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BRS DERMATOLOGY > 1b// The skin in systemic disease > Flashcards

1b// The skin in systemic disease Flashcards

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1
Q

What do you have to consider for differential diagnoses for derm? (9)

A

idiopathic
neoplastic
infection
inflammatory
drug-induced
autoimmune (or alloimmune)
traumatic
metabolic
genetic

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2
Q

What are the investigations you do for derm?

A

investigations are dependent on clinical findings

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3
Q

How may a patient with lupus present?(8)

A

rash
fatigue
arthritis
fever

pancytopenia
proteinuria
increased ESR/ CRP
dsDNA positive

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4
Q

What is pancytopenia?

A

involves having low levels of red blood cells, white blood cells and platelets

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5
Q

What is dsDNA?

A

Anti-double-stranded deoxyribonucleic acid antibodies (dsDNA Abs) are highly specific markers of systemic lupus erythematosus (SLE)

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6
Q

What are the 2 main groups of lupus erythematosus? And what are 3 other ones you need to know? (5)

A

Systemic Lupus Erythematosus

Cutaneous (Discoid) Lupus Erythematosus

(there can be overlap)
(they are broad groups)

  • the overlap is sub acute systemic lupus erythematosus
  • nephrogenic lupus (test with urinalysis, proteinuria)
  • neonatal lupus (50% risk of heart block)
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7
Q

What are the groups for the diagnostic criteria of systemic lupus erythematosus?

A

mucocutaneous findings

haematological findings

immunological findings

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8
Q

What are the mucocutaneous findings of systemic lupus erythematosus? (6)

A

Cutaneous lupus - acute
Cutaneous lupus - chronic
Oral ulcers
Alopecia
Synovitis
Serositis (pleurisy or pericarditis)

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9
Q

What are the haematological findings of systemic lupus erythematosus? (3)

A

Haemolytic anaemia
Thrombocytopenia
Leukopenia

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10
Q

What are the immunological findings of systemic lupus erythematosus? (6)

A

● ANA
● Anti-dsDNA
● Anti-Sm
● Antiphospholipid
● Low Complement
● Direct Coomb’s test

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11
Q

What are the disorders associated with systemic lupus erythematosus?

A

renal disorder
neurological disorder

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12
Q

What are some symptoms of systemic lupus erythematosus?

A

Photodistributed (erythematosus) rash
Cutaneous vasculitis
Chilblains (aka itchy swellings)
Alopecia
Livedo reticularis
Cutaneous vasculitis
Subacute cutaneous lupus (SCLE)

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13
Q

What are the types of cutaneous lupus erythematosus?

A

discoid lupus erythematosus

subacute cutaneous lupus erythematosus (SCLE)

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14
Q

What is the overlap between systemic and cutaneous lupus erythematosus?

A

SCLE (subacute cutaneous lupus)

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15
Q

What is a feature of discoid lupus?

A

scarring

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16
Q

Which lupus does not have scarring?

A

SCLE (subacute lupus erythematosus)

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17
Q

What is an autoimmune connective tissue disease?

A

dermatomyositis

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18
Q

What is dermatomyositis?

A

● Autoimmune connective tissue disease

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19
Q

What are the symptoms/ signs of dermatomyositis? (7)

A

Proximal extensor inflammatory myopathy

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

gottron’s papules

ragged cuticles

shawl sign

heliotrope rash

photosensitive erythema

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20
Q

How is dermatomyositis categorised?

A

in diff subtypes by antibodies

Subtypes with clinical features that can be predicted by autoantibody profile

Anti Jo-1 – fever, myositis, gottron’s papules

Anti SRP – necrotising myopathy

Anti-p155 – associated with malignancy (in adults)

Anti- MDA5 – interstitial lung disease, digital ulcers / ischaemia

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21
Q

What are the tests for dermatomyositis? (6)

A

● ANA
● CK
● Skin biopsy
● LFT (Alaninetransaminase often increased)
● EMG
● Screening for internal malignancy

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22
Q

How may a patient with vasculitis present?

A
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23
Q

What are the vessels that vasculitis can affect?

A

small
small and medium
medium
large

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24
Q

What is the classification of vasculitis?

A
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25
Q

What is the subclassifications on vasculitis?

A
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26
Q

What does it mean if IgA is involved in vasculitis?

A

organ involved

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27
Q

What are the medium vessel manifestations of vasculitis?

A

digital necrosis
retiform purpura ulcers
subcutaneous nodules along blood vessels

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28
Q

What are the small vessel manifestations of vasculitis?

A
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29
Q

What is sarcoidosis?

A

Systemic granulomatous disorder of unknown origin

non-infectious systemic inflammatory disorder

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30
Q

What does sarcoidosis affect?

A

Can affect multiple organs: most commonly lungs

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31
Q

Is there always a cutaneous manifestation of sarcoidosis?

A

Cutaneous manifestations in ~33%

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32
Q

What are the symptoms/ presentation of sarcoidosis?

A

*Highly variable – ‘the great mimicker’- have to exclude everything else first

  • Red-brown to violaceous papules and face, lips, upper back, neck, and extremities
    Lupus pernio – NB
    Ulcerative
    Scar sarcoid Erythema nodosum
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33
Q

What is the histology of sarcoidosis?

A
  • Histology–non-caseating epithelioid granulomas
34
Q

How do you diagnose sarcoidosis?

A

Diagnosis of exclusion

Requires evaluation for internal organ involvement

35
Q

What is DRESS?

A

Drug Reaction with Eosinophilia and Systemic Symptoms

Rash and systemic upset incorporating haematological and solid-organ disturbances

36
Q

How may someone with dress present?

A
37
Q

What is diagnosis of dress based on?

A

scoring criteria

38
Q

What is the criteria for diagnosis of dress?

A
  • Fever ≥ 38.5°C
  • L ymphadenopathy ⩾ 2 sites, > 1cm
  • Circulating atypical lymphocytes
  • Peripheral hypereosinophilia >0.7 × 109
  • Internal organs involved - (liver, kidneys, cardiac)
  • Negative ANA, Hepatitis / mycoplasma, chlamydia
  • Skin involvement
  • > 50% Body SA
  • Cutaneous eruption suggestive of DRESS
  • Biopsy suggestive of DRESS
39
Q

What are the internal organs involved with drug reaction with eosinophilia and systemic symptoms (dress)? (6)

A
  • Liver (hepatitis)
  • Kidneys (interstitial nephritis)
  • Heart (myocarditis)
  • Brain
  • Thyroid (thyroiditis)
  • Lungs (interstitial pneumonitis)
40
Q

What is the underlying mechanism of Drug Reaction with Eosinophilia and Systemic (dress)?

A

unk

41
Q

When does Drug Reaction with Eosinophilia and Systemic start?

A

2-6 weeks after drug exposure

42
Q

What is the most common internal organ involved with Drug Reaction with Eosinophilia and Systemic (dress)?

A

liver (most of deaths associated with this)

43
Q

What are common triggers for Drug Reaction with Eosinophilia and Systemic (dress)?

A

Sulfonamides,
anti-epileptics (carbamazepine, phenytoin,
lamotrigine),
allopurinol,
Antibiotics (vancomycin, amoxicillin, minocycline, piperacillin-tazobactam),
ibuprofen are common triggers

44
Q

What are the rash morphologies of Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS)?

A
  • Urticated papular exanthem
  • widespread papules
  • Maculopapular (morbilliform) eruption
  • Widespread erythema (Erythroderma)
  • Head / neck oedema
  • Erythema multiforme-like
45
Q

What is the treatment for dress?

A

Withdrawal of culprit

Corticosteroids are first line treatment - may require months of treatment

Mortality 5-10%

46
Q

What does alloimmune mean?

A

Alloimmunity is an immune response to nonself antigens from members of the same species

aka foreign immunity

47
Q

How can you know if a rash is caused by a drug or GvsHD?

A

face involvement
acral involvement
diarrhoea

(all indicate GvsHD)

48
Q

What does GvsHD stand for? And how would a patient present with GvsHD?

A

graft vs host disease

49
Q

What is GvsHD and who does it affect?

A

Multiple-organ disease

Affects ~10-80% of allogenic haematopoetic stem
cell transplants (HSCT)

50
Q

What is the pathogenesis of graft vs host disease?

A

donor-derived T-lymphocyte activity against antigens in an immunocompromised recipient

51
Q

What does graft vs host disease affect?

A

skin
liver
GI tract

52
Q

What is the word for sensation of itchiness?

A

pruritus

53
Q

What does it suggest if there is pruritus and no rash?

A

internal cause

54
Q

What are haematological causes of pruritus without rash?

A

lymphoma
polycythemia

55
Q

What are other causes of pruritus without rash? (8)

A
  • Uraemia
  • Cholestasis
  • Iron deficiency or iron overload
  • HIV/HepatitisA/B/C
  • Cancer
  • Drugs (NB opiates / opioids)
  • Psychogenic
  • Pruritus of old age
56
Q

What are investigations you do for pruritus?

A
  • FBC, LDH
  • Renal profile
  • Liver function tests
  • Ferritin
  • XR Chest
  • HIV/ Hepatitis A/ B/ C
57
Q

What happens from chronic scratching?

A

nodular prurigo

58
Q

What signifies metastases of a malignant carcinoid tumour?

A

Carcinoid syndrome

59
Q

What are symptoms of carcinoid syndrome?

A

diarrhoea
bronchospasm
hypotension
flushing (25% of cases)
wheezing
dizziness

60
Q

What is carcinoid syndrome?

A

Signifies metastases of a malignant carcinoid tumour
- 5-HT secretion

google=> Carcinoid syndrome occurs when a rare cancerous tumor called a carcinoid tumor secretes certain chemicals into your bloodstream

61
Q

How may someone with Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis present?

A
62
Q

Is Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis an emergency?

A

yes it is a derm emergency

63
Q

What is the prodromal of Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis?

A

flu like symptoms

64
Q

What are the symptoms Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis?

A

Abrupt onset of lesions on trunk > face/ limbs

Macules, blisters, erythema–atypical targetoid

Blisters merge–sheets of skin detachment ‘like wet
wallpaper’

65
Q

What is a macule?

A

A macule is a flat, distinct, discolored area of skin

66
Q

What are erythema–atypical targetoid?

A

Atypical target lesions show just two zones and/or an indistinct border. In erythema multiforme, these lesions are raised (papular)

67
Q

What happens in less than 2-3 days in Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis?

A

Extensive full thickness mucocutaneous (epidermal) necrosis <2-3 days

68
Q

How is Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis categorised?

A
69
Q

What are the differential diagnoses with Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis?

A

Staphylococcal scalded skin syndrome (SSSS)

Thermal burns

Cutaneous graft versus host disease

70
Q

What causes more than 80% of Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis cases?

A

drugs cause >80% of cases

71
Q

How long before the onset of the rash of Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis did taking the medications start?

A

up to 3 weeks prior to onset of rash

72
Q

What is the science behind Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis?

A

cell mediated cytotoxic reaction against epidermal cells

73
Q

What is the treatment for Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis?

A

antibiotics and NSAIDs

74
Q

What score is used to help assess severity of Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis and what is its criteria?

A

SCORTEN – score used to help assess severity

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

75
Q

What are the complications of Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis?

A

Death- Overall mortality 30%

Blindness,
dehydration,
hypothermia/hyperthermia,
renal tubular necrosis,
eroded GI tract,
interstitial pneumonitis, neutropaenia,
liver and heart failure

76
Q

What is erythroderma?

A

Generalized erythema affecting >90% BSA

77
Q

How may someone with erythroderma present?

A
78
Q

What do the systemic manifestations of erythroderma reflect?

A

impairment in skin function

79
Q

What are the systemic manifestations of erythroderma?

A
  • Peripheral edema
  • Tachycardia
  • Loss of fluid and proteins
  • Disturbances in thermoregulation
  • Risk of sepsis (loss of barrier)
80
Q

What are the etiologies of erythroderma?

A
  • Drug reactions
  • Cutaneous T-cell lymphoma – Sézary syndrome
  • Psoriasis
  • Atopic eczema
  • Idiopathic (25-30%)
81
Q

What is the management of 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 DERMATOLOGY (8 decks)

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