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BRS - Year 2 > 👍🏻Dermatology👍🏻- Skin in Systemic Disease > Flashcards

👍🏻Dermatology👍🏻- Skin in Systemic Disease Flashcards

1
Q

Why is the skin in systemic disease important?

A

Rashes may be more than “skin deep”
Prevent or reduce internal organ damage by early diagnosis
Allow detection of internal malignancy

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2
Q

What are the two main groups of lupus erythematosus?

A

Systemic lupus erythematosus
Cutaneous (discoid) lupus erythematosus

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3
Q

What is categories can the diagnostic criteria for lupus be broken down into?

A

Mucocutaneous
Haematological
Immunological

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4
Q

What are the haematological signs of SLE?

A

Haemolytic anaemia
Thrombocytopenia
Leukopenia

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5
Q

What immunological investigations can be carried out for lupus?

A

ANA (high)
Anti-dsDNA (high)
Anti-Sm (high)
Antiphospholipid (high)
Complement level (low)
Direct Coomb’s test (detects antibodies attached to red blood cells)

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6
Q

What is ANA?

A

Anti-nuclear antibodies
Screening test for pretty much all autoimmune conditions, particularly dermatological ones

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7
Q

What are the signs and symptoms of SLE?

A

Photodistributed rash
Cutaneous vasculitis
Chilblains
Alopecia
Livedo reticularis
Cutaneous vasculitis
Subacute cutaneous lupus (SCLE)

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8
Q

What are the signs and symptoms of cutaneous (discoid) lupus erythematosus?

A

Discoid lupus erythematosus
SCLE
Overlap with SLE

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9
Q

What is livedo reticularis?

A

Impaired blood flow leads to small clots and vessel spasms
Lacy, net like pattern on the skin

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10
Q

What are chilblains?

A

Inflamed swollen patches and blistering on the hands and feet

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11
Q

What is alopecia?

A

Hair loss (can be patches of hair or full head of hair)
Can be temporary or permanent

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12
Q

What is cutaneous vasculitis?

A

Inflammation of the small blood vessels in the skin

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13
Q

What is subacute cutaneous lupus (SCLE)?

A

red, scaly, or raised lesions on the skin

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14
Q

What is discoid lupus?

A

Red, inflamed, scaly, and crusty patches of skin
Lesions that are often found on the scalp, cheeks, and ears

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15
Q

How might a patient present with SLE?

A

New onset (weeks) of:
Rash
Fever
Arthritis
Fatigue
Bloods show:
Pancytopenia
Proteinuria
Increased ESR / CRP
ANA 1:640
dsDNA positive

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16
Q

What is dermatomyositis?

A

Autoimmune connective tissue disease
Proximal extensor inflammatory myopathy

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17
Q

What are the characteristic features of dermatomyositis?

A

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

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18
Q

How can the clinical features of dermatomyositis be predicted?

A

Subtypes with clinical features that can be predicted by autoantibody profile

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19
Q

What is myositis?

A

Autoimmune disease that causes muscle inflammation and weakness

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20
Q

What would you expect with Anti Jo-1 type dermatomyositis?

A

Fever
Myositis
Gottron’s papules

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21
Q

What would you expect with Anti SRP type dermatomyositis?

A

Necrotisisng myopathy
Dysphagia

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22
Q

What would you expect with Anti Mi-2 type dermatomyositis?

A

Mild muscle disease

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23
Q

What would you expect with Anti-p155/TIF1ɣ type dermatomyositis?

A

Associated with malignancy (in adults)

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24
Q

What would you expect with Anti-p140 type dermatomyositis?

A

juvenile, associated with calcinosis

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25
Q

What would you expect with Anti-SAE type dermatomyositis?

A

Amyopathic - no muscle weakness

26
Q

What would you expect with Anti-MDA5 type dermatomyositis?

A

Interstitial lung disease
Digital ulcers/ischaemia

27
Q

What might a dermatomyositis patient feature?

A
28
Q

What are the investigations for dermatomyositis?

A

Blood tests incl. ANA, CK, LFTs
Skin biopsy
LFT (ALT often increased)
EMG
Muscle MRI
Screening for internal malignancy (e.g. CT CAP)

29
Q

What is Henoch-Schonlein purpura?

A

IgA vasculitis

30
Q

What would you get with Henoch-Schonlein purpura?

A

Macular purpura
Abdominal pain, bleeding
Arthralgia/arthritis
IgA associated glomerulonephritis (may develop later)

31
Q

What are the investigations for IgA vasculitis?

A

Bloods
Urinalysis
Biopsy

32
Q

Outline the types of small vessel vasculitis

A
33
Q

Outline the types of small+medium vessel vasculitis

A
34
Q

Outline the types of medium vessel vasculitis

A

Polyarteritis nodosa (PAN)

-Benign cutaneous form
-Systemic form

35
Q

Outline the types of large vessel vasculitis

A

Temporal arteritis
Takayasu arteritis

36
Q

What are the manifestations of small vessel vasculitis?

A
37
Q

What are the medium vessel manifestations of vasculitis?

A
38
Q

What is DRESS?

A

Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS)
Severe, delayed hypersensitivity reaction to certain drugs, characterized by rash, haematological abnormalities, organ involvement, and systemic inflammation

39
Q

What is the scoring criteria for DRESS?

A

Fever ≥ 38.5°C
Lymphadenopathy (lymph node swelling) ⩾ 2 sites, > 1cm
Circulating atypical lymphocytes
Peripheral hypereosinophilia
Internal organs involved - (liver, kidneys, cardiac)
Negative ANA, Hepatitis / mycoplasma, chlamydia
Skin involvement

40
Q

What skin involvement would you see in DRESS?

A

> 50% BSA
Cutaneous eruption suggestive of DRESS
Biopsy suggestive of DRESS

41
Q

What is the onset for DRESS?

A

2-6 weeks after exposure to drug

42
Q

What internal organs are commonly affected in DRESS?

A

Liver most common internal organ involved – majority of deaths associated with this
Kidneys and cardiac system also commonly involved

43
Q

What rash morphologies would you see in DRESS?

A

Urticated papular exanthem - widespread papules
Maculopapular (morbilliform) eruption
Widespread erythema (Erythroderma)
Head / neck oedema
Erythema multiforme-like

44
Q

What is the treatment for DRESS?

A

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

45
Q

What is does itching without a rash suggest?

A

internal cause

46
Q

What is pruritus?

A

A sense of itchiness/itching

47
Q

What are the haematological causes of pruritus?

A

Lymphoma
Polycythaemia
Uraemia (due to renal failure)

48
Q

What are the other causes of pruritus?

A

Cholestasis
Iron deficiency/overload
HIV, hepatitis A/B/C
Cancer
Drugs (NB opiates/opioids)
Psychogenic
Pruritus of old age

49
Q

What are the investigations for a patient presenting with pruritus?

A

FBC, LDH
Renal profile
Liver function tests
Ferritin
XR Chest
HIV / Hepatitis A / B /C

50
Q

What is nodular prurigo?

A

Hard, raised bumps that are extremely itchy, especially at night
Can interfere with sleep and quality of life
Likely caused by chronic scratching and rubbing of the skin

51
Q

What is Stevens-Johnson syndrome?

A

Derm emergency! (Rare)
Extensive full thickness mucocutaneous (epidermal) necrosis <2-3 days
Caused by adverse reaction to a drug or infection

52
Q

What is the most common cause of Stevens-Johnson syndrome?

A

Drugs > infection

53
Q

What does untreated Stevens-Johnson syndrome lead to?

A

Toxic epidermal necrolysis (TEN)
Can be very lethal

54
Q

Outline the progression of Stevens-Johnson syndrome

A

Prodromal: flu-like sx
Abrupt onset of lesions on trunk > face/limbs
Macules, blisters, erythema – atypical targetoid
Blisters merge – sheets of skin detachment ‘like wet wallpaper’

55
Q

What are some differential diagnoses when considering TEN?

A

Staphylococcal scalded skin syndrome (SSSS)
Thermal burns
Cutaneous graft versus host disease

56
Q

What is the pathology of TEN?

A

Cell-mediated cytotoxic reaction against epidermal cells

Drugs cause >80% of cases
May be started up to 3 weeks prior to onset of rash

57
Q

What is used to assess the severity of TEN?

A

SCORTEN Criteria:
age >40
HR
initial % epidermal detachment
serum urea + glucose + bicarbonate
presence of malignancy

58
Q

What are the complications of TEN?

A

Death - Overall mortality 30%
Blindness
dehydration
hypothermia/hyperthermia
renal tubular necrosis
eroded GI tract
interstitial pneumonitis
neutropenia
liver and heart failure

59
Q

What is erythroderma?

A

Generalized erythema affecting >90% BSA

60
Q

What is the significance of erythroderma?

A

Systemic manifestations reflect impairment in skin function:
-Peripheral eodema
- Tachycardia
- Loss of fluid and proteins
- Disturbances in thermoregulation
- Risk of sepsis

61
Q

What are the possible aetiologies of erythroderma?

A
  • Psoriasis
  • Atopic eczema
  • Drug reactions
  • Cutaneous T-cell lymphoma – Sézary syndrome
  • Idiopathic (25-30%)
62
Q

What is the treatment/management for erythroderma?

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 temperature
Emollients to support skin barrier
+/- Topical steroids
+/- Antibiotics

BRS - Year 2 (61 decks)

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