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BRS YEAR 2 > Skin In Systemic Disease > Flashcards

Skin In Systemic Disease Flashcards

1
Q

What are 4 ways in which the skin links with systemic disease?

A

Skin targeted systemic disease eg sarcoidosis
Skin signs eg sign of interns, disorder flushing in carcinoid syndrome
Tell tale sign such as pyoderma gangrenosum in ibd
Secondary systemic involvement eg cardiac output failure in erythoderma

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2
Q

What investigations for neoplastic

A
  • Imaging to look for internal organ involvement and vascular supply
  • Skin biopsy and microscopy
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3
Q

IVX for infections

A
  • Viral/bacterial serology
  • Swabs for bacteria C&S, viral PCR
  • Tissue culture/PCR
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4
Q

IVX autoimmune

A
  • FBC
  • Renal profile
  • Liver function test
  • Inflammatory markers
  • Autoimmune serology
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5
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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6
Q

What are the 2 main categories of lupus erythematous

A
  • Systemic lupus erythematosus
  • Cutaneous (discoid) lupus erythematosus
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7
Q

What are the 3 types of diagnostic criteria for systemic lupus erythematosus

A

Mucocutaneous (4)

  • Cutaneous lupus- acute- e.g. chillblains and photodistributed (Sun-exposed areas) erythematosus rash
  • Cutaneous lupus- chronic
  • Oral ulcers
  • Alopecia

Hametaological

  • Haemolytic anaemia
  • Thrombocytopenia
  • Leukopenia

Immunological

  • ANA
  • Anti-dsDNA
  • Anti-Smith
  • Antiphospholipid
  • Low Complement
  • Direct Coomb’s test
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8
Q

What other diagnostic criteria are there for sle

A
  • Synovitis
  • Serositis (pleurisy or pericarditis)
  • Renal disorder
  • Neurological disorder
    Livedo reticularis (net like erythema)
    Palpable purpura (Sam,, vessel cutaneous vasculitis)
    Subacute cutaneous lupus (SCLE) (ring like annular plaques)
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9
Q
  • What are the diagnostic criteria for cutaneous (discoid) lupus erythematosus? (2)
A

Discoid lupus erythematosus
SCLE

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10
Q

Neonatal lupus

A

Do ecg as 50% risk of heart block

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11
Q

What is dermatomyosistis

A

Autoimmune connective tissue disease
Characterized by - Proximal extensor inflammatory myopathy
- Photodistributed pink-violet rash favouring scalp, periocular regional and extensor surfaces

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12
Q

Distinct features of dermatomyositis (5)

A

Gottron’s papules
MCP and DIP joints
They are violacious plaques

Ragged cuticles

Shawl sign
Redness of upper trunk

Heliotrope rash
Erythema of eyelids

Photosensitive erythema

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13
Q

What different subtypes are there and what clinical features are they associated with for dermatomyositis (7)

A
  • Anti Jo-1 (3)
    • Fever
    • Myositis
    • Gottron’s papules
  • Anti SRPNecrotising myopathy
  • Anti Mi-2Mild muscle disease
  • Anti p155Associated with malignancy in adults
  • Anti p140Juvenile, associated with calcinosis
  • Anti SAEWith or without amyopathy (no problems in muscle weakness)
  • Anti MDA5 (2)
    • Interstitial lung disease
    • Digital ulcers/ischaemia
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14
Q

Diagnostic tests for dermatomyositis

A

Anti nuclear antibody as its positive in most cases
Liver function test as ALT is usually increased
Dermatomyositis panel antibodies to figure out subtype
CK and EMB to look at muscles
Skin biopsy
Screening for internal malignancy via imaging and tumour markers as patients are at increased risk especially with p155

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15
Q

Macular purpara

A

Flat and not raised

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16
Q

Biopsy for direct immunofluorescence showing perivascular autoantibodies IgA- what is this?

A

IgA vasculitis (Henoch-Schonlein purpura)

  • Abdominal pain
  • GI bleeding
  • Arthralgia (joint stiffness)
  • Arthritis
  • IgA-associated glomerulonephritis (may develop later)
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17
Q

What types of vasculitis are different sizes of blood vessels affected by?

A

Small
Small and medium
Medium
Large

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18
Q

Small vessel vasculitis

A
  • Small vessel vasculitis- special types- subclassifications? (4)
    • IgA vasculitis (Henoch-Scholein)
    • Urticarial vasculitis
    • Acute haemorrhagic oedema of infancy
    • Erythema elevatum diutinum
  • Cutaneous small vessel (leukocytoclastic) vasculitis- what are the subclassifications? (4)
    • Idiopathic
    • Inflammatory (connective tissue disease)
    • Infectious
    • Medication exposure
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19
Q

Small and medium vasculitis

A
  • Cryoglobulinemia- subclassification?Type 2 or 3
  • ANCA-associated- subclassification? (3)
    • GPA (Wegener)
    • EGPA (Churg-Strauss)
    • Microscopic polyangiitis
20
Q

Medium vasculitis

A
  • Polyarteritis nodosa (PAN)- subclassifications? (2)
    • Benign cutaneous form
    • Systemic form
21
Q

Large vasculitis

A
  • Temporal arteritis
  • Tayakasu
22
Q

What are small vessel manifestations of vasculitis

A

Purpura both macular and palpable

23
Q

What are medium vessel manifestations of the disease

A

Digital necrosis
Retiform ‘net like’ purpura ulcer
Subcutaneous nodules

24
Q

What is drug reaction with eosinophilia and systemic symptoms

A

Rash and systemic upset involving haematological and solid-organ disturbances

25
Q

What are the scoring criteria for DRESS? (patients don’t have to have all of them, just score up to a certain amount of them) (7)

A
  • Fever ≥38.5°C
  • Lymphadenopathy ≥2 sites, 1cm
  • Circulating atypical lymphocytes
  • Peripheral hypereosinophilia >0.7 x 10^9
  • Internal organs involved- what does this include? (6)
    • Liver (hepatitis)- most frequent cause of death
    • Kidneys (interstitial nephritis)
    • Heart (myocarditis)
    • Thyroid (thyroiditis)
    • Lungs (interstitial pneumonitis)
    • Brain
  • Negative ANA, hepatitis/mycoplasma, chlamydia
  • Skin involvement
    • > 50% BSA (body surface area)
    • Cutaneous eruption suggestive of DRESS e.g. facial oedema
    • Biopsy suggestive of DRESS
26
Q

Mechanism of DRESS and common drug triggers

A
  • Underlying mechanism not known
  • Starts 2-6 weeks after drug exposure
  • Sulfonamides
  • Allopurinol
  • Anti-epileptics (lamotrigine, carbamazepine, phenytoin)
  • antibiotics (vancomycin, amoxicillin, minocycline, piperacillin-tazobactam)
  • Ibuprofen
27
Q

DRESS rash looks like

A
  • Urticated papular exanthem- widespread papules e.g. bottom right pic
  • Maculopapular (morbilliform) eruption
  • Widespread erythema (erythroderma)
  • Head/neck oedema
  • Erythema multiforme-like (with targetoid lesions) e.g. top right pic
28
Q

Treatment of DRESS

A
  • Withdrawal of culprit
  • Corticosteroids are first line treatment- may require months of treatment
  • 5-10% mortality
29
Q

What does itching without a rash suggest

A

Internal causes
Consider:

  • Haematological causes (lymphoma, polycythaemia)
  • Uraemia
  • Cholestasis
  • Iron deficiency or iron overload
  • HIV/hepatitis ABC
  • Cancer
  • Drugs (esp opiates/opioids)
  • Psychogenic
  • Pruritus of old age
30
Q

What tests do ‘itching without a rash’ patients need? (6)

A
  • FBC, LDH
  • Renal profile
  • Liver function test
  • Ferritin
  • CXR
  • HIV/hep ABC test
31
Q

What do patients develop if they keep itching?

A

Nodular prurigo- skin thickening as a defence mechanism from the scratch

32
Q

What is the sequence of development of symptoms? (5 steps) for steven Johnson syndrome/toxic epidermal necrolysis

A

1) Prodrome of 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

This is a derm emergency (rare)

33
Q

When is the disease called SJS and when TEN?

A
  • When <10% of BSA detachment → SJS
  • When 10-30% of BSA detachment → SJS/TEN
  • When >30% of BSA detachment → TEN
34
Q

What is its pathophysiology sjs

A

Cell-mediated cytotoxic reaction against epidermal cells
Caused by drugs and may be started up to 3 weeks before rash onset

  • Antibiotics e.g. beta-lactams, sulphonamides
  • Allopurinol
  • Anti-epileptic drugs e.g. phenytoin, carbamazepine, lamotrigine
  • NSAIDs
35
Q

Differential diagnosis for SJS

A
  • Staphylococcal scalded skin syndrome (SSSS)
  • Thermal burns
  • Cutaneous GvHD
36
Q

Severity if sjs determined by

A

SCORTEN score calculated with criteria:

  • Age >40
  • Initial % epidermal detachment
  • Serum urea + glucose + bicarbonate
  • Presence of malignancy
37
Q

What complications are there? (9) sjs

A
  • Death- overall mortality is 30%
  • Blindness
  • Dehydration
  • Hypothermia/hyperthermia
  • Renal tubular necrosis
  • Eroded GI tract
  • Interstitial pneumonitis
  • Neutropenia
  • Liver and heart failure
38
Q

Erythoderma

A

Generalised erythema affecting >90% BSA

39
Q

What can it manifest as systemically? (5) erythoderma

A
  • Peripheral oedema
  • Tachycardia
  • Loss of fluid and proteins
  • Disturbances in thermoregulation
  • Risk of sepsis
40
Q

What can cause erythoderma (5)

A
  • Drug reactions
  • Cutaneous T-cell lymphoma called Sezary syndrome
  • Sudden flare up of psoriasis
  • Atopic eczema
  • Idiopathic (25-30%)
41
Q

Management of erythoderma (6)

A
  • 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 temp
  • Emollients to support skin barrier
  • Maybe topical steroids
  • Maybe antibiotics
42
Q

Nutritional disdoers

A

Scurvy which is caused by vitamin c deficiency can cause spongy gingivae bleeding erosion petechiea echymoses corkscrew hair
Kwashiorkor caused by protein deficiency can cause sparse dry hair soft thin nails and cheilitis

43
Q

Malignancy

A

Leukaemia cutis
Paget’s disease if nipple
Extramammory pagets
Acanthosis nigricans
Leiomyomas

44
Q

Chronic kidney disease

A

Excoriations/prurigo
Xerosis
Half and half nails
Calciohylaxis

45
Q

Chronic live disease

A

Prurigo
Jaundice
Muehrckes line
Terrys nails
Palmar erythema
Spider telangeictasia
Clubbing if nails

46
Q

Endocrine disorder

A

Necrobiosis Lipoidica
Terry’s nails
Xerosis
Granuloma annulare
Xanthelesma
Xanthoma
Neuropathic ulcers

BRS YEAR 2 (56 decks)

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