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BRS DERMATOLOGY > 1b The Skin in Systemic Disease > Flashcards

1b The Skin in Systemic Disease Flashcards

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Dermatology Board Prep flashcards
1
Q

What is systemic lupus erythematosous (SLE)?

A
  • Systemic lupus erythematosus (SLE) is an autoimmune disease. In this disease, the immune system of the body mistakenly attacks healthy tissue. It can affect the skin, joints, kidneys, brain, and other organs, causing widespread inflammation and tissue damage.
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2
Q

What are the 3 main types of lupus erythematosous?

A
  • Systemic Lupus Erythematosus (SLE)
  • Cutaneous (Discoid) Lupus Erythematosus
  • Neonatal Lupus
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3
Q

What investigations are suggested in suspected systemic lupus erythematosous (SLE) (6)?

A
  • ANA anti nuclear antibodies
  • Anti-dsDNA
  • Anti-Sm
  • Antiphospholipid
  • Complement level (low!)
  • Direct Coomb’s test
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4
Q

Name the presentation.

A

Chilblains

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5
Q

Name the presentation.

A
  • Photodistributed (sun-exposed areas) erythematous rash
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6
Q

Name the presentation.

A
  • Livedo reticularis
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7
Q

Name the presentation.

A
  • Subacute cutaneous lupus
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8
Q

Name the presentation.

A
  • Palpable purpura
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9
Q

What cutaneous manifestations would one expect in a systemic lupus erythematosous (SLE) patient (6)?

A
  • Photodistributed rash
  • Cutaneous vasculitis
  • Chilblains
  • Alopecia
  • Livedo reticularis
  • Subacute cutaneous lupus (SCLE)
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10
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A
  • Systemic lupus erythematosous (SLE)
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11
Q

What cutaneous manifestations would one expect in a cutaneous (discoid) lupus erythematosous patient (2)?

A
  • Discoid lupus
  • Subacute cutaneous lupus (SCLE)
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12
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A
  • Cutaneous (discoid) lupus erythematosous
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13
Q

A newborn presents with these symptoms:

What is the most likely diagnosis?

A
  • Neonatal lupus

NB Test ECG – risk of heart block (50% risk)

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14
Q

What is dermatomyositis?

A
  • Dermatomyositis is a long-term inflammatory disorder which affects skin and the muscles
  • Its symptoms are generally a skin rash and worsening muscle weakness over time
    • Proximal extensor inflammatory myonathy
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15
Q

What investigations are recommended in suspected dermatomyositis (Bloods 3 / Specific tests 2)?

A

Bloods:
* ANA (anti nuclear antibodies this is the screening test, then do details or autoantibodies)
* Creatine kinase increased
* LFTs (ALT often increased due to muscle breakdown)

Specific tests:
* EMG (Electromyography)
* Diagnostic procedure that evaluates the health condition of muscles and the nerve cells that control them
* Skin biopsy

Autoantibody profile for specific subtype
* Anti Jo-1 – fever, myositis, gottron’s papules
* Anti SRP – nectrotising myopathy
* Anti Mi-2 – mild muscle disease
* Anti-p155 – associated with malignancy (in adults)
* Anti-p140 – juvenile, associated with calcinosis
* Anti-SAE- +/- amyopathic
* Anti- MDA5 – interstitial lung disease, digital ulcers / ischaemia

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16
Q

Name the presentation.

A
  • Gottron’s papules
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17
Q

Name the presentation.

A
  • Ragged cuticles
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18
Q

Name the presentation.

A
  • Digital ulcers
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19
Q

Name the presentation.

A
  • Shawl sign
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20
Q

Name the presentation.

A
  • Photosensitive erythema
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21
Q

Name the presentation.

A
  • Heliotrope rash
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22
Q

Name the presentation.

A
  • V-sign
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23
Q

What cutaneous manifestations would one expect in a dermatomyositis patient (7)?

A
  • Gottron’s papules
  • Ragged cuticles
  • Digital ulcers
  • Shawl sign
  • Photosensitive erythema
  • Heliotrope rash
  • V-sign

Photo-distributed
Pink-violet rash
Extensor surfaces + periocular

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24
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A
  • Dermatomyositis
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25
Q

What is vasculitis?

A
  • Vasculitis means inflammation of the blood vessels
    • For some reason the immune system attacks healthy blood vessels, causing them to become swollen and narrow
No need to know this much detail.

  • This may be triggered by an infection or a medicine, although often the cause is unknown.
  • Vasculitis can range from a minor problem that just affects the skin, to a more serious illness that causes problems with organs like the heart or kidneys.
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26
Q

Name the presentation.

A
  • Purpura (macular / palpable)
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27
Q

Name the presentation.

A
  • Digital necrosis
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28
Q

Name the presentation.

A
  • Retiform purpura and ulcers
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29
Q

Name the presentation.

A
  • Retiform purpura and ulcers
30
Q

Name the presentation.

A
  • Subcutaneous nodules along blood vessels
31
Q

What cutaneous manifestations would one expect in a vasculitis patient (5)?

A
  • Purpura (macular / palpable) (ususally small vessel vasculitis unlike the rest of the symptoms that are medium to large)
  • Digital necrosis
  • Retiform purpura
  • Ulcers
  • Subcutaneous nodules along blood vessels
32
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A
  • Vasculitis
33
Q

What is sarcoidosis?

A
  • Sarcoidosis is a rare condition that causes small patches of red and swollen tissue, called granulomas, to develop in the organs of the body
  • It usually affects the lungs and skin
34
Q

What is the presentation?

A
  • Granulomas
35
Q

What is a granuloma?

A
  • An organised aggregation or activated macrophages that forms in response to chronic inflammation
36
Q

What is the presentation?

A
  • Scar Scaroid
37
Q

What is the presentation?

A
  • Lupus Pernio
38
Q

What cutaneous manifestations would one expect in a sarcoidosis patient (5)?

Diagnosis of exclusion + check for internal organ involvement

A
  • Red-brown violaceous papules of face, lips, upper back, neck, extremities
  • Lupus Pernio
  • Ulcerative lesions
  • Scar Sarcoid
  • Non-caseating epithelioid granulomas

The great mimicker - very variable

39
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A

Sarcoidosis

40
Q

What is graft versus host disease (GvHD)?

A
  • Multiple-organ disease
    • Affects ~10-80% of allogenic haematopoetic stem cell transplants (HSCT)
  • Pathogenesis: the donor T cells attack antigens in recipient (who is immunocompromised)
41
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A
  • Graft versus host disease (GvHD)

How can you if a rash is caused by a drug or GvHD?
* Face or acral involvement
* Diarrhoea
* All indicate GHD more likely

42
Q

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

A
  • Widespread serious systemic reaction with cutaneous, haematological and solid-organ disturbances
    • Underlying mechanism not known
    • Mortality 5-10%
    • Common triggers- ASIAA:
      1. Antibiotics (vancomycin, amoxicillin, minocycline, piperacillin-tazobactam)
      2. Sulfonamides
      3. Ibuprofen
      4. Anti-epileptics (carbamazepine, phenytoin, lamotrigine)
      5. Allopurinol (reduces uric acid levels)
    • Starts 2-6 weeks after drug exposure
43
Q

What cutaneous manifestations would one expect in a drug reaction with eosinophilia and systemic symptoms (DRESS) patient (1)?

A
  • Face, upper trunk and extremities are initial sites of involvement
  • Characteristic rash e.g. facial oedema
    • Widespread rash > 50% BSA
44
Q

What non-cutaneous manifestations would one expect in a drug reaction with eosinophilia and systemic symptoms (DRESS) patient (4)?

A
  • Fever ≥ 38.5oC
  • Rash
  • Lymphadenopathy
  • Peripheral eosinophilia > 0.7 × 109

Internal organ involvement:
* Liver (hepatitis, most frequent cause of death)
* Kidneys (interstitial nephritis)
* Heart (myocarditis)
* Brain
* Thyroid (thyroiditis)
* Lungs (interstitial pneumonitis)

45
Q

A patient presents with these symptoms:
* Fever ≥ 38.5oC
* Rash
* Lymphadenopathy
* Peripheral eosinophilia > 0.7 × 109

What is the most likely diagnosis?

A
  • Drug reaction with eosinophilia and systemic symptoms (DRESS)
46
Q

What is the management of drug reaction with eosinophilia and systemic symptoms (DRESS) (2)?

A
  • Withdrawal of culprit medication
  • Corticosteroids are first line - may require months of treatment
47
Q

What is stevens-johnson syndrome / toxic epidermal necrolysis?

A
  • Derm emergency! (Rare)
    • Overall mortality 30%
    • Prodromal: flu-like sx
  • Abrupt onset of lesions on trunk > face/limbs
    • Macules, blisters, erythema - atypical targetoid
    • Blisters merge - sheets of skin detachment
48
Q

What cutaneous manifestations would one expect in a stevens-johnson syndrome / toxic epidermal necrolysis patient (1)?

A
  • Extensive full thickness mucocutaneous necrosis < 2-3 days
49
Q

What non-cutaneous complications would one expect in a stevens-johnson syndrome / toxic epidermal necrolysis patient (8)?

A
  • Blindness
  • Dehydration
  • Hypothermia / hyperthermia
  • Renal tubular necrosis
  • Eroded GI tract
  • Interstitial pneumonitis
  • Neutropenia
  • Liver and heart failure
50
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A
  • Stevens-johnson syndrome / toxic epidermal necrolysis
51
Q

What is erythroderma?

A
  • Generalized erythema affecting >90% BSA
  • Multiple etiologies:
    • Drug reactions
    • Cutaneous T-cell lymphoma - Sézary syndrome
    • Psoriasis
    • Atopic eczema
    • Idiopathic (25-30%)
52
Q

What cutaneous manifestations would one expect in an erythoderma patient (1)?

A
  • Generalized erythema affecting > 90% BSA
53
Q

What non-cutaneous manifestations would one expect in an erythoderma patient (4)?

A
  • Tachycardia
  • Loss of fluid and proteins
  • Disturbances in thermoregulation
  • Risk of sepsis
54
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A
  • Erythoderma
55
Q

What is the management of erythoderma (4)?

A
  • Treat underlying cause (e.g. treat psoriasis, withdraw drug if drug cause)
  • Hospitalisation if systemically unwell
  • Restore fluid and electrolyte balance, circulatory status and manage body temperature
  • Emollients to support skin barrier
    • +/- Topical steroids
    • +/- Antibiotics
56
Q

What are the cutaneous diseases associated with inflammatory bowel disease (IBD) (2)?

A
  • Pyoderma gangrenosoum
  • Erythema nodosum (Panniculitis)
57
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A
  • Inflammatory bowel disease (IBD)
58
Q

What is the cutaneous manifestation of coeliac disease?

A
  • Dermatitis herpetiformis
59
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A
  • Coeliac disease
60
Q

What is the cutaneous manifestation of Vitamin C deficiency (scurvy) (5)?

A
  • Bleeding, erosion of gums
  • Petechiae
  • Ecchymoses
  • Corkscrew hairs with perifollicular haemorrhage
  • Follicular hyperkeratosis
61
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A
  • Vitamin C deficiency (scurvy)
62
Q

What is the cutaneous and non-cutaneous manifestation of zinc deficiency (3)?

A
  • Dermatitis
  • Diarrhoea
  • Depression
63
Q

A patient presents with these symptoms:
* Diarrhoea
* Depression

What is the most likely diagnosis?

A
  • Zinc deficiency
64
Q

What is the cutaneous and non-cutaneous manifestation of Vitamin B3 deficiency (niacin) (4)?

A
  • Dermatitis
  • Diarrhoea
  • Dementia
  • Death
65
Q

A patient presents with these symptoms:
* Diarrhoea
* Dementia

What is the most likely diagnosis?

A
  • Vitamin B3 deficiency (niacin)
66
Q

What cutaneous manifestations would one expect in a chronic kidney disease (CKD) patient (4)?

A
  • Excoriations, prurigo
  • Xerosis
  • Calciphylaxis
  • Half and half nails
67
Q

A patient presents with these symptoms:
* Anaemia

What is the most likely diagnosis?

A
  • Chronic Kidney Disease (CKD)
68
Q

What cutaneous manifestations would one expect in a chronic liver disease patient (7)?

A
  • Excoriations, prurigo
  • Jaundice
  • Muehrcke’s lines of nails
  • Terry’s nails
  • Palmar ervthema
  • Spider telangiectasia
  • Clubbing
69
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A
  • Chronic liver disease
70
Q

What does the presence of xanthelesma & xanthomata suggest?

A
  • Hyperlipidemia
71
Q

What cutaneous manifestations would one expect in a diabetes mellitus patient (8)?

A
  • Terry’s nails
  • Granuloma annulare
  • Neuropathic ulcers
  • Skin infections
  • Xerosis
  • Xanthelesma & xanthomata
  • Acanthosis nigricans
  • Necrobiosis lipoidica
72
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A
  • Diabetes Mellitus

BRS DERMATOLOGY (9 decks)

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