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the skin in systemic disease Flashcards

1
Q

why is the assessment of skin important?

A

Comprehensive assessment coupled with dermatological diagnostic skills can:
- Prevent or reduce internal organ damage by early diagnosis

- Allow detection of internal 	malignancy
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2
Q

what are skin targeted systemic disease and give an example

A

multi-organ systemic disease targetting the skin e.g. sarcoidosis

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3
Q

what are skin signs of systemic disease and give an example

A

sign of internal disorder e.g. flushing in carcinoid syndrome

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4
Q

what are ‘tell-tale’ skin conditions in systemic disease and give an example

A

Skin conditions suggestive of underlying condition e.g. Pyoderma gangrenosum in inflammatory bowel disease

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5
Q

what are 2ndry systemic involvement and give an example

A

Systemic disease secondary to skin disorder e.g. high output cardiac failure in erythroderma

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6
Q

what are the two main forms of Lupus erythematosus?

A

Systemic Lupus Erythematosus
Cutaneous (Discoid) Lupus Erythematosus
- Overlap

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7
Q

outline the mucocutaneous findings associated with systemic lupus erythematosus

A

Cutaneous lupus - acute
Cutaneous lupus - chronic
Oral ulcers
Alopecia

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8
Q

outline the haematological findings associated with systemic Lupus erythematosus

A

Haemolytic anaemia
Thrombocytopenia
Leukopenia

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9
Q

outline the immunological perimeters associated with systemic Lupus erythematosus

A

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

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10
Q

what are the skin features of systemic lupus erythematous?

A

photodistributed (sun-exposed areas) erythematous rash, chilblains, livedo reticularis, alopecia, subcutaneous cutaneous lupus (SCLE) , cutaneous vasculitis,

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11
Q

in what two ways does cutaneous (discoid) lupus erythematosus manifest?

A

discoid lupus erythematosus or subacute cutaneous lupus (SCLE)

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12
Q

what is the characteristic difference between Discoid lupus erythematosus and SCLE?

A

Discoid lupus erythematosus causes scarring unlike SCLE

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13
Q

what does it mean if SCLE is seen without lupus erythematosus?

A

is systemic or skin limited

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14
Q

what is the most common presentation of neonatal lupus?

A

The most common presentation is a nonscarring, nonatrophic skin lesion which resemble subacute cutaneous lupus erythematosus.

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15
Q

what test must be done for a patient with neonatal lupus erythematosus and why?

A

ECG as associated with a 50% chance of heart block

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16
Q

what is dermatomyositis?

A

Dermatomyositis is a rare autoimmune condition that causes muscle inflammation. It presents with symmetric proximal muscle weakness, skin rash, and extramacular manifestations, such as esophageal dysfunction and interstitial lung disease. Dermatomyositis is strongly associated with malignancy, especially in adults.

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17
Q

what are the typical manifestations of dermatomyositis?

A

gottrons papules, ragged cuticles, shawl sign, heliotrope rash, photosensitive erythema

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18
Q

How can subtypes of dermatomyositis be predicted?

A

by autoantibody profile

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19
Q

what autoantibody is most commonly associated with malignancy in dermatomyositis?

A

anti-p155

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20
Q

which autoantibody profile is most typically associated with interstitial lung disease, digital ulcers/ ischaemia?

A

anti-MDA5

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21
Q

other than autoantibody screen, what other markers can we use to diagnose dermatomyositis?

A

ANA, CK, skin biopsy, LFT - ALT often increased, EMG, screening for internal malignancy

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22
Q

what is the typical manifestation of IgA vasculitis? (Henoch-Schonlein purpura)

A

Abdominal pain, bleeding, arthralgia, arthritis, IgA- associated glomerulonephritis- may develop later

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23
Q

what must be monitored often and why in patients with IgA vasculitis (Henoch-Schonlein purpura)
?

A

urine samples to check for kidney function as glomerulonephritis can develop

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24
Q

what are the 2 classifications of vasculitis that effect small vessels?

A

cutaneous small vessel (leukocytoclastic) vasculitis, and small vessel vasculitis

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25
what vasculitis affects both small and medium blood vessels?>
cryoglobuminemia and ANCA-associated
26
what type of vasculitis affects medium vessels?
polyarteritis nodosa (PAN)
27
what type of vasculitis affects large vessels?
temporal arteritis tayakasu
28
what are the typical manifestations of a small vessel vasculitis vs a medium vessel vasculitis?
small vessel: purpura medium vessel: digital necrosis, retiform purpura ulcers, subcutaneous nodules along blood vessels,
29
what is sarcoidosis?
non-infectious, systemic granulomatous disorder of unknown origin
30
Lupus erythematosus, SLE and CLE, dermatomyositis, and vasculitis are all examples of what type of chronic multi system disorders?
Autoimmune/ auto inflammatory
31
Sarcoidosis and systemic amyloidosis are both examples of what type of chronic multi system disorder?
Idiopathic
32
Graft versus host disease is an example of what type of chronic multi system disorder?
Allo-immune
33
Systemic lupus erythematosus can affect which areas of the body?
Skin, joints, kidneys, brain and other organs
34
In dermatomyoitis what happens to muscles over time?
Worsening muscle weakness
35
What are often the triggers of vasculitis?
An infection or medicine, however the cause is often unknown
36
What is the general prognosis for IgA vasculitis?
Prognosis is favourable but depends on severity of renal disease; IgA vasculitis lasts up to 6 months in 1/3 of patients Persistent nephropathy occurs in 8%, progressive renal failure in 1-3%; those with haematuria or proteinuria should be carefully followed
37
What are the symptoms and signs of granulomatosis with polyangiitis?
Cough, dyspnoea, and chest pain Stuffy nose and nose bleeds Pulmonary infiltrates Glomerulonephritis
38
Cutaneous findings are present in roughly what percentage of granulomatosis with polyangiitis?
50%
39
What are the small vessel manifestations associated with Granulomatosis with polyangiitis?
Purpura (macular/ flat or popular/ palpable)
40
What are the medium vessel manifestations associated with granulomatosis with Polyangiitis?
Digital necrosis, livedo reticularis, subcutaneous nodules distributed along vessels, ulcers
41
What are the symptoms or sarcoidosis?
The symptoms of sarcoidosis depend on which organs are affected, but typically include: tender, red bumps on the skin - Red-brown to violaceous papules and face, lips, upper back, neck, and extremities shortness of breath and a persistent cough Ulcerative lesions, scar sarcoidosis, erythema nodosum
42
What is a granuloma?
Granuloma: A granuloma is an organised aggregation of activated macrophages that forms in response to chronic inflammation.
43
What investigations are conducted in the diagnosis of sarcoidosis?
Diagnosis of exclusion Histology–non-caseating epithelioid granulomas Requires evaluation for internal organ involvement
44
What is amyloidosis?
Amyloidosis is the name for 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.
45
What are the presenting symptoms associated with amyloidosis?
Presenting symptoms often varied and non-specific: - Weight loss - Fatigue - Paraesthesias - Dyspnoea - Syncopal attacks (orthostatic hypotension)
46
What causes amyloidosis?
Is the manifestation of underlying plasma cell dyscrasia
47
The skin is involved in what percentage of systemic amyloidosis cases?
25%
48
What are the cutaneous features associated with amyloidosis?
Petechiae, purpura and ecchymoses - due to infiltration of vessel walls Periorbital purpura: Bruising around eyes ( ‘Raccoon sign’) Waxy, translucent or purpuric papules, nodules Face, neck, and scalp, anogenital region, digits
49
What is a Graft versus host disease?
Multiple organ disease that affects 10-80% of allergenic haematopoeitic stem cell transplants
50
What is the pathogenesis behind graft versus host disease?
The donor T cell attacks antigens in recipient
51
What are the typical manifestations of Graft versus host disease?
Rash with face or acral involvement Mainly affects the skin, liver and GI tract, Diarrhoea
52
What are three examples of emergency reactions that present with skin signs?
Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis Erythroderma
53
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%
54
What are the common triggers of DRESS and how long after exposure does the reaction set in?
Sulfonamides, anti-epileptics (carbamazepine, phenytoin, lamotrigine), allopurinol, Antibiotics (vancomycin, amoxicillin, minocycline, piperacillin-tazobactam), ibuprofen are common triggers Starts 2-6 weeks after drug exposure
55
What are the common features associated with DRESS?
Features Fever ≥ 38.5°C Rash Lymphadenopathy Peripheral eosinophilia >0.7 × 109
56
How is DRESS treated?
Treatment: Withdrawal of culprit medication Corticosteroids are first line - may require months of treatment
57
What are the skin manifestations associated with DRESS?
Skin manifestations: Face, upper trunk and extremities are initial sites of involvement Characteristic rash e.g. facial oedema Widespread rash >50% BSA
58
How are the internal organs involved in DRESS?
Internal organ involvement Liver= hepatitis, most frequent cause of death Kidneys (interstitial nephritis) Heart (myocarditis)  Brain  Thyroid (thyroiditis)  Lungs (interstitial pneumonitis) 
59
What is Stevens-Johnson syndrome / toxic epidermal necrolysis?
Dermatological emergency with 30% mortality Prodromal: flu-like sx Abrupt onset of lesions on trunk > face/limbs Macules, blisters, erythema – atypical targetoid Blisters merge – sheets of skin detachment Extensive full thickness mucocutaneous necrosis <2-3 days - skin falls off like wall paper
60
What are the complications associated with Stevens-Johnson syndrome / toxic epidermal necrolysis?
Blindness, dehydration, hypothermia/hyperthermia, renal tubular necrosis, eroded GI tract, interstitial pneumonitis, neutropaenia, liver and heart failure
61
What is erythroderma?
Generalized erythema affecting >90% BSA
62
What are the signs and symptoms of erythroderma?
Generalized erythema affecting >90% BSA Impaired in skin function and consequences: - Peripheral edema - Tachycardia - Loss of fluid and proteins - Disturbances in thermoregulation - Risk of sepsis
63
What is the aetiology behind erythroderma?
Multiple etiologies: - Drug reactions - Cutaneous T-cell lymphoma – Sézary syndrome - Psoriasis - Atopic eczema - Idiopathic (25-30%)
64
How is erythroderma managed?
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
65
Skin conditions can be suggestive of what main underlying conditions ?
Cutaneous Manifestations of: GI conditions Nutritional deficiencies Chronic Kidney Disease Chronic Liver Disease Immunodeficiency Diabetes Mellitus Other Endocrinological Disorders
66
What cutaneous diseases are associated with inflammatory bowel disease?
Pyoderma gangrenosum Erythema nodosum (Panniculitis)
67
What are the cutaneous manifestations of celiac disease?
Dermatitis herpetiformis
68
A deficiency in vitamin C manifests on the skin how?
Vit C (ascorbic acid): scurvy: bleeding, erosion of gums, petechiae, ecchymoses,corkscrew hairs with perifollicular haemorrhage, follicular hyperkeratosis
69
What are the cutaneous manifestations associated with chronic kidney disease?
Anaemia – pallor, mucosal pallor, hair thinning Excoriations, prurigo 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 Half and half nails Look for signs related to primary disease ie Vasculitis, SLE
70
What are the cutaneous manifestations associated with chronic liver disease?
Excoriations, prurigo Jaundice Muehrcke’s lines of nails Terry’s nails Palmar erythema Spider telangiectasia Clubbing
71
What are the cutaneous manifestations associated with immunodeficiency?
Infections: Viral Warts, opportunistic infections, dermatophytosis… Abnormal Cancers: Kaposi’s sarcoma
72
What are the cutaneous manifestations associated with diabetes Mellitus?
Necrobiosis Lipoidica Acanthosis nigricans Xerosis Skin infections (more common because of diabetes) Neuropathic and vascular ulcers on feet
73
What are the cutaneous manifestations associated with Graves’ disease?
Pre-tibial myxoedema
74
What are the cutaneous manifestations associated with Addison’s disease?
Hyperpigmentation
75
What are the cutaneous manifestations associated with PCOS?
Acne
76
What are the cutaneous manifestations associated with both acromegaly and Cushing’s syndrome?
Acne
77
What are the cutaneous manifestations associated with hyperlipidaemia?
Eruptive xanthoma, xanthelesma and xanthomata
78
58 year old male Acute: Rash Fever Tachycardia Renal function deteriorating Liver function tests deranged Recently treated with co-amoxiclav for suspected infection – source not identified: No improvement Diagnosis?
DRESS - drug reaction with eosinophilia and systemic symptoms
79
What term is given to a lesion that is flat, doesnt involve any change in the thickness or texture of the skin that is less than 1cm?
A macule
80
what term is given to a lesion that is flat, doesnt involve any change in the thickness or texture of the skin and is larger than 1cm?
A patch
81
What term is given to a fluid-filled sac or lesion, fluid trapped under a thin layer of skin that is less than 1cm?
Vesicle
82
What name is given to a fluid filled sac or lesion, fluid trapped under a thin later of skin that is larger than 1cm?
Bulla
83
What name is given to a solid elevation of skin with no visible fluid that is less than 1cm?
Papule
84
What name is given to a solid elevation of skin with no visible fluid that is larger than 1cm?
A nodule
85
What term is given to a flat but raised lesion?
A plaque
86
what term is given to a nodule but filled with pus?
Pustule
87
What is a rash?
A widespread eruption of lesions
88
What is a maculopapular rash?
Made of both flat and raised skin lesions

Y2 DERM (4 decks)

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