Managing skin disorders in systemic disease

Question 1

Pancytopenia

Answer 1

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

Question 2

Erythrocyte sedimentation rate (ESR)

Answer 2

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

Question 3

What is a punch biopsy

Answer 3

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

Question 4

Types of lupus erythematosus

Answer 4

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

Question 5

Overlap between lupus types

Answer 5

SCLE - Subacute Cutaneous Lupus Erythematosus

Question 6

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

Answer 6

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)

Question 7

What are some of the signs of lupus erythematosus?

Answer 7

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

Question 8

What is the characteristic feature of cutaneous lupus erythematosus?

Answer 8

Scarring, especially of the face
alopecia

Question 9

What presentation allows immediate diagnosis of neonatal lupus

Answer 9

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

Question 10

What is the risk of neonatal lupus patients?

Answer 10

50% risk heart block
ECG required upon diagnosis

Question 11

Investigations for SLE

Answer 11

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

Question 12

Alanine transaminase

Answer 12

ALT, indicates liver damage
investigation for dermatomyositis

Question 13

What is dermatomyositis?

Answer 13

Autoimmune connective tissue disease

Question 14

What are the two main effects of dermatomyositis?

Answer 14

Proximal Extensor Myopathy
Photodistributed Pink-Violet Rash

Question 15

Signs of dermatomyositis

Answer 15

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

Question 16

What is the relevance of the different autoantibodies

Answer 16

They predict the subtype and clinical features

Question 17

What autoantibodies are common in dermatomyositis?

Answer 17

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

Question 18

Investigations for diagnosing dermatomyositis

Answer 18

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

Question 19

What antibody is usually involved in vasculitis?

Answer 19

IgA

Question 20

What are the features of IgA vasculitis?

Answer 20

Abdominal pain, GI bleeding, glomerulonephritis, arthritis

Question 21

What is interesting about vasculitis

Answer 21

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

Question 22

What is sarcoidosis?

Answer 22

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

Question 23

Granuloma definition

Answer 23

Lumps, nodules caused by clusters of WBC

Question 24

What is a common facial sign of sarcoidosis?

Answer 24

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

Question 25

When would you consider diagnosing someone of sarcoidosis?

Answer 25

After excluding infection cutaneous TB + others have similar presentations

Question 26

Signs of sarcoidosis

Answer 26

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

Question 27

What does DRESS stand for?

Answer 27

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

Question 28

When can you diagnose DRESS

Answer 28

2-6wks post drug exposure

Question 29

What are the signs of DRESS?

Answer 29

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

Question 30

What is the most common organ affected by DRESS?

Answer 30

Liver - hepatitis

Question 31

What are the rash morphology characteristics of DRESS?

Answer 31

Maculopapular eruption, erythroderma multiforme

Question 32

What is the primary treatment of DRESS?

Answer 32

Corticosteroids can also withdraw causal drug

Question 33

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

Answer 33

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

Question 34

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

Answer 34

Face, Liver, Acral (digits) Involvement, Diarrhoea

Question 35

What are the skin signs of graft vs host disease?

Answer 35

Erythematous Macules, oral ulcers and Papules (lesions)

Question 36

What is Pruritus

Answer 36

Itching w/o a rash, suggestive of internal issues

Question 37

Causes of pruritus

Answer 37

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

Question 38

Investigations for pruritus

Answer 38

FBC - elevated LFTs ferritin CXR RFT HIV/Hep ABC

Question 39

Nodular prurigo

Answer 39

skin thickens into focused nodules

Question 40

What is carcinoid syndrome?

Answer 40

Systemic effects from metastases of malignant carcinoid tumour

Question 41

What are carcinoid tumours?

Answer 41

Tumours of neuroendocrine cells

Question 42

What are the symptoms of carcinoid syndrome?

Answer 42

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

Question 43

What is SJS? EMERGENCY

Answer 43

Steven Johnson Syndrome - cell-mediated cytotoxic reaction against epidermis

Question 44

What is the physiology of Steven Johnson Syndrome?

Answer 44

Painful rash, with flu-like symptoms

Question 45

Why can SJS lead to a big problem?

Answer 45

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

Question 46

What percentage of epidermal detachment is SJS classified as?

Answer 46

<10%

Question 47

What is the more serious version of SJS?

Answer 47

Toxic Epidermal Necrolysis

Question 48

What percentage of epidermal detachment is TEN classified as?

Answer 48

> 30%

Question 49

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

Answer 49

SJS-TEN

Question 50

What can cause SJS?

Answer 50

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

Question 51

How can you assess the severity of SJS?

Answer 51

SCORTEN scale

Question 52

Complications of SJS

Answer 52

blindness, liver/heart failure

Question 53

What is erythroderma?

Answer 53

Rash that covers 90% of the body

Question 54

What are the potential causes of erythroderma?

Answer 54

Drugs, Psoriasis, Atopic eczema, Idiopathic

Question 55

What are the signs of erythroderma?

Answer 55

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

Question 56

Management of erythroderma

Answer 56

treat underlying cause restore fluid/electrolyte balance steroids antibiotics