Managing skin disorders in systemic disease Flashcards

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1
Q

Pancytopenia

A

Deficiency of all blood components
-> RBC, WBC, platelets

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2
Q

Erythrocyte sedimentation rate (ESR)

A

Measures the speed of RBC settling in tube
The higher the ESR, the greater the inflammation

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3
Q

What is a punch biopsy

A

Skin sample sent to histology to check for antibodies and tissue culture

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4
Q

Types of lupus erythematosus

A

SLE - Systemic lupus erythematosus
CLE - Cutaneous lupus erythematosus (Discoid)

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5
Q

Overlap between lupus types

A

SCLE - Subacute Cutaneous Lupus Erythematosus

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6
Q

What are the regions of systemic effects in systemic lupus erythematosus?
HOMI

A

Haematology (low blood cells) - Haemolytic anaemia, thrombocytopenia, leukopenia (^ESR)
Organs - serositis, synovitis, arthritis, renal + neural issues
Mucocutaneous - ulcers, alopecia
Immunological - ANA (anti-nuclear antibody), Anti-dsDNA (double stranded)

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7
Q

What are some of the signs of lupus erythematosus?

A

Chilblains (itchy swelling),
Photodistributed rash,
Cutaneous vasculitis (blood vessel inflammation)
Livedo reticularis (discoloration due to disturbed blood flow)
Palpable purpura
Alopecia

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8
Q

What is the characteristic feature of cutaneous lupus erythematosus?

A

Scarring, especially of the face
alopecia

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9
Q

What presentation allows immediate diagnosis of neonatal lupus

A

Subacute cutaneous lupus
Anti-ro/la antibodies
ring like/ annular pattern

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10
Q

What is the risk of neonatal lupus patients?

A

50% risk heart block
ECG required upon diagnosis

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11
Q

Investigations for SLE

A

FBC
U&E,
ANA,
anti-dsDNA,
complement,
urinalysis

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12
Q

Alanine transaminase

A

ALT, indicates liver damage
investigation for dermatomyositis

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13
Q

What is dermatomyositis?

A

Autoimmune connective tissue disease

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14
Q

What are the two main effects of dermatomyositis?

A

Proximal Extensor Myopathy
Photodistributed Pink-Violet Rash

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15
Q

Signs of dermatomyositis

A

Ragged cuticles
Shawl sign - rash of the upper trunk
Heliotrope rash - round eyes
Gottron’s papules - red plaques on DIP,MCPs

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16
Q

What is the relevance of the different autoantibodies

A

They predict the subtype and clinical features

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17
Q

What autoantibodies are common in dermatomyositis?

A

Malignancy - anti-p155
Interstitial lung disease/ulcers/ischaemia - anti-MDA5

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18
Q

Investigations for diagnosing dermatomyositis

A

ANA - antinuclear antibody, indicator of autoimmunity
CK - indicates muscular degeneration
Skin biopsy
LFT (ALT) - Liver fct w/ alanine transaminase
EMG - Electromyography, measures muscle response to electric activity
Screening for internal malignancy

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19
Q

What antibody is usually involved in vasculitis?

A

IgA

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20
Q

What are the features of IgA vasculitis?

A

Abdominal pain, GI bleeding, glomerulonephritis, arthritis

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21
Q

What is interesting about vasculitis

A

Affects dif sized vessels w/ dif effects for each
small - Purpura(macular/palpable), caused by internal bleeding

medium - digital necrosis
- subcutaneous nodules along blood vessels
- retiform purpura ulcers

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22
Q

What is sarcoidosis?

A

Non-infectious systemic granulomatous disorder, affects multiple organ systems esp lungs

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23
Q

Granuloma definition

A

Lumps, nodules caused by clusters of WBC

24
Q

What is a common facial sign of sarcoidosis?

A

lupus pernio - Redness of nose and mouth, with small blisters

25
Q

When would you consider diagnosing someone of sarcoidosis?

A

After excluding infection
cutaneous TB + others have similar presentations

26
Q

Signs of sarcoidosis

A

Lupus Pernio
granulomatous papules
hypopigmented areas
scar sarcoid
Panniculitis - subcutaneous fat inflammation

27
Q

What does DRESS stand for?

A

Drug Reaction w/ Eosinophilia & Systemic Symptoms
Rash and systemic dysfunction diagnosed with scoring mechanism

28
Q

When can you diagnose DRESS

A

2-6wks post drug exposure

29
Q

What are the signs of DRESS?

A

Fever, lymphadenopathy, eosinophilia, multi-organ involvement (liver, kidney, heart, brain, thyroid, lungs), swelling of head/neck

30
Q

What is the most common organ affected by DRESS?

A

Liver - hepatitis

31
Q

What are the rash morphology characteristics of DRESS?

A

Maculopapular eruption, erythroderma multiforme

32
Q

What is the primary treatment of DRESS?

A

Corticosteroids
can also withdraw causal drug

33
Q

What is a systemic skin condition that occurs after a graft?

A

Graft vs Host Disease
Alloimmune disease (donor T cells vs host antigens)

34
Q

How would you ensure this was not a drug-induced/autoantibody condition?

A

Face, Liver, Acral (digits) Involvement, Diarrhoea

35
Q

What are the skin signs of graft vs host disease?

A

Erythematous Macules, oral ulcers and Papules (lesions)

36
Q

What is Pruritus

A

Itching w/o a rash, suggestive of internal issues

37
Q

Causes of pruritus

A

Haematology - lymphoma, polycythaemia
Uraemia - renal failure -> ^ blood in urea
Cholestasis - slow bile flow from liver
Iron deficiency/ overload
HIV/Hep ABC
Cancer
Drugs
Psychogenic
Old age

38
Q

Investigations for pruritus

A

FBC - elevated
LFTs
ferritin
CXR
RFT
HIV/Hep ABC

39
Q

Nodular prurigo

A

skin thickens into focused nodules

40
Q

What is carcinoid syndrome?

A

Systemic effects from metastases of malignant carcinoid tumour

41
Q

What are carcinoid tumours?

A

Tumours of neuroendocrine cells

42
Q

What are the symptoms of carcinoid syndrome?

A

Flushing, diarrhoea, bronchospasm, hypotension
5HT (serotonin) secretion

43
Q

What is SJS?
EMERGENCY

A

Steven Johnson Syndrome - cell-mediated cytotoxic reaction against epidermis

44
Q

What is the physiology of Steven Johnson Syndrome?

A

Painful rash, with flu-like symptoms

45
Q

Why can SJS lead to a big problem?

A

Blisters merge together, leading to epidermal detachment (also known as wet wallpaper like)

46
Q

What percentage of epidermal detachment is SJS classified as?

A

<10%

47
Q

What is the more serious version of SJS?

A

Toxic Epidermal Necrolysis

48
Q

What percentage of epidermal detachment is TEN classified as?

A

> 30%

49
Q

What is the percentage in between of epidermal detachment classified as?

A

SJS-TEN

50
Q

What can cause SJS?

A

Drug induced - AB/NSAIDs/B lactams/Anti-epileptics
Thermal burns
GvHd

51
Q

How can you assess the severity of SJS?

A

SCORTEN scale

52
Q

Complications of SJS

A

blindness, liver/heart failure

53
Q

What is erythroderma?

A

Rash that covers 90% of the body

54
Q

What are the potential causes of erythroderma?

A

Drugs, Psoriasis, Atopic eczema, Idiopathic

55
Q

What are the signs of erythroderma?

A

Peripheral oedema, due ot protein loss in epidermis
tachycardia,
few fluid/proteins
disrupted thermoregulation,
sepsis due to loss of microbiological barrier

56
Q

Management of erythroderma

A

treat underlying cause
restore fluid/electrolyte balance
steroids
antibiotics