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)
What is the presentation?
- Periorbital purpura (raccoon’s eyes)
A patient presents with these symptoms:
* Weight loss
* Fatigue
* Paraesthesias
* Dyspnoea
* Syncopal attacks
What is the most likely diagnosis?
- Systemic amyloidosis
What is graft versus host disease (GvHD)?
-
Multiple-organ disease
- Affects ~10-80% of allogenic haematopoetic stem cell transplants (HSCT)
- Pathogenesis: the donor T cells attack antigens in recipient (who is immunocompromised)
A patient presents with these symptoms:
What is the most likely diagnosis?
- Graft versus host disease (GvHD)
How can you if a rash is caused by a drug or GvHD?
* Face or acral involvement
* Diarrhoea
* All indicate GHD more likely
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%
- Sulfonamides, anti-epileptics (carbamazepine, phenytoin, lamotrigine), allopurinol, Antibiotics (vancomycin, amoxicillin, minocycline, piperacillin-tazobactam), ibuprofen are common triggers
- Starts 2-6 weeks after drug exposure
What cutaneous manifestations would one expect in a drug reaction with eosinophilia and systemic symptoms (DRESS) patient (1)?
- Face, upper trunk and extremities are initial sites of involvement
-
Characteristic rash e.g. facial oedema
- Widespread rash > 50% BSA
What non-cutaneous manifestations would one expect in a drug reaction with eosinophilia and systemic symptoms (DRESS) patient (4)?
- Fever ≥ 38.5oC
- Rash
- Lymphadenopathy
- Peripheral eosinophilia > 0.7 × 109
Internal organ involvement:
* Liver (hepatitis, most frequent cause of death)
* Kidneys (interstitial nephritis)
* Heart (myocarditis)
* Brain
* Thyroid (thyroiditis)
* Lungs (interstitial pneumonitis)
A patient presents with these symptoms:
* Fever ≥ 38.5oC
* Rash
* Lymphadenopathy
* Peripheral eosinophilia > 0.7 × 109
What is the most likely diagnosis?
- Drug reaction with eosinophilia and systemic symptoms (DRESS)
What is the management of drug reaction with eosinophilia and systemic symptoms (DRESS) (2)?
- Withdrawal of culprit medication
- Corticosteroids are first line - may require months of treatment
What is stevens-johnson syndrome / toxic epidermal necrolysis?
-
Derm emergency! (Rare)
- Overall mortality 30%
- Prodromal: flu-like sx
-
Abrupt onset of lesions on trunk > face/limbs
- Macules, blisters, erythema - atypical targetoid
- Blisters merge - sheets of skin detachment
What cutaneous manifestations would one expect in a stevens-johnson syndrome / toxic epidermal necrolysis patient (1)?
- Extensive full thickness mucocutaneous necrosis < 2-3 days
What non-cutaneous complications would one expect in a stevens-johnson syndrome / toxic epidermal necrolysis patient (8)?
- Blindness
- Dehydration
- Hypothermia / hyperthermia
- Renal tubular necrosis
- Eroded GI tract
- Interstitial pneumonitis
- Neutropenia
- Liver and heart failure
A patient presents with these symptoms:
What is the most likely diagnosis?
- Stevens-johnson syndrome / toxic epidermal necrolysis
What is erythroderma?
- Generalized erythema affecting >90% BSA
- Multiple etiologies:
- Drug reactions
- Cutaneous T-cell lymphoma - Sézary syndrome
- Psoriasis
- Atopic eczema
- Idiopathic (25-30%)
What cutaneous manifestations would one expect in an erythoderma patient (1)?
- Generalized erythema affecting > 90% BSA
What non-cutaneous manifestations would one expect in an erythoderma patient (4)?
- Tachycardia
- Loss of fluid and proteins
- Disturbances in thermoregulation
- Risk of sepsis
A patient presents with these symptoms:
What is the most likely diagnosis?
- Erythoderma
What is the management of erythoderma (4)?
- Treat 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
What is the presentation?
- Pyoderma gangrenosoum
What is the presentation?
- Erythema nodosum (Panniculitis)
What are the cutaneous diseases associated with inflammatory bowel disease (IBD) (2)?
- Pyoderma gangrenosoum
- Erythema nodosum (Panniculitis)
A patient presents with these symptoms:
What is the most likely diagnosis?
- Inflammatory bowel disease (IBD)
What is the presentation?
- Dermatitis herpetiformis
What is the cutaneous manifestation of celiac disease?
- Dermatitis herpetiformis
A patient presents with these symptoms:
What is the most likely diagnosis?
- Celiac disease
What is the presentation?
- Petechiae, ecchymoses, corkscrew hairs with perifollicular haemorrhage & follicular hyperkeratosis
What is the cutaneous manifestation of Vitamin C deficiency (scurvy) (5)?
- Bleeding, erosion of gums
- Petechiae
- Ecchymoses
- Corkscrew hairs with perifollicular haemorrhage
- Follicular hyperkeratosis
A patient presents with these symptoms:
What is the most likely diagnosis?
- Vitamin C deficiency (scurvy)
What is the presentation?
- Dermatitis
What is the cutaneous and non-cutaneous manifestation of zinc deficiency (3)?
- Dermatitis
- Diarrhoea
- Depression
A patient presents with these symptoms:
* Diarrhoea
* Depression
What is the most likely diagnosis?
- Zinc deficiency
What is the presentation?
- Dermatitis
What is the cutaneous and non-cutaneous manifestation of Vitamin B3 deficiency (niacin) (4)?
- Dermatitis
- Diarrhoea
- Dementia
- Death
A patient presents with these symptoms:
* Diarrhoea
* Dementia
What is the most likely diagnosis?
- Vitamin B3 deficiency (niacin)
What is the presentation?
- Excoriations / prurigo
What is the presentation?
- Xerosis
What is the presentation?
- Calciphylaxis
- 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
What is the presentation?
- Half and half nails
What cutaneous manifestations would one expect in a chronic kidney disease (CKD) patient (4)?
- Excoriations, prurigo
- Xerosis
- Calciphylaxis
- Half and half nails
A patient presents with these symptoms:
* Anaemia
What is the most likely diagnosis?
- Chronic Kidney Disease (CKD)
What is the presentation?
- Muehrcke’s lines
What is the presentation?
- Jaundice
What is the presentation?
- Porphyria cutaneous tarda
What is the presentation?
- Clubbing
What is the presentation?
- Terry’s nails
What is the presentation?
- Spider telangiectasia
What is the presentation?
- Palmar erythema
What cutaneous manifestations would one expect in a chronic liver disease patient (7)?
- Excoriations, prurigo
- Jaundice
- Muehrcke’s lines of nails
- Terry’s nails
- Palmar ervthema
- Spider telangiectasia
- Clubbing
A patient presents with these symptoms:
What is the most likely diagnosis?
- Chronic liver disease
What is the presentation?
- Kaposis’ Sarcoma
What is the presentation?
- Terry’s nails
What is the presentation?
- Granuloma annulare
What is the presentation?
- Neuropathic ulcers
What is the presentation?
- Skin infections
What is the presentation?
- Xerosis
What is the presentation?
- Xanthelesma & Xanthomata
What does the presence of xanthelesma & xanthomata suggest?
- Hyperlipidemia
What is the presentation?
- Acanthosis nigricans
What is the presentation?
- Necrobiosis lipoidica
What cutaneous manifestations would one expect in a diabetes mellitus patient (8)?
- Terry’s nails
- Granuloma annulare
- Neuropathic ulcers
- Skin infections
- Xerosis
- Xanthelesma & xanthomata
- Acanthosis nigricans
- Necrobiosis lipoidica
A patient presents with these symptoms:
What is the most likely diagnosis?
- Diabetes Mellitus
What is the presentation?
What diagnosis does it suggest?
- Pre-tibial myxoedema
- Grave’s disease
What is the presentation?
What diagnosis does it suggest?
- Hyperpigmentation
- Addison’s disease
What is the presentation?
What diagnosis does it suggest (3)?
- Acne
- Acromegaly / Cushing’s syndrome / Polycystic ovarian syndrome (PCOS)
What is the presentation?
What diagnosis does it suggest?
- Cutis gyrata verticis
- Acromegaly
