The Skin In Systemic Disease

Question 1

In what four ways can the skin be involved in systemic disease?

Answer 1

Skin targeted
- Multi-organ systemic disease targeting skin, e.g. sarcoidosis
Skin signs
- Sign of internal disorder, e.g. flushing in Carcinoid syndrome
‘Tell-tale’ skin conditions
- Skin conditions suggestive of an underlying condition, e.g. Pyoderma gangrenosum in IBD
Secondary systemic involvement
- Systemic disease is secondary to skin disorder, e.g. high cardiac output failure in erythroderma

Question 2

What blood tests are run for investigation in dermatology?

Answer 2

FBC
Renal profile
LFT
Inflammatory markers
Autoimmune serology

Question 3

What microbiology tests are performed in dermatology?

Answer 3

Viral/bacterial serology
Swabs for bacteria C&S
Viral PCR tissue culture
PCR

Question 4

What does imaging investigate I’m dermatology?

Answer 4

Internal organ involvement
Vascular supply

Question 5

Aside from blood tests, microbiology and imaging, what other clinical tests are performed in dermatology?

Answer 5

Skin biopsy - microscopy
Specific tests - urinalysis, nerve conduction studies, endocrine investigations

Question 6

What technique is observed in the image below?

Answer 6

Punch biopsy - sample of skin is taken under local anaesthetic
Sample can be sent for histology, tissue culture and antibody analysis

Question 7

What are the two main groups of lupus erythematosus and are they distinct?

Answer 7

Systemic Lupus Erythematosus
Cutaneous (discoid) Lupus Erythematosus
There is an overlap between the groups

Question 8

What categories do the diagnostic criteria of SLE fit under?

Answer 8

Mucocutaneous
Involvement of the joints and organs
Haematological - specifically reduced RBC count
Immunological - presence of antibodies

Question 9

What does this image show?

Answer 9

Photodistributed (sun-exposed areas) erythematous rash
Symptom of SLE

Question 10

What does this image show?

Answer 10

Chilblains (itchy swellings)
Found in SLE

Question 11

What are the features of SLE?

Answer 11

Photodistributed rash
Cutaneous vasculitis (inflammation of blood vessels)
Chilblains
Alopecia (hair loss)
Livedo reticularis (net-like redness)
Subacute cutaneous lupus (SCLE) - ring/annular pattern

Question 12

What does this image show?

Answer 12

Livedo reticularis

Question 13

What does this image show?

Answer 13

Subacute cutaneous lupus

Question 14

What does this image show?

Answer 14

Palpable purpura

Question 15

What are the features of Cutaneous Lupus Erythematosus?

Answer 15

Discoid lupus erythematosus
SCLE (overlap)

Question 16

What is the difference between discoid lupus and subacute lupus?

Answer 16

Discoid lupus characteristically causes scarring and SCLE doesn’t

Question 17

Does SCLE always present with SLE?

Answer 17

No, can be systemic (SLE) or skin-limited (cutaneous lupus)

Question 18

What is the underlying disorder in this picture and what test should be performed?

Answer 18

Neonatal lupus (Ro positive)
ECG should be performed - up to 50% risk of heart block

Question 19

What is dermatomyositis and what are its key features?

Answer 19

Autoimmune connective tissue disease
Proximal extensor inflammatory myopathy
Photo-distributed pink-violet rash favouring scalp, periocular region and extensor surfaces

Question 20

Label these signs. What condition are they characteristic of?

Answer 20

Dermatomyositis

Question 21

What can the different subtypes of dermatomyositis be predicted by?

Answer 21

Subtypes with clinical features can be predicted by autoantibody profile
E.g. Anti-MDA5 - interstitial lung disease, digital ulcers/ischaemia

Question 22

What other tests can be used to support a diagnosis of dermatomyositis?

Answer 22

ANA
CK
Skin biopsy
LFT (ALT often increased)
Electromyography (EMG)
Screening for malignancy - important for management of condition

Question 23

What symptoms does IgA vasculitis typically present with?

Answer 23

Abdominal pain
Bleeding
Arthralgia (joint pain)
Arthritis
GI-A associated glomerulonephritis (may develop later)

Question 24

What does this image show and what is it a manifestation of?

Answer 24

Purpura (macular/palpable)
Manifestation of small vessel vasculitis

Question 25

Label these images. What condition are they manifestations of?

Answer 25

Medium vessel vasculitis

Question 26

What is sarcoidosis?

Answer 26

Non-infectious systemic inflammatory disorder in which granulomas infiltrate different organ systems

Question 27

What organ is most commonly affected in sarcoidosis?

Answer 27

Lungs

Question 28

What condition is referred to as the ‘great mimicker’ and why?

Answer 28

Sarcoidosis
Highly variable presentation - cutaneous manifestations in 33%

Question 29

What are the different cutaneous manifestations of sarcoidosis?

Answer 29

Red-brown violaceous papules on face , lips, upper back, neck and extremities
Lupus permit - don’t confuse with LE
Ulcerative
Scar sarcoid
Erythema nodosum

Question 30

What do histology results for sarcoidosis typically show?

Answer 30

Non-caseating epithelioid granulomas

Question 31

What does this image show?

Answer 31

Lupus pernio

Question 32

These manifestations are found in which condition?

Answer 32

Sarcoidosis

Question 33

These manifestations are found in which condition?

Answer 33

Sarcoidosis

Question 34

These manifestations are found in which condition?

Answer 34

Sarcoidosis

Question 35

What does DRESS stand for?

Answer 35

Drug Reaction with Eosinophilia and Systemic Symptoms

Question 36

What is DRESS?

Answer 36

Rash and systemic upset incorporating haematological and solid-organ disturbances

Question 37

How is DRESS diagnosed?

Answer 37

Based on a scoring criteria:

Question 38

What organs can be involved in DRESS?

Answer 38

Liver (hepatitis)
Kidneys (interstitial nephritis)
Heart (myocarditis)
Brain
Thyroid (thyroiditis)
Lungs (interstitial pneumonitis)