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

The Skin in Systemic Disease Flashcards

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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
A patient presents with these symptoms: What is the most likely diagnosis?
* **Dermatomyositis**
26
What is vasculitis?
* Vasculitis means **inflammation of the blood vessels** * For some reason the immune system attacks healthy blood vessels, causing them to become swollen and narrow ## Footnote * 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.
27
Name the presentation.
* **Purpura** (macular / palpable)
28
Name the presentation.
* **Digital necrosis**
29
Name the presentation.
* **Retiform purpura** and **ulcers**
30
Name the presentation.
* **Retiform purpura** and **ulcers**
31
Name the presentation.
* **Subcutaneous nodules** along blood vessels
32
What cutaneous manifestations would one expect in a vasculitis patient (5)?
* 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
33
A patient presents with these symptoms: What is the most likely diagnosis?
* **Vasculitis**
34
What is sarcoidosis?
* 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
35
What is the presentation?
* **Granulomas**
36
What is a granuloma?
* An **organised aggregation** or **activated macrophages** that forms in response to **chronic inflammation**
37
What is the presentation?
* **Scar Scaroid**
38
What is the presentation?
* **Lupus Pernio**
39
What cutaneous manifestations would one expect in a sarcoidosis patient (5)? ## Footnote **Diagnosis of exclusion** + check for **internal organ involvement**
* **Red-brown violaceous papules** of face, lips, upper back, neck, extremities * **Lupus Pernio** * **Ulcerative** * **Scar Sarcoid** * Non-caseating epithelioid **granulomas** ## Footnote The great mimicker - very variable
40
A patient presents with these symptoms: What is the most likely diagnosis?
Sarcoidosis
41
What is systemic amyloidosis?
* 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**
42
What non-cutaneous manifestations would one expect in a systemic amyloidosis patient (5)?
* Weight loss * Fatigue * Paraesthesias * Dyspnoea * Syncopal attacks
43
What cutaneous manifestations would one expect in a systemic amyloidosis patient (1)?
* Periorbital purpura **(raccoon's eyes)**
44
What is the presentation?
* Periorbital purpura **(raccoon's eyes)**
45
A patient presents with these symptoms: * Weight loss * Fatigue * Paraesthesias * Dyspnoea * Syncopal attacks What is the most likely diagnosis?
* **Systemic amyloidosis**
46
What is graft versus host disease (GvHD)?
* **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)
47
A patient presents with these symptoms: What is the most likely diagnosis?
* **Graft versus host disease** (GvHD) ## Footnote How can you if a rash is caused by a drug or GvHD? * Face or acral involvement * Diarrhoea * All indicate GHD more likely
48
What is drug reaction with eosinophilia and systemic symptoms (DRESS)?
* 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
What cutaneous manifestations would one expect in a drug reaction with eosinophilia and systemic symptoms (DRESS) patient (1)?
* Face, upper trunk and extremities are initial sites of involvement * **Characteristic rash** e.g. facial oedema * **Widespread rash > 50% BSA**
50
What non-cutaneous manifestations would one expect in a drug reaction with eosinophilia and systemic symptoms (DRESS) patient (4)?
* **Fever** ≥ 38.5oC * **Rash** * **Lymphadenopathy** * **Peripheral eosinophilia** > 0.7 × 109 ## Footnote Internal organ involvement: * Liver (hepatitis, most frequent cause of death) * Kidneys (interstitial nephritis) * Heart (myocarditis) * Brain * Thyroid (thyroiditis) * Lungs (interstitial pneumonitis)
51
A patient presents with these symptoms: * Fever ≥ 38.5oC * Rash * Lymphadenopathy * Peripheral eosinophilia > 0.7 × 109 What is the most likely diagnosis?
* Drug reaction with eosinophilia and systemic symptoms **(DRESS)**
52
What is the management of drug reaction with eosinophilia and systemic symptoms (DRESS) (2)?
* **Withdrawal of culprit medication** * **Corticosteroids** are first line - may require months of treatment
53
What is stevens-johnson syndrome / toxic epidermal necrolysis?
* **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
What cutaneous manifestations would one expect in a stevens-johnson syndrome / toxic epidermal necrolysis patient (1)?
* Extensive full thickness **mucocutaneous necrosis** < 2-3 days
55
What non-cutaneous complications would one expect in a stevens-johnson syndrome / toxic epidermal necrolysis patient (8)?
* Blindness * Dehydration * Hypothermia / hyperthermia * Renal tubular necrosis * Eroded GI tract * Interstitial pneumonitis * Neutropenia * Liver and heart failure
56
A patient presents with these symptoms: What is the most likely diagnosis?
* **Stevens-johnson syndrome** / toxic epidermal necrolysis
57
What is erythroderma?
* **Generalized erythema affecting >90% BSA** * Multiple etiologies: * Drug reactions * Cutaneous T-cell lymphoma - Sézary syndrome * Psoriasis * Atopic eczema * Idiopathic (25-30%)
58
What cutaneous manifestations would one expect in an erythoderma patient (1)?
* Generalized erythema affecting **> 90% BSA**
59
What non-cutaneous manifestations would one expect in an erythoderma patient (4)?
* **Tachycardia** * **Loss of fluid and proteins** * **Disturbances** in **thermoregulation** * Risk of **sepsis**
60
A patient presents with these symptoms: What is the most likely diagnosis?
* **Erythoderma**
61
What is the management of erythoderma (4)?
* **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
What is the presentation?
* **Pyoderma gangrenosoum**
63
What is the presentation?
* **Erythema nodosum** (Panniculitis)
64
What are the cutaneous diseases associated with inflammatory bowel disease (IBD) (2)?
* **Pyoderma gangrenosoum** * **Erythema nodosum** (Panniculitis)
65
A patient presents with these symptoms: What is the most likely diagnosis?
* Inflammatory bowel disease **(IBD)**
66
What is the presentation?
* **Dermatitis herpetiformis**
67
What is the cutaneous manifestation of celiac disease?
* **Dermatitis herpetiformis**
68
A patient presents with these symptoms: What is the most likely diagnosis?
* **Celiac disease**
69
What is the presentation?
* **Petechiae**, **ecchymoses**, **corkscrew hairs** with **perifollicular haemorrhage** & **follicular hyperkeratosis**
70
What is the cutaneous manifestation of Vitamin C deficiency (scurvy) (5)?
* Bleeding, erosion of gums * Petechiae * Ecchymoses * Corkscrew hairs with perifollicular haemorrhage * Follicular hyperkeratosis
71
A patient presents with these symptoms: What is the most likely diagnosis?
* **Vitamin C deficiency** (scurvy)
72
What is the presentation?
* **Dermatitis**
73
What is the cutaneous and non-cutaneous manifestation of zinc deficiency (3)?
* Dermatitis * Diarrhoea * Depression
74
A patient presents with these symptoms: * Diarrhoea * Depression What is the most likely diagnosis?
* **Zinc deficiency**
75
What is the presentation?
* **Dermatitis**
76
What is the cutaneous and non-cutaneous manifestation of Vitamin B3 deficiency (niacin) (4)?
* Dermatitis * Diarrhoea * Dementia * Death
77
A patient presents with these symptoms: * Diarrhoea * Dementia What is the most likely diagnosis?
* **Vitamin B3 deficiency** (niacin)
78
What is the presentation?
* **Excoriations** / **prurigo**
79
What is the presentation?
* **Xerosis**
80
What is the presentation?
* **Calciphylaxis** ## Footnote * 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
What is the presentation?
* **Half and half nails**
82
What cutaneous manifestations would one expect in a chronic kidney disease (CKD) patient (4)?
* Excoriations, prurigo * Xerosis * Calciphylaxis * Half and half nails
83
A patient presents with these symptoms: * Anaemia What is the most likely diagnosis?
* **Chronic Kidney Disease** (CKD)
84
What is the presentation?
* **Muehrcke’s lines**
85
What is the presentation?
* **Jaundice**
86
What is the presentation?
* **Porphyria cutaneous tarda**
87
What is the presentation?
* **Clubbing**
88
What is the presentation?
* **Terry’s nails**
89
What is the presentation?
* **Spider telangiectasia**
90
What is the presentation?
* **Palmar erythema**
91
What cutaneous manifestations would one expect in a chronic liver disease patient (7)?
* Excoriations, prurigo * Jaundice * Muehrcke's lines of nails * Terry's nails * Palmar ervthema * Spider telangiectasia * Clubbing
92
A patient presents with these symptoms: What is the most likely diagnosis?
* **Chronic liver disease**
93
What is the presentation?
* **Kaposis' Sarcoma**
94
What is the presentation?
* **Terry’s nails**
95
What is the presentation?
* **Granuloma annulare**
96
What is the presentation?
* **Neuropathic ulcers**
97
What is the presentation?
* **Skin infections**
98
What is the presentation?
* **Xerosis**
99
What is the presentation?
* **Xanthelesma** & **Xanthomata**
100
What does the presence of xanthelesma & xanthomata suggest?
* **Hyperlipidemia**
101
What is the presentation?
* **Acanthosis nigricans**
102
What is the presentation?
* **Necrobiosis lipoidica**
103
What cutaneous manifestations would one expect in a diabetes mellitus patient (8)?
* Terry’s nails * Granuloma annulare * Neuropathic ulcers * Skin infections * Xerosis * Xanthelesma & xanthomata * Acanthosis nigricans * Necrobiosis lipoidica
104
A patient presents with these symptoms: What is the most likely diagnosis?
* **Diabetes Mellitus**
105
What is the presentation? What diagnosis does it suggest?
* **Pre-tibial myxoedema** * **Grave’s disease**
106
What is the presentation? What diagnosis does it suggest?
* **Hyperpigmentation** * **Addison’s disease**
107
What is the presentation? What diagnosis does it suggest (3)?
* **Acne** * **Acromegaly** / **Cushing’s** syndrome / Polycystic ovarian syndrome **(PCOS)**
108
What is the presentation? What diagnosis does it suggest?
* **Cutis gyrata verticis** * **Acromegaly**
109
A patient presents with epilepsy, flu-like illness, sore eyes and oral ulceration followed by extensive painful rash. What is it likely to be?
Stevens-Johnson syndrome/ Toxic epidermal necrolysis
110
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?
DRESS
111
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?
Vasculitis
112
A patient presents withlong standing histiry of psoriasis, flare following streptococcal threat, worsened. What is it likely to be?
Erythroderma
113
A patient presents with flushing, diarrhoea, wheezing and dizziness. What is it likely to be?
Carcinoid syndrome
114
A patient presents with a rash, fever, arthritis and fatigue, what is it likely to be?
SLE
115
A patient presents with rash on upper arm, longstanding dry cough, joint pain. What is it likely to be?
Sarcoidosis
116
A patient presents with pruritus (itch), no rash, anti histamines unhelpful, polycythemia. What is it likely to be?
Pruritus
117
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?
Dermatomyositis
118
A patient presents with extensive rash after stem sell transplant with diarrhoea, extensive rash, oral ulceration. What is it likely to be?
Graft versus host disease

DERMATOLOGY phase 1b (6 decks)

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