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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
Q

What is anti Mi-2 dermatomyositis?

A

mild muscle disease

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26
Q

What is anti-p155

dermatomyositis?

A

associated with malignancy (in adults)

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27
Q

What is anti-p140 dermatomyositis?

A

juvenile, associated with calcinosis

28
Q

What is anti-SAE dermatomyositis?

A

+/- amyopathic

29
Q

What is anti-MDA5 dermatomyositis?

A

interstitial lung disease, digital ulcers / ischaemia

30
Q

What are the investigations for dermatomyositis?

A
  1. ANA
  2. CK
  3. Skin biopsy
  4. LFT (ALT often increased)
  5. EMG
  6. Screening for internal malignancy
31
Q

When would you do biopsy for direct immunofluorescence perivascular IgA?

A

Henoch-Schonlein purpura (IgA vasculitis)

32
Q

What is the classification of small affected vessels in vasculitis?

A
  1. Cutaneous small vessel (leukocytoclastic) vasculitis

2. Small vessle vasculatisi- special type

33
Q

What is the subclassification of small affected vessels in vasculitis?

A
  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
Q

What is the classification of small and medium affected vessels in vasculitis?

A
  1. Cyroglobulinemia

2. ANCA-associated

35
Q

What is the classification of small and medium affected vessels in vasculitis?

A
  1. Type I and II
  2. EGPA (Churg-Strauss)
  3. Microscopic polyangitis
  4. GPA (wegener)
36
Q

What is the classification of small and medium affected vessels in vasculitis?

A

Polyarteritis nodosa (PAN)

37
Q

What is the subclassification of medium affected vessels in vasculitis?

A
  1. Benign cutaneous form

2. Systemic form

38
Q

What are the small vessel manifestations of vasculitis?

A

purpura (macular/palpable)

39
Q

What are the medium vessel manifestations of vasculitis?

A
  1. Digital decrosis
  2. Retiform purpura ulcers
  3. Subcutaenous nodules along blood vessels
40
Q

What is the initial presentation of IgA vasculitis like?

A
  • Initial presentation often indistinguishable from other small vessel vasculitis
    1. Abdominal pain, bleeding (65%), arthralgia / arthritis (64%)
    2. IgA-associated glomerulonephritis (40%)
41
Q

What is the prognosis for IgA vasculitis like?

A
  • 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
Q

What signs are in granulomatosis with polyangiitis?

A

•Cough, dyspnoea, and chest pain:

  • Pulmonary infiltrates (70%)
  • Glomerulonephritis (85%)
  • Cutaneous findings (50%)
43
Q

What are the small vessel manifestations in cutaneous findings of granulomatosis with polyangiitis?

A

Purpura (macular / flat or popular / palpable)

44
Q

What are the large vessel manifestations in cutaneous findings of granulomatosis with polyangiitis?

A
  1. Retiform purpura
  2. Digital necrosis
  3. Livedo reticularis
  4. Subcutaneous nodules distributed along blood vessels
  5. Ulcers
45
Q

What is sarcoidosis?

A

Systemic granulomatous disorder of unknown origin

•Can affect multiple organs: most commonly lungs

46
Q

What are the cutaneous manifestations (in 33%) of sarcoidosis?

A
  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
Q

What is the histology of sarcoidosis?

A

non-caseating epithelioid granulomas

48
Q

What does the diagnosis of sarcoidosis require?

A
  • Diagnosis of exclusion

* Requires evaluation for internal organ involvement

49
Q

What is drug reaction with eosinophilia and systemic symptoms (DRESS)?

A

Rash and systemic upset incorporating haematological and solid‐organ disturbances

50
Q

What are the features of DRESS?

A
  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
Q

What is the internal organ involvement in DRESS like?

A
  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
Q

What does the diagnosis of DRESS require?

A

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
Q

What skin involvement is in DRESS?

A
  • > 50% BSA
  • Cutaneous eruption suggestive of DRESS e.g. facial oedema
  • Biopsy suggestive of DRESS
54
Q

When does DRESS start?

A
  • Underlying mechanism not known

* Starts 2-6 weeks after drug exposure

55
Q

What are the most common symptoms for DRESS?

A
  1. fever
  2. rash
  3. Face, upper trunk and extremities are initial sites of involvement
56
Q

What are common triggers for DRESS?

A
  • Sulfonamides
  • anti-epileptics (carbamazepine
  • phenytoin, lamotrigine)
  • allopurinol
  • Antibiotics (vancomycin, amoxicillin, minocycline, piperacillin-tazobactam)
  • ibuprofen
57
Q

Why does DRESS result in death?

A

Liver most common internal organ involved

58
Q

What is the rash morphology for DRESS?

A
  1. Urticated papular exanthem - widespread papules
  2. Morbilliform eruption
  3. Erythroderma / widespread exfoliative erythema
  4. Head / neck oedema
  5. Erythema multiforme-like
59
Q

What is the treatment for DRESS?

A
  • Withdrawal of culprit
  • Corticosteroids are first line treatment - may require months of treatment
  • Mortality 5-10%
60
Q

What is schnitzler syndrome?

A

Late‐onset acquired autoinflammatory syndrome

61
Q

When does schnitzler syndrome usually manifest?

62
Q

What is the first sign of schnitzler syndrome?

A
  • 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
Q

What are the other signs of schnitzler syndrome?

A
  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
Q

What is the treatment for mild schnitzler syndrome?

A

colchicine

65
Q

What is the treatment of severe anakinra?

Z Year 2 BRS MSK (18 decks)

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