The Skin in Systemic Disease Flashcards
What is systemic lupus erythematosous (SLE)?
- Systemic lupus erythematosus (SLE) is an autoimmune disease. In this disease, the immune system of the body mistakenly attacks healthy tissue. It can affect the skin, joints, kidneys, brain, and other organs, causing widespread inflammation and tissue damage.
What are the 3 main types of lupus erythematosous?
- Systemic Lupus Erythematosus (SLE)
- Cutaneous (Discoid) Lupus Erythematosus
- Neonatal Lupus
What investigations are suggested in suspected systemic lupus erythematosous (SLE) (6)?
- ANA anti nuclear antibodies
- Anti-dsDNA
- Anti-Sm
- Antiphospholipid
- Complement level (low!)
- Direct Coomb’s test
Name the presentation.
Chilblains
Name the presentation.
- Photodistributed (sun-exposed areas) erythematous rash
Name the presentation.
- Livedo reticularis
Name the presentation.
- Subacute cutaneous lupus
Name the presentation.
- Palpable purpura
What cutaneous manifestations would one expect in a systemic lupus erythematosous (SLE) patient (6)?
- Photodistributed rash
- Cutaneous vasculitis
- Chilblains
- Alopecia
- Livedo reticularis
- Subacute cutaneous lupus (SCLE)
A patient presents with these symptoms:
What is the most likely diagnosis?
- Systemic lupus erythematosous (SLE)
What cutaneous manifestations would one expect in a cutaneous (discoid) lupus erythematosous patient (2)?
- Discoid lupus
- Subacute cutaneous lupus (SCLE)
A patient presents with these symptoms:
What is the most likely diagnosis?
- Cutaneous (discoid) lupus erythematosou
A newborn presents with these symptoms:
What is the most likely diagnosis?
- Neonatal lupus
NB Test ECG – risk of heart block (50% risk)
What is dermatomyositis?
- 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
What investigations are recommended in suspected dermatomyositis (Bloods 3 / Specific tests 2)?
Bloods:
* ANA (anti nuclear antibodies this is the screening test, then do details or autoantibodies)
* Creatine kinase increased
* LFTs (ALT often increased due to muscle breakdown)
Specific tests:
* EMG (Electromyography)
* Diagnostic procedure that evaluates the health condition of muscles and the nerve cells that control them
* Skin biopsy
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
Name the presentation.
- Gottron’s papules
Name the presentation.
- Ragged cuticles
Name the presentation.
- Digital ulcers
Name the presentation.
- Shawl sign
Name the presentation.
- Photosensitive erythema
Name the presentation.
- Heliotrope rash
Name the presentation.
- V-sign
What cutaneous manifestations would one expect in a dermatomyositis patient (7)?
- Gottron’s papules
- Ragged cuticles
- Digital ulcers
- Shawl sign
- Photosensitive erythema
- Heliotrope rash
- V-sign
Photo-distributed
Pink-violet rash
Extensor surfaces + periocular
A patient presents with these symptoms:
What is the most likely diagnosis?
- Dermatomyositis
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
- 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.
Name the presentation.
- Purpura (macular / palpable)
Name the presentation.
- Digital necrosis
Name the presentation.
- Retiform purpura and ulcers
Name the presentation.
- Retiform purpura and ulcers
Name the presentation.
- Subcutaneous nodules along blood vessels
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
A patient presents with these symptoms:
What is the most likely diagnosis?
- Vasculitis
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
What is the presentation?
- Granulomas
What is a granuloma?
- An organised aggregation or activated macrophages that forms in response to chronic inflammation
What is the presentation?
- Scar Scaroid
What is the presentation?
- Lupus Pernio
What cutaneous manifestations would one expect in a sarcoidosis patient (5)?
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
The great mimicker - very variable
A patient presents with these symptoms:
What is the most likely diagnosis?
Sarcoidosis
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
What non-cutaneous manifestations would one expect in a systemic amyloidosis patient (5)?
- Weight loss
- Fatigue
- Paraesthesias
- Dyspnoea
- Syncopal attacks
What cutaneous manifestations would one expect in a systemic amyloidosis patient (1)?
- Periorbital purpura (raccoon’s eyes)