular necrosis
Viral hepatitis, ischaemic necrosis, toxic hepatitis
167
What would you expect the AST:ALT to be in alcoholic hepatits?
>2.5 (basically raised)
168
What would you expect the AST:ALT to be in toxic/viral hepatitis?
169
What can cause increased ALP?
Liver - biliary obstruction (e.g. malignancy/infection)
Pregnancy (non-pathological) - released from placenta in late pregnancy
Hyperparathyroidism
Bone - Osteomalacia; bone metastasis; Pagets disease; Deficiency induced Ricket's
Blood type O and B (non-pathological) release from small intestine after fatty meal
and many more!
170
Normal range of AST?
171
Which is more specific for liver damage - AST or ALT?
ALT (It's got 'L' for liver in it)
172
Normal range for ALT?
173
Normal range for ALP?
30-150iu/L
174
Normal range for GGT?
30-150iu/L
175
What dye can be used to measure hepatic perfusion levels?
Indocyanine green
176
What is indocyanine green used for?
Measuring hepatic/splanchnic perfusion
177
What is Bromsulphthalein used for?
A dye used to measure liver function - measuring the rate of removal of the dye.
178
What body system do acute porphyrias usually affect?
Nervous system
179
What are the major symptoms of a porphyria's acute attack?
abdominal pain
vomiting
hypertension
tachycardia
180
What two distinct patterns are there of skin disease seen in the cutaneous porphyrias?
```
Immediate photosensitivity (pain, burning, discomfort but not always visible)
Vesciulo-erosive skin disease (blistering and open sores)
```
181
What enzyme is deficient in Porphyria Cutanea Tarda?
Uroporphyrinogen decarboxylase
182
What enzyme is deficient in Acute Intermittent Porphyria?
Hydroxymethylbilane synthase
183
Which acute porphyrias have skin lesions?
Hereditary coproporphyria
| Variegate porphyria
184
What enzyme is deficient in hereditary coproporphyria?
Coproporphyrinogen oxidase
185
What enzyme is deficient in variegate porphyria?
Protoporphyrinogen oxidase
186
What dietary measures should be taken in an acute attack of porphyria?
High carbohydrate diet, in severe cases a dextrose infusion
187
What are skin features in guttate psoriasis and what is the onset/resolve time?
multiple small scaly plaques - 'tear drops' - on the trunk and arms.
Quick onset following an infection, typically streptococcal throat, which will usually resolve spontaneously completely.
188
Name that derm condition:
Thick, silvery-white scale over well-defined red thickened skin located on the back of the head.
Scalp Psoriasis
189
Treatment for scalp psoriasis?
Shampoos , e.g. coal tar or antifungal shampoos.
Short hair - helps treatment application
Phototherapy
190
What is subungal hyperkeratosis?
Scaling under the nail due to excessive proliferation of keratinocytes in the nail bed and hyponychium
191
What are the two types of plaque psoriasis?
Large plaque psoriasis
| Small plaque psoriasis
192
What are the plaques like in Large plaque psoriasis?
thick, well-demarcated, red with silvery scale.
193
What are the plaques like in Small plaque psoriasis?
numerous small (mm to few cm) thin, pinkish with a fine scale
194
How do Large and Small plaque psoriasis compare in terms of: age of onset; family history; treatment options.
Large plaque: early onset (under 40); positive family history; treatment resistant.
Small plaque: Any age (usually over 40); less relevant family history; responds well to phototherapy
195
How does flexural psoriasis look compared to typical psoriasis?
Usually no silvery scale - instead is shiny and smooth.
Possibly a fissure in skin crease
Still has deep red colour and well-defined borders
196
What health conditions are associated with psoriasis?
```
Psoriatic arthritis
IBD
Uveitis
Coeliac disease
Metabolic syndrome (obesity, hypertension, hyperlipidaemia, T2DM
Pustulosis
```
197
What is Auspitz's sign?
bleeding spots when psoriasis scales are scraped off
198
What is koebner phenomenon?
When skin lesions form at sites of trauma
199
What are the characteristic histological findings in psoriasis?
Rete ridges
Epidermal thickening
Munro's microabscesses
Granular layer loss
Parakeratosis
200
What are Rete Ridges?
epidermal projections that extend into the underlying dermis - seen in psoriasis
201
What is Munro's microabscess?
Abscess in the stratum corneum of the epidermis due to infiltration of neutrophils from papillary dermis - cardinal sign of psoriasis.
202
What is the PASI score?
Psoriasis Area and Severity Index (PASI) score
203
What treatment options are there for a person with psoriasis?
Conservative: stop smoking; drink less; maintain optimal weight.
Medical:
- Topical: emollients, coal tar; salicylic acid; steroids
- Phototherapy
- If severe, methotrexate or ciclosporin
- avoid systemic steroids because of withdrawal flare risk
- biologics like infliximab reserved for severe treatment resistant variants
204
What can precipitate erythrodermic psoriasis?
Infections; low calcium; steroid withdrawal; strong coal tar preparations; excess alcohol
205
Treatment for erythrodermic psoriasis?
Hospitalisation - fluids and temperature regulation
Bed rest
Emollients and wet dressings
Low-dose methotrexate or ciclosporin
Treat complications - Abx, diuretics, etc.
206
What are the six P's of Lichen Planus?
```
Lesions are :
Pruritic
Purple
Polygonal
Papules
Plaques
Mother-of-Pearl sheen
```
207
What are Wickham striae?
fine white lines that cross the plaques in Lichen Planus
208
Where is Lichen Planus usually distributed?
Front of wrists
Lower back
Ankles
209
You look inside the patient's mouth and see white streaks in a lacy pattern which are painless along with painful erosions and inflammation. What is the diagnosis?
Oral lichen planus with erosions
210
What histological appearances might you see with lichen planus?
hyperkeratosis with saw-toothing of rete ridges
211
Rete ridges are histological findings in what condition? and saw-toothing rete ridges?
Rete ridges = psoriasis
| Saw-toothing rete ridges = lichen planus
212
What treatment is there for lichen planus?
topical steroids
| topical retinoids
213
What is the prognosis for lichen planus?
Cutaneous form likely to clearly within a couple of years - unpredictable.
Oral - persist for a decade or so.
214
What can trigger erythema multiforme?
Infection with HSV (commonly 1 rather than 2) or Mycoplasma pneumoniae
Drugs - v. uncommon e.g. penicillins, salicylates.
215
What is the clinical progression of the erythema multiforme skin lesions? What characteristic lesions can be seen? What area of the body does it affect?
erupt within 24 hours and evolve over 72 hours.
demarcated, red/pink macules --> papules --> plaques which eventually develop blistering/crusting.
Target lesions: dusky centre, paler middle ring, outer bright ring.
Start peripherally then to trunk
a/w pruritis
216
Target lesions are associated with what condition?
Erythema Multiforme
217
Who gets Erythema multiforme?
Usually the young adults (20-40s) but anyone can.
| M>F
218
What might a skin biopsy of erythema multiforme show?
apoptotic keratinocytes
| blisters within/under the epidermis
219
What is the treatment for Erythema Multiforme?
Rash usually settles over few weeks
Treat any underlying cause (aciclovir for HSV, erythromycin for M pneumoniae)
Supportive
220
What condition is associated with dermatitis herpetiformis?
Coeliac disease
221
What are the risk factors for dermatitis herpetiformis?
Predominantly caucasian 15-40 year olds
| M>F
222
Dermatitis Herpetiformis has associations with which HLAs?
DQ2 and DQ8
223
What's the pathophysiology of dermatitis herpetiformis?
Dermal autoantibodies similar to those seen in Coeliac
224
What are the clinical features of dermatitis herpetiformis?
Itchy vesicles in groups on the scalp, shoulders, buttocks, elbows or knees.
225
What might you see on histology for dermatitis herpetiformis?
subepidermal bullae
| IgA deposits at tips of dermal papillae
226
What treatment would you give for dermatitis herpetiformis?
Gluten-free diet
| Dapsone (it's a sulphone antibiotic) to reduce itch
227
What causes the bullae in pemphigoid?
IgG binding the hemidesmosomes of the epidermal basement membrane --> subepidermal bullae
228
How do the location of bullae in pemphigoid and pemphigus differ?
Pemphigoid bullae are subepidermal (PemphigoiD = Deep)
Pemphigus bullae are intraepidermal (PemphiguS = Superficial)
229
What are the clinical features of Pemphigoid?
Large tense bullae which rupture --> crusted erosions
Severe itch
Usually affects skin fold areas
230
What histological findings might there be in pemphigoid?
Subepidermal bullae with eosinophils
| Linear deposition of IgG along the basement membrane
231
You see a skin biopsy come back with subepidermal bullae with eosinophils and deposits of IgG. What's the diagnosis?
Pemphigoid
232
What causes the bullae in Pemphigus?
IgG binding desmosomal proteins
233
How do Pemphigoid and Pemphigus differ in terms of how readily the bullae rupture?
Pemphigoid - difficult to rupture
| Pemphigus - easily ruptured
234
Where can the bullae of pemphigus be found?
skin and mucosa
235
What ages tend to get seborrhoeic keratoses?
begin 30s/40s
| Most people over 60 have them
236
Where can seborrhoeic keratoses be found/not found on the body?
Any area of skin except palms, soles and mucous membranes
237
What colour do seborrhoeic keratoses tend to have?
Yellow to brown to black
238
What dermatoscopic findings would suggest a seborrhoeic keratosis?
orange or brown clods
white 'milia-like' clods
curved thick ridges and furrows forming a brain-like pattern
239
Seborrhoeic Keratosis is classified as benign, premalignant or malignant?
benign
240
Actinic keratosis is classified as benign, premalignant or malignant?
premalignant
241
Keratoacanthoma is classified as benign, premalignant or malignant?
premalignant
242
Bowen's Disease is classified as benign, premalignant or malignant?
premalignant
243
What's are risk factors for developing actinic keratosis?
Sun exposure - geography/photoageing/sunburn/fair skin
| Poor immune function
244
What's the mechanism for development of actinic keratosis?
DNA damage by UVB
245
What region of the body do actinic keratoses tend to form?
Sun-exposed areas, e.g. back of hands, face, balding scalp
246
What appearance do actinic keratoses tend to have?
Flat/thickend papule or plaque
White/yellow, scaly, warty surface
Skin coloured/pigmented
Tender/asymptomatic
247
What is the main concern with actinic keratoses?
Development of squamous cell carcinoma
248
A Rosette sign is seen on dermoscopy, what condition does this suggest?
Actinic Keratosis
249
What dermoscopic features might you see in actinic keratosis?
Rosette sign - four bright white points like a four leaf clover
250
What are the histological features of actinic keratosis?
```
"SPAIN"
Solar elastosis
Parakeratosis
Atypia/Dysplasia
Inflammation
Not full thickness
```
251
What is solar elastosis?
Accumulation of elastin in the dermis
252
What is parakeratosis?
Keratinisation characterised by retention of nuclei in the stratum corneum.
253
What type of cells do keratoacanthomas arise from?
hair follicle skin cells
254
A small pimple that grows rapidly to about 2cm with a central crusted area on a region of sun-damaged skin is suggestive of what?
Keratoacanthoma
255
How can keratoacanthomas present?
Rapidly growing nodule
Central crusted region
Located on sun-damaged skin
256
How can you treat keratoacanthomas?
Removal with cryotherapy, curettage and cautery, excision or radiotherapy.
257
Why should you treat keratoacanthomas?
Obtain pathology - r/o SCC
| Cosmetic - get rid of ugly lesion and minimise scar which can be worse if left to spontaneously resolve
258
What is Bowen's disease?
Intra-epidermal squamous cell carcinoma in situ
259
What are risk factors for Bowen's Disease?
sun exposure
arsenic ingestion
HPV infection
Immunosuppression
260
What are the clinical features of Bowen's disease?
one or more (asymmetrical) irregular scaly plaques, red/pigemented
261
What dermoscopic features support a diagnosis of Bowen's disease?
crops of rounded/coiled blood vessels
262
What histological findings would support a diagnosis of Bowen's disease?
full thickness dysplasia of the epidermis - no dermis involvement
263
What is your main concern with Bowen's disease?
development of invasive SCC
264
What layers of the skin does SCC affect?
Derived from epidermis and has invaded beyond, i.e. to the dermis.
265
Which gene mutation is commonly associated with SCC?
mutation of the p53 tumour suppression gene
266
What are the clinical features of SCC?
enlarging scaly/crusted lumps arising from pre-existing actinic keratosis/Bowen's disease.
Located on sun-exposed areas
May be tender/painful
May ulcerate
267
What is known as a rodent ulcer?
Basal Cell Carcinoma
268
What are the main clinical characteristics of a basal cell carcinoma?
Slow growing plaque or nodule
Skin coloured, pink/pigmented
Spontaneous bleeding/ulceration
269
What histological features are typical of basal cell carcinomas?
Mass of basal cells pushing down into dermis
| Palisading - nuclei align in outermost layer
270
A 'buckshot' appearance is associated with what condition? and what is this appearance
Melanoma
| atypical epithelioid melanocytes found singly or in clusters scattered throughout the epidermis
271
A mass of basal cells pushing down into the dermis are features associated with what condition?
basal cell carcinoma
272
Breslow thickness is used for what?
Staging melanoma
273
What system can we use for staging Melanoma?
Breslow thickness
274
Where does lentigo maligna melanoma occur?
Sun-exposed areas
275
What age group does nodular malignant melanoma tend to affect?
younger folks
276
Where does acral lentiginous melanoma tend to affect?
Palms, soles and subungal areas
277
What triggers Stevens Johnson Syndrome/Toxic Epidermal Necrolysis?
Reactions to Drugs/Medications - technically any but some are more common than others, e.g. antibiotics or anticonvulsants
278
What are the clinical symptoms of Stevens Johnson Syndrome?
Prodromal 'flu-like' illness (fever, sore throat, aches)
Abrupt onset of tender/painful red skin rash on the trunk which extends rapidly to limbs and face - usually reached a max by 4 days
Blisters --> sheets of skin detachment with oozing dermis.
279
What is Nikolsky sign and what condition is it associated with?
Blisters/erosions appear in response to gentle skin rubbing.
A/w Stevens Johnson Syndrome
280
How do the classifications of Stevens Johnson Syndrome and Toxic Epidermal Necrolysis compare?
SJS = Skin detachment less than 10% body surface area
TEN = detachment greater than 30%
281
What blood test can be predictive of Stevens Johnson Syndrome/Toxic Epidermal Necrolysis?
elevated serum granulysin
282
Elevated serum granulysin in the first few days of a drug eruption may indicate what?
Stevens Johnson Syndrome/Toxic Epidermal Necrolysis
283
What are the histopathological features of Stevens Johnson Syndrome/Toxic Epidermal Necrolysis?
Keratinocyte necrosis
Full thickness epidermal necrosis
Minimal inflammation
Negative antibodies
284
Keratinocyte necrosis, full thickness epidermal necrosis, minimal inflammation and negative antibodies on a skin biopsy indicate what condition?
Stevens Johnson Syndrome/Toxic Epidermal Necrolysis
285
The SCORTEN system is used for what condition?
Stevens Johnson Syndrome/Toxic Epidermal Necrolysis
286
How to treat Stevens Johnson Syndrome?
```
Cease suspected causative drug
Admit
Nutritional and fluid support
Temperature maintenance
Analgesia
```
287
What triggers pityriasis rosea?
viral illness
288
How might pityriasis rosea present clinically?
Post-viral illness
Herald patch - oval pink/red plaque with scale inside the edge
Secondary rash in Christmas tree distribution - scaly patches/plaques appear on chest and back
289
What does a herald patch look like and what condition is it associated with?
Oval pink/red plaque with a scale inside the edge.
Pityriasis Rosea
290
What is the causative agent in erythema infectiosum?
Parvovirus B19
291
Parvovirus B19 causes which condition?
Erythema infectiosum
292
What is the treatment for erythema infectiosum?
Supportive
293
Can children with erythema infectiosum go to school?
yes
| the infectious stage has passed by the time the rash is evident.
294
What age do infants typically develop seborrhoeic dermatitis and when will it usually resolve?
Less than 3 months, resolve by 6-12 months.
295
What are the clinical features of infantile seborrhoeic dermatitis?
Cradle cap (diffuse, greasy scaling on scalp)
Salmon-pink patches that may flake or peel
Non-itchy, i.e. calm baby
296
How do you treat infantile seborrhoeic dermatitis?
Reassurance
Regular washing with baby shampoo
Emollient during nappy changes
297
What causes molluscum contaiosum?
Poxvirus - molluscum contagiosum virus
298
How can molluscum contagiosum be spread?
Skin-to-skin contact
shared towels/other items
Auto-inoculation by scratching into another site
Sexual transmission in adults
299
What is the incubation period for molluscum contagiosum?
2 weeks to 6 months
300
What are the clinical features of molluscum contagiosum?
Clusters of small white/pink/brown papules
Shiny with a small central pit
Warm places like armpit or groin
301
How long does molluscum contagiosum usually last?
1-2 years
302
What typically causes non-bullous impetigo?
Staphylococci and/or streptococci invasion of minor trauma site
303
What typically causes ecthyma?
Streptococcus pyogenes
| co-infection with Staph. aureus may occur
304
What typically causes bullous impetigo?
Staphylococcal infection
305
What is the progression of skin lesions in non-bullous impetigo?
pink macule --> vesicle/pustule --> crusted erosion --> untreated will heal without scarring
306
What is the progression of the skin lesion in ecthyma?
pink macule --> punched-out necrotic ulcer --> heals slowly, leaving scar
307
What is the clinical progression of bullous impetigo?
small vesicles --> flaccid transparent bullae --> heals without scarring
308
How to treat impetigo?
```
Cleanse wound using moist soaks
Avoid close contact with others
Apply antiseptics or Abx ointment
Cover affected areas
If extensive, oral Abx
```
309
Can children with impetigo go to school?
Stay away from school until crusts have dried out
310
Clinical features of chickenpox in children?
itchy rash of red papules --> vesicles on trunk
311
When is a person with chickenpox contagious?
1-2 days before the rash appears and all the way until every single blister has formed scabs (approx 5-10 days)
312
Which plasmodium species works by sequestration?
P. Falciparum
313
Which plasmodium:
early trophozoites
absence of mature trophozoites or schizonts
Plasmodium falciparum
314
What are the treatment options for plasmodium falciparum?
Quinine with doxcycline for seven days
Artemisinin-based combination therapy
315
Schuffner's dots are seen on microscopy. What organism does this indicate?
Plasmodium ovale or Plasmodium vivax
316
What drug would you give to prevent relapse of P. vivax or P. ovale?
Primaquine
317
What drug would you give for P. vivax or ovale?
Artemisinin-based combination therapy
| or Chloroquinine
318
What are the fever intervals for the plasmodium species?
P. falciparum, vivax and ovale - 48 hours
P. malariae - 72 hours (quartan fever)
319
Which plasmodium species is characterised by a compact parasite that does not alter host erythrocyte shape or size?
P. malariae P. knowlesi
320
What are the reasons for performing a thick and thin film in Malaria management?
Thick - to identify parasites
| Thin - distinguish malarial species
321
Botulinum toxin acts on what family of proteins to mediate its effects?
SNARE family of proteins - involved in vesicle fusion and NT release
322
What is the mechanism of action of botulinum toxin?
Cleavage of SNARE proteins
| Inhibition of ACh release
323
What are the signs/symptoms of botulism?
bulbar palsy and descending paralysis
apyrexial
clear sensorium (normal senses and mental state)
324
What are potential sources of Clostridium Botulinum?
Canned foods
325
What are potential sources of Clostridium Perfringens?
Poorly prepared meat/poultry
| Long standing prepared food
326
Which agent is the most common cause of gas gangrene?
Clostridium Perfringens
327
What is the treatment for botulism?
Supportive care
| Botulism antitoxin
328
Common cause for pseudomembranous colitis?
Clostridium dificile
329
What does C. dificile require in order to cause pseudomembranous colitis?
Toxin A or Toxin B
330
Treatment for C. dificile?
Oral metronidazole 10-14 days
| if severe, Oral Vancomycin 10-14 days
331
A few weeks after an infection a symmetrical ascending muscle weakness begins.
Guillan-Barre Syndrome
332
What is the 'big danger' with Guillan-Barre Syndrome?
Respiratory Failure
333
What are the two main modalities of treatment for Guillan-Barre Syndrome?
Plasmapheresis and IV immunoglobulin
334
What type of antibodies are involved in myasthenia gravis?
Post-synaptic acetylcholine receptor antibodies
335
What type of antibodies are involved in Lambert-Eaton syndrome?
Pre-synaptic voltage-gated calcium channel antibodies
336
What muscle groups tend to be affected first in myasthenia gravis?
Ocular
337
What muscle groups tend to be affected first in Lambert-Eaton syndrome?
Legs
338
What is a medical treatment in myasthenia gravis and what is its mechanism of action?
Pyridostigmine - acetylcholinesterase inhibitor thus increases ACh duration in synaptic cleft
339
How might you treat a myasthenic crisis?
Identify trigger for relapse (infection, medications, etc.)
?Ventilate
Treat with plasmapharesis or IV Ig
340
What surgical treatment is available for myasthenia gravis?
Thymectomy - if thymoma present
341
A muscle weakness that improves after exercise.
Lambert-Eaton syndrome
342
A muscle weakness that worsens with use and improves upon resting.
Myasthenia Gravis
343
What skin and eye signs can be present in neurofibromatosis type 1?
Cafe-au-lait spots
Lisch nodules - brown mounds on iris
Freckling
