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Z Year 2 BRS MSK > Skin in Systemic Disease > Flashcards

Skin in Systemic Disease Flashcards

1
Q

Why is the skin in systemic disease important?

A

•Rashes may be more than skin deep
•Comprehensive assessment coupled with dermatological diagnostic skills can:
1. Prevent or reduce internal organ damage by early diagnosis
2. Allow detection of internal malignancy

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2
Q

What is skin targeted systemic disease?

A

Multi-organ systemic disease targeting skin e.g. Sarcoidosis

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3
Q

What are skin signs in systemic disease?

A

Sign of internal disorder e.g. flushing in Carcinoid syndrome

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4
Q

What are ‘tell-tale’ skin conditions in systemic disease?

A

Skin conditions suggestive of underlying condition e.g. Pyoderma gangrenosum in inflammatory bowel disease

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5
Q

What is secondary systemic involvement in systemic disease

A

Systemic disease secondary to skin disorder e.g. high output cardiac failure in erythroderma

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6
Q

What blood tests may be ordered?

A
  1. FBC
  2. Renal profile
  3. Liver function tests
  4. Inflammatory markers
  5. Autoimmune serology (could show autoimmune)
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7
Q

What microbiology may be ordered?

A
  1. Viral/bacterial serology
  2. Swabs for bacteria C and S, viral PCR
  3. Tissue culture / PCR
    - (could show infection)
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8
Q

What imaging may be ordered?

A
  • Internal organ involvement
  • Vascular supply
  • (could show neoplastic)
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9
Q

What do you for a skin biopsy?

A
  • Microscopy

- (could show neoplastic)

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10
Q

What specific tests would you do?

A
  1. Urinalysis
  2. Never conduction studies
  3. Endcorine investigations etc
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11
Q

What are the two main groups of lupus erythematosus?

A
  1. Systemic Lupus Erythematosus
  2. Cutaneous (Discoid) Lupus Erythematosus
    - Overlap
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12
Q

What is in the mucotaenous SLE diagnostic criteria?

A
  1. Cutaneous lupus - acute
  2. Cutaneous lupus - chronic
  3. Oral ulcers
  4. Alopecia
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13
Q

What is in the haematological SLE diagnostic criteria?

A
  1. Haemolytic anaemia
  2. Thrombocytopenia Haematological
  3. Leukopenia
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14
Q

What is in the immunological SLE diagnostic criteria?

A
  1. ANA
  2. Anti-dsDNA
  3. Anti-Sm
  4. Antiphospholipid Immunological
  5. Low Complement
  6. Direct Coomb’s test
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15
Q

What else is in the SLE diagnostic criteria?

A
  1. Synovitis
  2. Serositis (pleurisy or pericarditis)
  3. Renal disorder
  4. Neurological disorder
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16
Q

What is part of SLE?

A 
1. Photodistributed rash 
2. Cutaneous vasculitis
3. Chilblains 
4. Alopecia
5. Livedo reticularis
6. Cutaneous vasculitis 
7.. Subacute cutaneous lupus  (SCLE)
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17
Q

What is part of Cutaneous (Discoid) Lupus Erythematosus

A
  1. Discoid lupus erythematosus

2. SCLE

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18
Q

Why do you test ECG in neonatal lupus?

A
  1. Underlying disorder – neonatal lupus
    (Ro positive)
  2. Test ECG
    – risk of heart block (50% risk)
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19
Q

What is dermatomyositis?

A

-Autoimmune connective tissue disease

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20
Q

What are signs of dermatomyositis?

A
  • Proximal extensor inflammatory myopathy

- Photo-distributed pink-violet rash favouring scalp, periocular regional and extensor surfaces

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21
Q

How can you predict subtypes of dermatomyositis?

A

-subtypes with clinical features that can be predicted by autoantibody profile
- Malignancy
- Interstitial lung disease
- Digital ischaemia

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22
Q

What are symptoms of dermatomyositis?

A
  1. Gottron’s papules
  2. Ragged cuticles
  3. Shawl sign
  4. Heliotrope rash
  5. Photosensitive erythema
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23
Q

What is anti Jo-1 dermatomyositis?

A

fever, myositis, gottron’s papules

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24
Q

What is anti SRP dermatomyositis?

A

nectrotising myopathy

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25
What is anti Mi-2 dermatomyositis?
mild muscle disease
26
What is anti-p155 | dermatomyositis?
associated with malignancy (in adults)
27
What is anti-p140 dermatomyositis?
juvenile, associated with calcinosis
28
What is anti-SAE dermatomyositis?
+/- amyopathic
29
What is anti-MDA5 dermatomyositis?
interstitial lung disease, digital ulcers / ischaemia
30
What are the investigations for dermatomyositis?
1. ANA 2. CK 3. Skin biopsy 4. LFT (ALT often increased) 5. EMG 6. Screening for internal malignancy
31
When would you do biopsy for direct immunofluorescence perivascular IgA?
Henoch-Schonlein purpura (IgA vasculitis)
32
What is the classification of small affected vessels in vasculitis?
1. Cutaneous small vessel (leukocytoclastic) vasculitis | 2. Small vessle vasculatisi- special type
33
What is the subclassification of small affected vessels in vasculitis?
1. Idiopathic 2. Infectious 3. Medication exposure 4. Inflammatory (connective tissue disease) 5. IgA vasculitis (Henoch-Scholein) 6. Urticarial vasculitis 7. acute haemorrhagic oedema of infancy 8. Erythema elevatum ditutinum
34
What is the classification of small and medium affected vessels in vasculitis?
1. Cyroglobulinemia | 2. ANCA-associated
35
What is the classification of small and medium affected vessels in vasculitis?
1. Type I and II 2. EGPA (Churg-Strauss) 3. Microscopic polyangitis 4. GPA (wegener)
36
What is the classification of small and medium affected vessels in vasculitis?
Polyarteritis nodosa (PAN)
37
What is the subclassification of medium affected vessels in vasculitis?
1. Benign cutaneous form | 2. Systemic form
38
What are the small vessel manifestations of vasculitis?
purpura (macular/palpable)
39
What are the medium vessel manifestations of vasculitis?
1. Digital decrosis 2. Retiform purpura ulcers 3. Subcutaenous nodules along blood vessels
40
What is the initial presentation of IgA vasculitis like?
- Initial presentation often indistinguishable from other small vessel vasculitis 1. Abdominal pain, bleeding (65%), arthralgia / arthritis (64%) 2. IgA-associated glomerulonephritis (40%)
41
What is the prognosis for IgA vasculitis like?
- favourable but depends on severity of renal disease 1. IgA vasculitis lasts up to 6 months in 1/3 of patients 2. Persistent nephropathy occurs in 8%, progressive renal failure in 1-3% 3. those with haematuria or proteinuria should be carefully followed
42
What signs are in granulomatosis with polyangiitis?
•Cough, dyspnoea, and chest pain: - Pulmonary infiltrates (70%) - Glomerulonephritis (85%) - Cutaneous findings (50%)
43
What are the small vessel manifestations in cutaneous findings of granulomatosis with polyangiitis?
Purpura (macular / flat or popular /   palpable)
44
What are the large vessel manifestations in cutaneous findings of granulomatosis with polyangiitis?
1. Retiform purpura 2. Digital necrosis 3. Livedo reticularis 4. Subcutaneous nodules distributed along   blood vessels  5. Ulcers
45
What is sarcoidosis?
Systemic granulomatous disorder of unknown origin | •Can affect multiple organs: most commonly lungs
46
What are the cutaneous manifestations (in 33%) of sarcoidosis?
1. Highly variable – ‘the great mimicker’ 2. Red-brown to violaceous papules and face, lips,   upper back, neck, and extremities 3. Lupus pernio – NB 4. Ulcerative 5. Scar sarcoid 6. Erythema nodosum
47
What is the histology of sarcoidosis?
non-caseating epithelioid granulomas
48
What does the diagnosis of sarcoidosis require?
* Diagnosis of exclusion | * Requires evaluation for internal organ involvement
49
What is drug reaction with eosinophilia and systemic symptoms (DRESS)?
Rash and systemic upset incorporating haematological and solid‐organ disturbances
50
What are the features of DRESS?
1. Rash 2. May manifest as: - Characteristic rash e.g. facial oedema - Widespread rash >50% BSA 3. Fever ≥ 38.5°C 4. Lymphadenopathy 5. Peripheral eosinophilia >0.7 × 109 6. Internal organ involvement (liver, kidneys, cardiac)
51
What is the internal organ involvement in DRESS like?
1. Liver (hepatitis) - most frequent cause of death 2. Kidneys (interstitial nephritis)  3. Heart (myocarditis)  4. Brain  5. Thyroid (thyroiditis)  6. Lungs (interstitial pneumonitis) 
52
What does the diagnosis of DRESS require?
scoring criteria criteria including: 1. Fever 2. Lymphadenopathy  ⩾ 2 sites, > 1cm 3. Circulating atypical lymphocytes 4. Peripheral hypereosinophilia 5. Internal organs involved 6. Negative ANA, Hepatitis / mycoplasma, chlamydia 7. Skin involvement
53
What skin involvement is in DRESS?
- >50% BSA - Cutaneous eruption suggestive of DRESS e.g. facial oedema - Biopsy suggestive of DRESS
54
When does DRESS start?
* Underlying mechanism not known | * Starts 2-6 weeks after drug exposure
55
What are the most common symptoms for DRESS?
1. fever 2. rash 3. Face, upper trunk and extremities are initial sites of involvement
56
What are common triggers for DRESS?
- Sulfonamides - anti-epileptics (carbamazepine - phenytoin, lamotrigine) - allopurinol - Antibiotics (vancomycin, amoxicillin, minocycline, piperacillin-tazobactam) - ibuprofen
57
Why does DRESS result in death?
Liver most common internal organ involved
58
What is the rash morphology for DRESS?
1. Urticated papular exanthem - widespread papules 2. Morbilliform eruption 3. Erythroderma / widespread exfoliative erythema 4. Head / neck oedema 5. Erythema multiforme-like
59
What is the treatment for DRESS?
* Withdrawal of culprit * Corticosteroids are first line treatment  - may require months of treatment * Mortality 5-10%
60
What is schnitzler syndrome?
Late‐onset acquired autoinflammatory syndrome
61
When does schnitzler syndrome usually manifest?
adulthood
62
What is the first sign of schnitzler syndrome?
- A recurrent urticarial rash is usually the first sign -  non‐pruritic urticated macules, papules or plaques, particularly on the trunk, which resolve with brownish hyperpigmentation
63
What are the other signs of schnitzler syndrome?
1. Raised monoclonal IgM 2. Recurrent fever above 40°C 3. Bone or joint pain (especially over the ilium or tibia) 4. Lymphadenopathy, hepatomegaly or splenomegaly, 5. Neutrophilia 6. Elevated acute phase reactants or abnormal bone imaging
64
What is the treatment for mild schnitzler syndrome?
colchicine
65
What is the treatment of severe anakinra?
anakinra

Z Year 2 BRS MSK (18 decks)

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