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Year 2 - BRS > 7.1 - The skin in systemic disease > Flashcards

7.1 - The skin in systemic disease Flashcards

1
Q

What different causes of dermatological conditions are there? (9)

A
  • idiopathic
  • neoplastic
  • infection
  • inflammatory
  • drug-induced
  • autoimmune
  • traumatic
  • metabolic
  • genetic
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2
Q

What are examples of clinical assessments used in dermatology? (5)

A
  • blood tests (FBC, renal profile, LFTs, inflammatory markers, autoimmune serology)
  • microbiology (viral/bacterial serology, swabs for bacteria C&S, viral PCR, tissue culture/PCR)
  • imaging (internal organ involvement, vascular supply)
  • skin biopsy (microscopy)
  • specific (urinalysis, nerve conduction studies, endocrine investigations etc)
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3
Q

What is a punch biopsy?

A
  • biopsy of skin done under local anaesthetic
  • cells taken and examined for inflammatory patterns/cell abnormalities to look for neoplasia
  • can also look for autoantibodies through immunofluorescence
  • can also be sent for tissue culture
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4
Q

What are the two main groups of lupus erythematosus?

A
  • systemic lupus erythematosus
  • cutaneous (discoid) lupus erythematosus
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5
Q

How can the diagnostic criteria for systemic lupus erythematosus be grouped? (4)

A
  • mucocutaneous
  • haematological
  • immunological
  • joint + organ involvement
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6
Q

What are the mucocutaneous diagnostic criteria for systemic lupus erythematosus? (4)

A
  • cutaneous lupus - acute (e.g. chilblains and photodistributed erythematosus rash)
  • cutaneous lupus - chronic
  • oral ulcers
  • alopecia
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7
Q

What are the haematological diagnostic criteria for systemic lupus erythematosus? (3)

A
  • haemolytic anaemia
  • thrombocytopenia
  • leukopenia
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8
Q

What are the immunological diagnostic criteria for systemic lupus erythematosus? (6)

A
  • ANA
  • anti-dsDNA
  • anti-Sm
  • antiphospholipid
  • low complement
  • direct Coomb’s test
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9
Q

What are the joint and organ involvement diagnostic criteria + other diagnostic criteria for systemic lupus erythematosus? (2 + 2)

A
  • synovitis
  • serositis (pleurisy/pleuritis or pericarditis)
  • renal disorder
  • neurological disorder
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10
Q

What are some manifestations of systemic lupus erythematosus? (6)

A
  • photodistributed rash (sun-exposed)
  • chilblains
  • alopecia
  • livedo reticularis (net-like erythema)
  • cutaneous vasculitis
    • palpable purpura - small vessel cutaneous vasculitis
  • subacute cutaneous lupus (SCLE) - ring-like (annular) plaques
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11
Q

What are some manifestations of cutaneous (discoid) lupus erythematosus? (2)

A
  • discoid lupus erythematosus (often causes scarring)
  • SCLE (subacute cutaneous lupus) - ring-like/annular plaques
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12
Q

What does a ring-like or annular rash on a baby indicate?

A

Immediate diagnosis of neonatal lupus - do ECG as there is 50% risk of heart block

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13
Q

What is dermatomyositis?

A

Autoimmune connective tissue disease

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14
Q

What is dermatomyositis characterised by? (2)

A
  • proximal extensor inflammatory myopathy
  • photodistributed pink-violet rash favouring scalp, periocular regional and extensor surfaces
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15
Q

What are some distinct clinical features of dermatomyositis? (5)

A
  • Gottron’s papules (violaceous red plaques on MCP and DIP joints)
  • ragged cuticles
  • shawl sign (rash of upper trunk)
  • heliotrope rash (erythema of upper eyelids)
  • photosensitive erythema
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16
Q

Does dermatomyositis have subtypes?

A

Yes - subtypes with distinct clinical features that can be predicted by autoantibody profile

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17
Q

What different subtypes of dermatomyositis exist and what clinical features are they associated with? (7)

A
  • anti Jo-1 (fever, myositis, Gottron’s papules)
  • anti SRP (necrotising myopathy)
  • anti Mi-2 (mild muscle disease)
  • anti-p155 (associated with malignancy in adults)
  • anti-p140 (juvenile, associated with calcinosis)
  • anti-SAE (+/- amyopathic)
  • anti-MDA5 (intersitital lung disease, digital ulcers/ischaemia)
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18
Q

What diagnostic tests do we do for dermatomyositis? (6)

A
  • ANA (anti-nuclear AB) - often +ve
  • LFT - ALT often increased
  • CK and EMG (muscle involvement)
  • skin biopsy
  • screening for internal malignancy (imaging and tumour markers)
  • dermatomyositis panel antibodies - subtype
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19
Q

Vasculitis - biopsy for direct immunofluorescence shows perivascular IgA autoantibodies, what is this?

A

IgA vasculitis (Henoch-Schonlein purpura)

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20
Q

What are some manifestations of IgA vasculitis? (5)

A
  • abdominal pain
  • bleeding (GI)
  • arthralgia (joint stiffness)
  • arthritis
  • IgA-associated glomerulonephritis (may develop later, monitor)
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21
Q

What types of vasculitis are small vessels affected by? (2)

A
  • cutaneous small vessel (leukocytoclastic) vasculitis
  • small vessel vasculitis - special types
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22
Q

What are the subclassifications of cutaneous small vessel (leukocytoclastic) vasculitis? (4)

A
  • idiopathic
  • infectious
  • inflammatory (connective tissue disease)
  • medication exposure
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23
Q

What are some subclassifications of small vessel vasculitis (4)

A
  • IgA vasculitis (Henoch-Schonlein)
  • urticarial vasculitis
  • acute haemorrhagic oedema of infancy
  • erythema elevatum diutinum
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24
Q

What types of vasculitis are small and medium vessels affected by? (2)

A
  • cryoglobulinemia
  • ANCA-associated
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25
What are some subclassifications of cryoglobulinemia?
Type II & III
26
What are some subclassifications of ANCA-associated vasculitis? (3)
- EGPA (Churg-Strauss) - microscopic polyangiitis - GPA (Wegener)
27
What type of vasculitis is medium vessels affected by, and what are its subclassifications?
Polyarteritis nodosa (PAN) - benign cutaneous form - systemic form
28
What types of vasculitis are large vessels affected by? (2)
- temporal arteritis - Takayasu
29
What is a small vessel manifestation of vasculitis?
Purpura (macular / palpable)
30
What are the medium vessel manifestations of vasculitis? (3)
- digital necrosis - retiform 'net-like' purpura ulcers - subcutaneous nodules along blood vessels
31
What type of deformity is associated with ANCA-associated vasculitis?
Saddle nose deformity
32
What is sarcoidosis?
Systemic granulomatous disorder of unknown origin (non-infectious systemic inflammatory disorder in which granulomas infiltrate different organ systems)
33
What can sarcoidosis affect?
Can affect multiple organs, most commonly lungs
34
How common are cutaneous (skin) manifestations of sarcoidosis?
33%
35
What makes sarcoidosis hard to diagnose?
Highly variable - 'the great mimicker'
36
What are some of the cutaneous (skin) manifestations of sarcoidosis? (5)
- red-brown violaceous papules of face, lips, upper back, neck and extremities - lupus pernio (involvement of nose and cheeks, nothing to do with lupus erythematosus despite name) - ulcers - scar sarcoid - erythema nodosum (swollen fat under skin of legs)
37
What does histology of sarcoidosis show?
Non-caseating (means no necrosis) epithelioid granulomas
38
What kind of diagnosis is sarcoidosis?
Diagnosis of exclusion - must exclude infection e.g. through TB culture/PCR
39
What else must be monitored in sarcoidosis?
Internal organ involvement e.g. CXR for lung involvement
40
What does DRESS stand for?
Drug Reaction with Eosinophilia and Systemic Symptoms
41
What is DRESS?
Rash and systemic upset incorporating haematological and solid-organ disturbances
42
What are the diagnostic criteria for DRESS? (7 - do not need to memorise?)
- fever >38.5 - lymphadenopathy >/=2 sites, >1cm - circulating atypical lymphocytes - peripheral hypereosinophilia >0.7x10^9 - internal organs involved - negative ANA, hepatitis/mycoplasma, chlamydia - skin involvement - >50% BSA - cutaneous eruption suggestive of DRESS - biopsy suggestive of DRESS
43
What internal organs are involved in DRESS? (6)
- liver (hepatitis) - most common, majority of deaths associated with this - kidneys (interstitial nephritis) - heart (myocarditis) - brain - thyroid (thyroiditis) - lungs (interstitial pneumonitis)
44
What do we know about the mechanism for DRESS?
- underlying mechanism not known - starts 2-6 weeks after drug exposure
45
What are some common drug triggers for DRESS? (5)
- sulfonamides - allopurinol - anti-epileptics (lamotrigine, carbamazepine, phenytoin) - antibiotics (vancomycin, amoxicillin, minocycline, piperacillin-tazobactam) - ibuprofen
46
What can the rash look like in DRESS? (5)
- urticated papular exanthem - widespread papules - maculopapular (morbilliform) eruption - widespread erythema (erythroderma) - head and neck oedema - erythema multiforme-like (targetoid lesions)
47
How do we treat DRESS?
- withdrawal of culprit - corticosteroids are first line treatment - may require months of treatment - mortality 5-10%
48
What kind of disorder is Graft vs Host disease (GvHD)?
Multiple-organ disease, alloimmune
49
Who does Graft vs Host disease (GvHD) affect?
10-80% of allogenic haematopoietic stem call transplants (HSCT)
50
How do we know if a rash is caused by Graft vs Host disease (GvHD) as opposed to drugs? (3)
- face involvement - acral involvement - diarrhoea - (indicates GvHD more likely)
51
What is the pathogenesis of Graft vs Host disease (GvHD)?
Donor-derived T-lymphocyte activity against antigens in an immunocompromised patient
52
What body parts does Graft vs Host disease (GvHD) mainly affect? (3)
- skin - liver - GI tract
53
What does itching (pruritus) without a rash suggest?
Internal cause - something else going on
54
What needs to be considered when there is an itch without a rash? (9)
- haematological causes - lymphoma, polycythaemia - uraemia - cholestasis - iron deficiency or iron overload - HIV / hepatitis A/B/C - cancer - drugs (esp opiates/opioids) - psychogenic - pruritus of old age
55
What investigations do we do for itching without a rash? (6)
- FBC, LDH - renal profile - liver function tests - ferritin - chest X-ray - HIV / hepatitis ABC test
56
What do patients develop if they keep itching?
Nodular prurigo - skin thickening as a defence mechanism from the scratch
57
What is carcinoid syndrome?
When a malignant carcinoid tumour metastasises and secretes 5-HT into system
58
What symptom occurs in 25% of carcinoid syndrome cases?
Flushing
59
What are some other symptoms of carcinoid syndrome (apart from flushing)? (3)
- diarrhoea - bronchospasm - hypotension
60
What is Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS:TEN)?
A dermatological emergency!! (Rare)
61
What is the sequence of development of symptoms in Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS:TEN)? (5 steps)
1. prodromal flu-like symptoms 2. abrupt onset of lesions on trunk, then face/limbs 3. macules, blisters, erythema - atypical targetoid 4. blisters then merge and sheets of skin detach 'like wet wallpaper' 5. this represents extensive full thickness mucocutaneous (epidermal) necrosis in 2-3 days
62
When is SJS:TEN called SJS and when is it called TEN?
- when <10% of BSA detachment = SJS (<10% mortality) - when 10-30% of BSA detachment = SJS/TEN - when >30% of BSA detachment = TEN (>30% mortality)
63
What is the pathophysiology of SJS:TEN?
Cell-mediated cytotoxic reaction against epidermal cells
64
What is SJS:TEN mostly caused by?
- drugs cause >80% of cases - antibiotics e.g. beta-lactams, sulfonamides - allopurinol - anti-epileptics e.g. phenytoin, carbamazepine, lamotrigine - NSAIDs - may be started up to 3 weeks prior to onset of rash
65
What are the differential diagnoses for SJS:TEN? (3)
- staphylococcal scalded skin syndrome (SSSS) - thermal burns - cutaneous Graft vs Host Disease (GvHD)
66
How is the severity of SJS:TEN determined?
- SCORTEN score calculated with criteria: (do not need to memorise) - age > 40 - initial % epidermal detachment - serum area + glucose + bicarbonate - presence of malignancy
67
What are some complications of SJS:TEN? (9)
- death (overall mortality 30%) - blindness - dehydration - hypothermia/hyperthermia - renal tubular acidosis - eroded GI tract - interstitial pneumonitis - neutropenia - liver and heart failure
68
What is erythroderma?
Generalised erythema affecting >90% BSA (impairs skin function)
69
What can erythroderma manifest as systemically? (5)
- peripheral oedema - tachycardia - loss of fluid and proteins - disturbances in thermoregulation - risk of sepsis
70
What can cause erythroderma? (5)
- drug reactions - cutaneous T-cell lymphoma (Sezary syndrome) - psoriasis - atopic eczema - idiopathic (25-30%)
71
Describe the management of erythroderma. (6)
- underlying cause (e.g. treat psoriasis, withdraw drug if drug cause, etc) - hospitalisation if systemically unwell - restore fluid and electrolyte balance, circulatory status and manage body temperature - emollients to support skin barrier - maybe topical steroids - maybe antibiotics
72
What should we be aware of regarding common diseases?
A variety of common diseases can also cause skin manifestations, and recognition of these can allow early diagnosis
73
What are some examples of types of systemic diseases that have dermatological manifestations? (7)
- nutritional disorders: - scurvy (vitamin C deficiency) - Kwashiorkor - malignancy - chronic kidney disease - chronic liver disease - endocrine disorders (DM, Graves, Addison's, acromegaly, Cushing's, PCOS) - GI disease (IBD, Crohn's, Coeliac) - infection
74
Compare SLE vs DLE vs subacute LE.
- SLE - redness of sun-exposed areas (photo-distributed rash) - DLE - scars, plaques, can have underlying SLE - subacute LE - ring/annular - to disregard SLE - negative systems review, no photo-distributed rash, negative antibodies, FBC normal etc

Year 2 - BRS (84 decks)

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