Stuff Flashcards
1
Q
Typical presentation of porphyria cutanea tarda?
A
Fragility and blisters
Other features: hyperpigmentation, hypertrichosis, solar urticaria, morphoea
2
Q
Investigation for porphyria cutanea tarda?
A
Woods lamp
3
Q
Causes of porphyria cutanea tarda?
A
Alcohol, viral hepatitis, oestrogens, haemochromatosis
4
Q
Defect in uroporphyrinogen decarboxylase causes what?
A
Porphyria cutanea tarda
5
Q
Cancer you might get if you have PCT?
A
Hepatoma (hepatocellular carcinoma)
6
Q
Defect in ferrochelatase causes which condition?
A
Erythropoietic protoporphyria
7
Q
Investigations for erythropoietic protoporphyria
A
Quantitative RBC porphyrins Fluorocytes (patient, relatives) Transaminases [Hb], red cell indices (Biliary tract USS) (Phototesting)
8
Q
Management of EPP
A
6 monthly LFTs and RBC porphyrins
Visible light photoprotection measures (particular need for caution if surgery needed)
Prophylactic TL-01 phototherapy
Anti-oxidants (beta-carotene, cysteine, high dose vitamin C)
(Avoid iron)
Incipient liver failure: oral charcoal; cholestyramine; ? ALA synthase inhibition
Liver failure: transplant liver; ? Bone marrow
9
Q
Acute intermittent porphyria differentials?
A
Acute abdomen
Mononeuritis multiplex
Guillain-Barré syndrome
Psychoses
10
Q
(female) patient with abdominal pain +/- neurological +/- psychiatric +/- hyponatremia
A
Acute intermittent porphyria
11
Q
Fluid and electrolyte balance
A
Sweat glands
12
Q
Epidermis cells
A
Mainly squamous
Stratified keratinising
13
Q
Where are the melanocytes found?
A
Dermo-epidermal junction
14
Q
Ratio of melanocytes : basal cells
A
1:10
15
Q
Which layer of epidermis are melanocytes found in?
A
The basal layer
16
Q
Where are langerhans cells found?
A
Mid - upper epidermis
17
Q
Dermis
A
The dermis: a thicker inner portion. This is the connective tissue layer of skin. It is important for sensation, protection and thermoregulation. It contains nerves, the blood supply, fibroblasts, etc, as well as sweat glands, which open out onto the surface of the skin, and in some regions, hair. The apical layers of the dermis are folded, to form dermal papillae, which are particularly prominent in thick skin
18
Q
Acanthosis
A
Thickening of epithelium
19
Q
Parakeratosis
A
Persistence of nuclei in the keratin layer
20
Q
Hyperkeratosis
A
Increased thickening of keratin layer
21
Q
Papillomatosis
A
Irregular epithelial thickening
22
Q
Psoriasiform
A
Elongation of rete ridges e.g. psoriasis
23
Q
Munro micro abcess
A
Associated with psoriasis
-an abcess (collection of neutrophils) in the stratum corneum
24
Q
Which investigation should all patients presenting with erythema nodosum be given?
A
x-ray for chest involvement
25
Anti-endomysial antibodies
Dermatitis herpetiformis
26
Areas affected by dermatitis herpetiformis?
Elbows, knees, back and buttocks
27
Side effect of nifedipine?
Gingival hyperplasia
28
People with polymyalgia rheumatica may also develop what?
Giant cell arteritis
29
Lattisimus dorsi?
Adducts shoulder
Extends shoulder
Internally rotates shoulder
30
SLE biologics
Anti-CD20 (rituximab)
| Anti-Blys (Belimumab)
31
Serum and synovial keratin sulphate
Levels of serum and synovial keratin sulphate increase with age and in OA
32
SLE is which kind of hypersensitivity?
Type III hypersensitivty
33
Nucleolar ANA
Scleroderma
34
Speckled antibody
SLE and sjogrens
35
What would you find in a type III hypersensitivity biopsy?
Immune complexes
Neutrophils and macrophages
Products of complement activation
36
Rheumatoid arthritis type of hypersensitivity
```
Type IV (delayed)
Characterised by infiltration of synovium with CD4+ cells
```
37
TNF AND IL-1 PLAY CRITICAL ROLE IN WHICH DISEASE?!
RA
| -Important mediators of joint damage!!
38
What is rheumatoid factor?
antibody directed against the Fc region of IgG
39
Major indications for Rheumatoid factor testing?
Good indicator of PROGNOSIS of RA (more severe etc, associated with extra-articular manifestations e.g. nodules and vasculitis)
May be helpful in diagnosis of Sjogrens and cryoglobulinemia
40
Best predictor of aggressive disease course in RA?
Anti-CCP
41
Systemic inflammatory response syndrome?
HB > 90
RR > 20
WBC >12,000 or <4.3 kPa
42
Salmonella may cause osteomyelitis in which specific set of patients?
Sickle cell anemia patients
43
Poorly controlled diabetics, immunocompromised and IV drug users are particularly at risk of osteomyelitis where?
In the spine!!
44
Risks of HRT
Endometrial and breast cancer
| DVT
45
How to diagnose stress fracture
Ultrasound or bone scintigraphy
They are common in the pelvis
Show first radiological signs of healing 4-8 weeks post fracture
46
Galeazzi fracture?
Fracture of the distal third of the radius and dislocation of the ulna
47
Fracture just above the elbow with displacement of the distal fragment
Supracondylar fracture
48
Supracondylar fracture risks
Median nerve
Brachial artery
Compartment syndrome
49
SUFE associated with which endocrine disorder?
Hypothyroidism
50
Osgood schlatters disease
Osteochondritis of the tibial tuberosity
- Classically affects teenagers and those who enjoy sport
- local swelling or tender lump over tibial tuberosity
- symptoms exacerbated by exercise and relieved by rest
51
Pink/blue sweaty hand that is tender to touch and stiff
| Injury occurred some weeks ago
Complex regional pain syndrome
52
Swelling an calf tenderness following THR?
Deep vein thrombosis
53
Chondromalacia
Damage to the cartilage at the back of the knee cap
| -More commonly found in girls who exercise regularly
54
What commonly precedes tranient synovitis of the hip?
Upper respiratory tract infection
55
Where would you develop a chondroma?
Hands and feet
56
Distinct clinical picture of osteoid osteoma?
Worse at night, dull pain, characteristically relieved by NSAIDs or aspirin
57
Enchondroma histology?
Small round pyknotic nuclei, no atypia
58
Classical RADIOLOGICAL image of osteoid osteoma?
Radiolucent niddus surrounded by reactive sclerosis in cortex of bone
59
Chicken wire calcification
Chondroblastoma
60
Chondroblastoma histology
Closely packed polygonal cells plus areas of immature chondroid
-Mitotic activity low
61
Treatment for chondroblastoma?
Biopsy and curretage plus adjuvant liquid nitrogen
62
Multi-nucleated cells in a sea of round to oval cells
Giant cell tumour
63
Irregular spicules of mineralised bone and osteoid surrounded by osteoblasts
Vascular stroma with pleomorphic SPINDLE CELLS
Osteoid and woven bone are seen
Osteoblastoma
64
Lobules and fibrous septa
Chorda
Lobules and fibrous septa
The malignant cells have eosinophilic cytoplasm and vacuoles of mucus push the nuclei to the side
65
Separation of nodules by fibrous bands would be highly suggestive of what?
Malignancy
Malignant nodules infiltrate between the lamellar bone obliterating the marrow. Separation of the nodules by fibrous bands would be highly suggestive of malignancy
66
Peripheral primitive neuroectodermal tumour
Ewings sarcoma
67
Treatment for Ewings Sarcoma
Surgery
Radiation therapy
Chemotherapy with vincristine, dactomycin and cyclophosphamide
Post-operative adjuvant chemotherapy
68
Ideal pH?
7.35-7.45
69
Which organ excretes/resorbs bicarbonate?
Kidneys
70
Tumour derived from remnants of notocord?
Chordoma
71
Chondrosarcoma shows pure differentiation of which type of cartilage?
Chondrosarcoma shows pure differentiation of hyaline cartilage
72
PNET
Peripheral neuroectodermal tumour
| Ewing's sarcoma
73
Multiple myeloma
Malignant proliferation of plasma cells in bone marrow
Occurs in old age
Often causes renal failure
Results in bone destruction of the axial skeleton
74
Chicken wire calcification
Chondroblastoma
75
Dermatomyositis may lead to which type of cancers?
Breast, ovarian, stomach, lung, bladder, colon
76
Malignancy risk related to dermatomyositis is highest in which group of people?
Men over 45
77
Anti-Jo-1 antibodies
Myositis
Indicate poor prognosis with interstitial lung disease
The lung disease occurs in about 1/3rd
78
Drug treatment for polymyositis/dermatomyositis
Early initiation of therapy is essential.[8]
Steroids are the most important drugs. In mild disease, topical steroids may suffice. In more severe disease, high doses of systemic steroids are used and tapered off. Improvement is usually apparent by the second or third month. The usual precautions must be exercised when giving high doses of steroids for long periods.
If steroids fail then immunosuppressive drugs such as azathioprine can be used. As an alternative, cyclophosphamide is usually better than methotrexate.
Intravenous immunoglobulin is appropriate in patients with resistant dermatomyositis or aggressive disease
79
Inclusion myositis histology
Inflammatory infiltrate.
Cytoplasmic vacuolation.
Characteristic tubo-filamentous inclusions within the cytoplasm and nuclei of muscle cells.
80
Papular eruption characterised by its violacous colour and polygonal shape, sometimes with a fine scale
It is most often found on the flexor surfaces of the upper extremities, genitalia and mucous membranes!!
Lichen planus
81
Somebody has primary biliary psoriasis, what skin condition might they have?
Lichen planus
82
Drugs which could cause eruptions similar to lichen planus?
Thiazides, anti-malarials and gold
83
When does lichen planus tend to spontaneously resolve?
6-12 months
84
Ring fibres in muscle are a sign of what?
Dystrophic change
Also: fatty replacement, regeneration, segmental necrosis, increased central nuclei, myocyte hypertrophy and splitting, variability in muscle fibre size and endomysial fibrosis
85
Myotonic dystrophy
Face, respiratory, limbs
| Dominant
86
Duchenne and Becker muscular dystrophy
X-linked recessive
87
Limb girdle and scapulohumeral muscular dystrophy
autosomal recessive
88
Fascisuloscapulohumeral, oculopharyngeal and myotonic dystrophy method of inheritance?
Dominant
89
Pseudohypertrophy of calves
DMD
90
Myotonic dystrophy
Myotonic dystrophy is characterized by progressive muscle wasting and weakness. People with this disorder often have prolonged muscle contractions (myotonia) and are not able to relax certain muscles after use. For example, a person may have difficulty releasing their grip on a doorknob or handle. Also, affected people may have slurred speech or temporary locking of their jaw.
Other signs and symptoms of myotonic dystrophy include clouding of the lens of the eye (cataracts) and abnormalities of the electrical signals that control the heartbeat (cardiac conduction defects). In affected men, hormonal changes may lead to early balding and an inability to father a child (infertility). The features of this disorder often develop during a person's twenties or thirties, although they can occur at any age. The severity of the condition varies widely among affected people, even among members of the same family.
There are two major types of myotonic dystrophy: type 1 and type 2. Their signs and symptoms overlap, although type 2 tends to be milder than type 1. The muscle weakness associated with type 1 particularly affects the lower legs, hands, neck, and face. Muscle weakness in type 2 primarily involves the muscles of the neck, shoulders, elbows, and hips. The two types of myotonic dystrophy are caused by mutations in different genes.
91
Atrophy of type 1 fibres?
Myotonic dystrophy
92
Chromosomes involved in myotonic dystrophy?
Ch3 and Ch19
93
Parts of the body affected in myotonic dystrophy?
Adolescent: face and limbs
Later: respiratory
94
Upper body erythema, swelling of eyelids with purple discolouration
(plus polymyositis)
Dermatomyositis
95
Target lesions
```
Erythema multiforme
(doesn't affect mucous membranes)
```
96
Progressive degeneration in anterior horn cells
Motor neuron disease
97
Chromosome involved in spinal muscular atrophy?
Chromosome 5
| -autosomal recessive
98
What might cause myoglobulinuria?
Rhabdomyolysis
99
What does rhabdomyolysis cause?
```
Hyperkalaemia
Myoglobulinuria
Necrosis and shock
OUTCOME;
Acute Renal Failure (breakdown products of skeletal muscle and myoglobin are harmful to the kidneys)
```
Hypovolaemia & hyperkalaemia
Metabolic acidosis
Disseminated intravascular coagulation
100
SLE hypersensitivity?
Visceral lesions – mediated by Type III hypersensitivity
Haematological effects – mediated by Type II hypersensitivity
101
Coombs test
Checks for antibodies that affect red blood cells
102
PAN
§fever, weight loss, abdominal pain, and polyneuropathy revealed areas of focal inflammatory exudations that gave rise to palpable nodules along the course of medium-sized arteries
103
Temporal artery biopsy; inflammation,+/- giant cells, fragmentation of internal elastic lamina
Giant cell arteritis
104
Cells seen in Rosacea inflammation?
Plasma
105
Rhinophyma?
Thickening of skin, e.g. in rosacea
106
Common to all variants of pemphigus is the process of
Acantholysis :)
| =lysis of inracellular adhesion sites
107
Papillary dermal microabscesses
Dermatitis herpetiformis!!
108
Follicular demodex mite is often seen in which condition?
Rosacea
109
Patchy inflammation with plasma cells?
| Perifollicular granulomas
Rosacea
110
Pemphigus vulgaris, bullous pemphigoid and dermatitis herpetiformis, IgA or IgG?
Pemphigus vulgaris and bullous pemphigoid = IgG
| Dermatitis herpetiformis = IgA
111
Elongation of rete ridges
Psoriasis
112
Munro microabscesses
Psoriasis
113
What is the epidermal BM made of?
Laminin and collagen IV
114
Which part of the dermis contains appendage structures-sweat glands, pilosebaceous units?
The reticular dermis
115
Where are melanocytes found in the dermis?
In the basal layer
116
Epidermis
Stratified squamous keratinising epithelium
117
Key hallmarks of cancer (photocarcinogenesis lecture)
1. Insensitivity to growth signals
2. Insensitivity to anti-growth signals
3. Evade death (apoptosis)
4. Limitless potential to divide
5. Angiogenesis
6. Invasion and metastasis
118
Sun exposure length and skin cancer type
Intermittent exposure: BCC & melanoma
| Chronic exposure: SCC
119
UV signature mutation
Cytosine to thymine
120
Which type of UV causes initiation mutations in cancer?
UVB --> initiation mutation
| UVB/UVA --> promotion
121
MCC
Merkel skin cancer
122
Syndromes predisposing to BCC?
XP
| Basal cell nevus syndrome (Gorlin Syndrome)
123
Drugs associated with photocarcinogenesis?
```
Psoralen
Voricanzole
Azathioprine
Vemurfanib
Immunosuppressants
Photosensitising drugs
Topicals
```
124
Inflammatory mediators up regulated in photocarcinogenesis
IL-10 and PGE2
- decrease langerhans cells
- increase oxidative stress
- interact with endogenous compounds
125
Papillomatosis
Irregular epithelial thickening
126
Acanthosis
Increased thickening of epidermis
127
Contact allergic dermatitis
Delayed, type 4
128
Drug related dermatitis
Type1/4 hypersensitivity
| spongiotic dermatitis and eosinophils
129
Immunopathology of contact dermatitis
Langerhans cell in epidermis processes antigen
( immunogenicity).
Processed antigen is then presented to Th cells in dermis
Sensitised Th cells migrate into lymphatics and then to regional nodes where antigen presentation is amplified
130
What type of dermatitis is nappy rash?
Irritant contact dermatitis
| Sparing of flexures
131
Which other allergic conditions is eczema associated with?
Allergic rhinitis
Asthma
Food allergy
132
Chronic changes in eczema
Lichenification
Excoriation
Secondary infection
133
Monomorphic punched out lesions??
Herpes simplex virus
| Eczema herpeticum
134
Diagnostic criteria for Eczema
```
Itching plus 3 or more:
Visible flexural rash*
History of flexural rash*
Personal history of atopy (or first degree relative if under 4 yo)
Generally dry skin
Onset before age 2 years
```
*cheeks and extensor surfaces in infants
135
Treatment of Eczema
```
Plenty of emollients
Avoid irritants including shower gels and soaps
Topical steroids
Treat infection
Phototherapy – mainly UVB
Systemic immunosuppressants
(Biologic agents)
```
136
Chronic Actinic Dermatitis
Photosensitive dermatitis
137
Stasis eczema is secondary to what?
hydrostatic pressure
oedema
red cell extravasation
138
Pompholyx eczema
Spongiotic vesicles
| -tiny blisters on hands and feet
139
Upper cervical spine
C1 and C2
140
Atlanto-occipital joint
Flexion/extension
| Lateral extension
141
Ligamentum flavum
Link vertebral laminae
142
Example where one column of the spine would be fractured?
Osteoporotic wedge fracture
143
Intermediate muscle of the spine?
Serratus posterior (spinous processes --> ribs)
144
Dorsal scapula nerve
C3/C4
| innervates rhomboid major and rhomboid minor
145
Transverse process of C1 --> superior angle of scapula
Levator scapularis
| Innervation: branches of C4/C5
146
What forms the intervertebral foramina?
Notches in the pedicles
147
Biceps tendonitis
Usually a vague anterior shoulder pain which has an aching quality
148
Shoulder girdle
Clavicel and scapula
149
Where does a SLAP tear occur?
A SLAP tear occurs where the biceps tendon attaches to the labrum
150
TFCC
triangular fibrocartilage complex
151
Which nerve supplies the thenar muscles?
The median nerve
152
What innervates the abductor digiti minimi muscle?
The ulnar nerve
153
Solution to eyes immune system or lack thereof
Blink reflex
Physical and chemical properties of eye surface.
Limit exposure/size
154
Lysozyme
Gram negative and fungi
155
Secretory IgA
Prevents attachment
156
Lactoferrin and transferrin
Gram positive
157
Tear lipids
Anti-bacterial to cell membranes
158
Cornea
Clear dome over the iris
159
Which is the only part of the eye with lymphatic drainage?
The conjunctiva
- Also has dendritic cells
- Mucosa associated lymphoid tissue
- commensal bacteria
160
Choroid
```
Vascular layer of the eye containing connective tissue
Lies between the retina and sclera
-Blood ocular layer
-Relative lack of APC's
-down regulated immune system
```
161
Hypersensitivity reaction
Immune response that results in bystander damage to the self
162
Fab
Antigen specific region
163
Optical Cicatricial Pemphigoid type of hypersensitivIty?
Type II
164
Autoimmune corneal melting type of hypersensitivity?
Type III
165
Corneal graft rejection type of hypersensitivity?
Type IV
166
Where does the subarachnoid space end?
Ends at the level of S2
