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

The Skin in Systemic Disease 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.

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2
Q

What are the 2 main types of lupus erythematosous?

A

Systemic Lupus Erythematosus (SLE)
Cutaneous (Discoid) Lupus Erythematosus

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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

What are signs of Lupus erythematous in bloods?

A

Pancytopenia
Proteinuria
Increased ESR/CRP
ANA high
dsDNA positive

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5
Q

Name the presentation.

A

Chilblains

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6
Q

Name the presentation.

A
  • Photodistributed (sun-exposed areas) erythematous rash
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7
Q

Name the presentation.

A
  • Livedo reticularis
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8
Q

Name the presentation.

A
  • Subacute cutaneous lupus
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9
Q

Name the presentation.

A
  • Palpable purpura
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10
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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11
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A
  • Systemic lupus erythematosous (SLE)
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12
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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13
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A
  • Cutaneous (discoid) lupus erythematosou
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14
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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15
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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16
Q

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

A

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

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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17
Q

Name the presentation.

A
  • Gottron’s papules
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18
Q

Name the presentation.

A
  • Ragged cuticles
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19
Q

Name the presentation.

A
  • Digital ulcers
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20
Q

Name the presentation.

A
  • Shawl sign
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21
Q

Name the presentation.

A
  • Photosensitive erythema
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22
Q

Name the presentation.

A
  • Heliotrope rash
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23
Q

Name the presentation.

A
  • V-sign
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24
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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25
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A
  • Dermatomyositis
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26
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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27
Q

Name the presentation.

A
  • Purpura (macular / palpable)
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28
Q

Name the presentation.

A
  • Digital necrosis
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29
Q

Name the presentation.

A
  • Retiform purpura and ulcers
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30
Q

Name the presentation.

A
  • Retiform purpura and ulcers
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31
Q

Name the presentation.

A
  • Subcutaneous nodules along blood vessels
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32
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
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33
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A
  • Vasculitis
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34
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
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35
Q

What is the presentation?

A
  • Granulomas
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36
Q

What is a granuloma?

A
  • An organised aggregation or activated macrophages that forms in response to chronic inflammation
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37
Q

What is the presentation?

A
  • Scar Scaroid
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38
Q

What is the presentation?

A
  • Lupus Pernio
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39
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
  • Scar Sarcoid
  • Non-caseating epithelioid granulomas

The great mimicker - very variable

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40
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A

Sarcoidosis

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41
Q

What is systemic amyloidosis?

A
  • A group of rare, serious conditions caused by a build-up of an abnormal protein called amyloid in organs and tissues throughout the body
    • The build-up of amyloid proteins (deposits) can make it difficult for the organs and tissues to work properly
  • Without treatment, this can lead to organ failure
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42
Q

What non-cutaneous manifestations would one expect in a systemic amyloidosis patient (5)?

A
  • Weight loss
  • Fatigue
  • Paraesthesias
  • Dyspnoea
  • Syncopal attacks
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43
Q

What cutaneous manifestations would one expect in a systemic amyloidosis patient (1)?

A
  • Periorbital purpura (raccoon’s eyes)
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44
Q

What is the presentation?

A
  • Periorbital purpura (raccoon’s eyes)
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45
Q

A patient presents with these symptoms:
* Weight loss
* Fatigue
* Paraesthesias
* Dyspnoea
* Syncopal attacks

What is the most likely diagnosis?

A
  • Systemic amyloidosis
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46
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)
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47
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

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48
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%
    • Sulfonamides, anti-epileptics (carbamazepine, phenytoin, lamotrigine), allopurinol, Antibiotics (vancomycin, amoxicillin, minocycline, piperacillin-tazobactam), ibuprofen are common triggers
    • Starts 2-6 weeks after drug exposure
49
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
50
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)

51
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)
52
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
53
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
54
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
55
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
56
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A
  • Stevens-johnson syndrome / toxic epidermal necrolysis
57
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%)
58
Q

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

A
  • Generalized erythema affecting > 90% BSA
59
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
60
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A
  • Erythoderma
61
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
62
Q

What is the presentation?

A
  • Pyoderma gangrenosoum
63
Q

What is the presentation?

A
  • Erythema nodosum (Panniculitis)
64
Q

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

A
  • Pyoderma gangrenosoum
  • Erythema nodosum (Panniculitis)
65
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A
  • Inflammatory bowel disease (IBD)
66
Q

What is the presentation?

A
  • Dermatitis herpetiformis
67
Q

What is the cutaneous manifestation of celiac disease?

A
  • Dermatitis herpetiformis
68
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A
  • Celiac disease
69
Q

What is the presentation?

A
  • Petechiae, ecchymoses, corkscrew hairs with perifollicular haemorrhage & follicular hyperkeratosis
70
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
71
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A
  • Vitamin C deficiency (scurvy)
72
Q

What is the presentation?

A
  • Dermatitis
73
Q

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

A
  • Dermatitis
  • Diarrhoea
  • Depression
74
Q

A patient presents with these symptoms:
* Diarrhoea
* Depression

What is the most likely diagnosis?

A
  • Zinc deficiency
75
Q

What is the presentation?

A
  • Dermatitis
76
Q

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

A
  • Dermatitis
  • Diarrhoea
  • Dementia
  • Death
77
Q

A patient presents with these symptoms:
* Diarrhoea
* Dementia

What is the most likely diagnosis?

A
  • Vitamin B3 deficiency (niacin)
78
Q

What is the presentation?

A
  • Excoriations / prurigo
79
Q

What is the presentation?

A
  • Xerosis
80
Q

What is the presentation?

A
  • Calciphylaxis

  • Calciphylaxis: a serious, uncommon disease, calcium accumulates in small blood vessels of the fat and skin.
  • Causes blood clots, painful skin ulcers and may cause serious infections that can lead to death
81
Q

What is the presentation?

A
  • Half and half nails
82
Q

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

A
  • Excoriations, prurigo
  • Xerosis
  • Calciphylaxis
  • Half and half nails
83
Q

A patient presents with these symptoms:
* Anaemia

What is the most likely diagnosis?

A
  • Chronic Kidney Disease (CKD)
84
Q

What is the presentation?

A
  • Muehrcke’s lines
85
Q

What is the presentation?

A
  • Jaundice
86
Q

What is the presentation?

A
  • Porphyria cutaneous tarda
87
Q

What is the presentation?

A
  • Clubbing
88
Q

What is the presentation?

A
  • Terry’s nails
89
Q

What is the presentation?

A
  • Spider telangiectasia
90
Q

What is the presentation?

A
  • Palmar erythema
91
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
92
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A
  • Chronic liver disease
93
Q

What is the presentation?

A
  • Kaposis’ Sarcoma
94
Q

What is the presentation?

A
  • Terry’s nails
95
Q

What is the presentation?

A
  • Granuloma annulare
96
Q

What is the presentation?

A
  • Neuropathic ulcers
97
Q

What is the presentation?

A
  • Skin infections
98
Q

What is the presentation?

A
  • Xerosis
99
Q

What is the presentation?

A
  • Xanthelesma & Xanthomata
100
Q

What does the presence of xanthelesma & xanthomata suggest?

A
  • Hyperlipidemia
101
Q

What is the presentation?

A
  • Acanthosis nigricans
102
Q

What is the presentation?

A
  • Necrobiosis lipoidica
103
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
104
Q

A patient presents with these symptoms:

What is the most likely diagnosis?

A
  • Diabetes Mellitus
105
Q

What is the presentation?

What diagnosis does it suggest?

A
  • Pre-tibial myxoedema
  • Grave’s disease
106
Q

What is the presentation?

What diagnosis does it suggest?

A
  • Hyperpigmentation
  • Addison’s disease
107
Q

What is the presentation?

What diagnosis does it suggest (3)?

A
  • Acne
  • Acromegaly / Cushing’s syndrome / Polycystic ovarian syndrome (PCOS)
108
Q

What is the presentation?

What diagnosis does it suggest?

A
  • Cutis gyrata verticis
  • Acromegaly
109
Q

A patient presents with epilepsy, flu-like illness, sore eyes and oral ulceration followed by extensive painful rash. What is it likely to be?

A

Stevens-Johnson syndrome/ Toxic epidermal necrolysis

110
Q

A patient presents with multiple myeloma, rash, fever, tachycardia, lfts deranged, deteriorating renal function and recently treated with antibiotics. What is it likely to be?

A

DRESS

111
Q

A patient presents with GI bleed, lung cancer, lower leg rash, FSR ESR U&E normal. Urinalysis 50-100 RBCs. What is it likely to be?

A

Vasculitis

112
Q

A patient presents withlong standing histiry of psoriasis, flare following streptococcal threat, worsened. What is it likely to be?

A

Erythroderma

113
Q

A patient presents with flushing, diarrhoea, wheezing and dizziness. What is it likely to be?

A

Carcinoid syndrome

114
Q

A patient presents with a rash, fever, arthritis and fatigue, what is it likely to be?

A

SLE

115
Q

A patient presents with rash on upper arm, longstanding dry cough, joint pain. What is it likely to be?

A

Sarcoidosis

116
Q

A patient presents with pruritus (itch), no rash, anti histamines
unhelpful, polycythemia. What is it likely to be?

A

Pruritus

117
Q

A patient presents with a new rash across her face, chest, upper back and dorsal hand, weakness, weight loss and fatigue. Additionally increased ALT and CK. What is it likely to be?

A

Dermatomyositis

118
Q

A patient presents with extensive rash after stem sell transplant with diarrhoea, extensive rash, oral ulceration. What is it likely to be?

A

Graft versus host disease

DERMATOLOGY phase 1b (6 decks)

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