1b// The skin in systemic disease Flashcards
What do you have to consider for differential diagnoses for derm? (9)
idiopathic
neoplastic
infection
inflammatory
drug-induced
autoimmune (or alloimmune)
traumatic
metabolic
genetic
What are the investigations you do for derm?
investigations are dependent on clinical findings
How may a patient with lupus present?(8)
rash
fatigue
arthritis
fever
pancytopenia
proteinuria
increased ESR/ CRP
dsDNA positive
What is pancytopenia?
involves having low levels of red blood cells, white blood cells and platelets
What is dsDNA?
Anti-double-stranded deoxyribonucleic acid antibodies (dsDNA Abs) are highly specific markers of systemic lupus erythematosus (SLE)
What are the 2 main groups of lupus erythematosus? And what are 3 other ones you need to know? (5)
Systemic Lupus Erythematosus
Cutaneous (Discoid) Lupus Erythematosus
(there can be overlap)
(they are broad groups)
- the overlap is sub acute systemic lupus erythematosus
- nephrogenic lupus (test with urinalysis, proteinuria)
- neonatal lupus (50% risk of heart block)
What are the groups for the diagnostic criteria of systemic lupus erythematosus?
mucocutaneous findings
haematological findings
immunological findings
What are the mucocutaneous findings of systemic lupus erythematosus? (6)
Cutaneous lupus - acute
Cutaneous lupus - chronic
Oral ulcers
Alopecia
Synovitis
Serositis (pleurisy or pericarditis)
What are the haematological findings of systemic lupus erythematosus? (3)
Haemolytic anaemia
Thrombocytopenia
Leukopenia
What are the immunological findings of systemic lupus erythematosus? (6)
● ANA
● Anti-dsDNA
● Anti-Sm
● Antiphospholipid
● Low Complement
● Direct Coomb’s test
What are the disorders associated with systemic lupus erythematosus?
renal disorder
neurological disorder
What are some symptoms of systemic lupus erythematosus?
Photodistributed (erythematosus) rash
Cutaneous vasculitis
Chilblains (aka itchy swellings)
Alopecia
Livedo reticularis
Cutaneous vasculitis
Subacute cutaneous lupus (SCLE)
What are the types of cutaneous lupus erythematosus?
discoid lupus erythematosus
subacute cutaneous lupus erythematosus (SCLE)
What is the overlap between systemic and cutaneous lupus erythematosus?
SCLE (subacute cutaneous lupus)
What is a feature of discoid lupus?
scarring
Which lupus does not have scarring?
SCLE (subacute lupus erythematosus)
What is an autoimmune connective tissue disease?
dermatomyositis
What is dermatomyositis?
● Autoimmune connective tissue disease
What are the symptoms/ signs of dermatomyositis? (7)
Proximal extensor inflammatory myopathy
Photo-distributed pink-violet rash favouring scalp, periocular regional and extensor surfaces
gottron’s papules
ragged cuticles
shawl sign
heliotrope rash
photosensitive erythema
How is dermatomyositis categorised?
in diff subtypes by antibodies
Subtypes with clinical features that can be predicted by autoantibody profile
Anti Jo-1 – fever, myositis, gottron’s papules
Anti SRP – necrotising myopathy
Anti-p155 – associated with malignancy (in adults)
Anti- MDA5 – interstitial lung disease, digital ulcers / ischaemia
What are the tests for dermatomyositis? (6)
● ANA
● CK
● Skin biopsy
● LFT (Alaninetransaminase often increased)
● EMG
● Screening for internal malignancy
How may a patient with vasculitis present?
What are the vessels that vasculitis can affect?
small
small and medium
medium
large
What is the classification of vasculitis?
What is the subclassifications on vasculitis?
What does it mean if IgA is involved in vasculitis?
organ involved
What are the medium vessel manifestations of vasculitis?
digital necrosis
retiform purpura ulcers
subcutaneous nodules along blood vessels
What are the small vessel manifestations of vasculitis?
What is sarcoidosis?
Systemic granulomatous disorder of unknown origin
non-infectious systemic inflammatory disorder
What does sarcoidosis affect?
Can affect multiple organs: most commonly lungs
Is there always a cutaneous manifestation of sarcoidosis?
Cutaneous manifestations in ~33%
What are the symptoms/ presentation of sarcoidosis?
*Highly variable – ‘the great mimicker’- have to exclude everything else first
- Red-brown to violaceous papules and face, lips, upper back, neck, and extremities
Lupus pernio – NB
Ulcerative
Scar sarcoid Erythema nodosum
What is the histology of sarcoidosis?
- Histology–non-caseating epithelioid granulomas
How do you diagnose sarcoidosis?
Diagnosis of exclusion
Requires evaluation for internal organ involvement
What is DRESS?
Drug Reaction with Eosinophilia and Systemic Symptoms
Rash and systemic upset incorporating haematological and solid-organ disturbances
How may someone with dress present?
What is diagnosis of dress based on?
scoring criteria
What is the criteria for diagnosis of dress?
- Fever ≥ 38.5°C
- L ymphadenopathy ⩾ 2 sites, > 1cm
- Circulating atypical lymphocytes
- Peripheral hypereosinophilia >0.7 × 109
- Internal organs involved - (liver, kidneys, cardiac)
- Negative ANA, Hepatitis / mycoplasma, chlamydia
- Skin involvement
- > 50% Body SA
- Cutaneous eruption suggestive of DRESS
- Biopsy suggestive of DRESS
What are the internal organs involved with drug reaction with eosinophilia and systemic symptoms (dress)? (6)
- Liver (hepatitis)
- Kidneys (interstitial nephritis)
- Heart (myocarditis)
- Brain
- Thyroid (thyroiditis)
- Lungs (interstitial pneumonitis)
What is the underlying mechanism of Drug Reaction with Eosinophilia and Systemic (dress)?
unk
When does Drug Reaction with Eosinophilia and Systemic start?
2-6 weeks after drug exposure
What is the most common internal organ involved with Drug Reaction with Eosinophilia and Systemic (dress)?
liver (most of deaths associated with this)
What are common triggers for Drug Reaction with Eosinophilia and Systemic (dress)?
Sulfonamides,
anti-epileptics (carbamazepine, phenytoin,
lamotrigine),
allopurinol,
Antibiotics (vancomycin, amoxicillin, minocycline, piperacillin-tazobactam),
ibuprofen are common triggers
What are the rash morphologies of Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS)?
- Urticated papular exanthem
- widespread papules
- Maculopapular (morbilliform) eruption
- Widespread erythema (Erythroderma)
- Head / neck oedema
- Erythema multiforme-like
What is the treatment for dress?
Withdrawal of culprit
Corticosteroids are first line treatment - may require months of treatment
Mortality 5-10%
What does alloimmune mean?
Alloimmunity is an immune response to nonself antigens from members of the same species
aka foreign immunity
How can you know if a rash is caused by a drug or GvsHD?
face involvement
acral involvement
diarrhoea
(all indicate GvsHD)
What does GvsHD stand for? And how would a patient present with GvsHD?
graft vs host disease
What is GvsHD and who does it affect?
Multiple-organ disease
Affects ~10-80% of allogenic haematopoetic stem
cell transplants (HSCT)
What is the pathogenesis of graft vs host disease?
donor-derived T-lymphocyte activity against antigens in an immunocompromised recipient
What does graft vs host disease affect?
skin
liver
GI tract
What is the word for sensation of itchiness?
pruritus
What does it suggest if there is pruritus and no rash?
internal cause
What are haematological causes of pruritus without rash?
lymphoma
polycythemia
What are other causes of pruritus without rash? (8)
- Uraemia
- Cholestasis
- Iron deficiency or iron overload
- HIV/HepatitisA/B/C
- Cancer
- Drugs (NB opiates / opioids)
- Psychogenic
- Pruritus of old age
What are investigations you do for pruritus?
- FBC, LDH
- Renal profile
- Liver function tests
- Ferritin
- XR Chest
- HIV/ Hepatitis A/ B/ C
What happens from chronic scratching?
nodular prurigo
What signifies metastases of a malignant carcinoid tumour?
Carcinoid syndrome
What are symptoms of carcinoid syndrome?
diarrhoea
bronchospasm
hypotension
flushing (25% of cases)
wheezing
dizziness
What is carcinoid syndrome?
Signifies metastases of a malignant carcinoid tumour
- 5-HT secretion
google=> Carcinoid syndrome occurs when a rare cancerous tumor called a carcinoid tumor secretes certain chemicals into your bloodstream
How may someone with Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis present?
Is Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis an emergency?
yes it is a derm emergency
What is the prodromal of Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis?
flu like symptoms
What are the symptoms Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis?
Abrupt onset of lesions on trunk > face/ limbs
Macules, blisters, erythema–atypical targetoid
Blisters merge–sheets of skin detachment ‘like wet
wallpaper’
What is a macule?
A macule is a flat, distinct, discolored area of skin
What are erythema–atypical targetoid?
Atypical target lesions show just two zones and/or an indistinct border. In erythema multiforme, these lesions are raised (papular)
What happens in less than 2-3 days in Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis?
Extensive full thickness mucocutaneous (epidermal) necrosis <2-3 days
How is Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis categorised?
What are the differential diagnoses with Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis?
Staphylococcal scalded skin syndrome (SSSS)
Thermal burns
Cutaneous graft versus host disease
What causes more than 80% of Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis cases?
drugs cause >80% of cases
How long before the onset of the rash of Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis did taking the medications start?
up to 3 weeks prior to onset of rash
What is the science behind Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis?
cell mediated cytotoxic reaction against epidermal cells
What is the treatment for Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis?
antibiotics and NSAIDs
What score is used to help assess severity of Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis and what is its criteria?
SCORTEN – score used to help assess severity
Criteria :age >40, HR, initial % epidermal detachment,
serum urea + glucose + bicarbonate, presence of malignancy
What are the complications of Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis?
Death- Overall mortality 30%
Blindness,
dehydration,
hypothermia/hyperthermia,
renal tubular necrosis,
eroded GI tract,
interstitial pneumonitis, neutropaenia,
liver and heart failure
What is erythroderma?
Generalized erythema affecting >90% BSA
How may someone with erythroderma present?
What do the systemic manifestations of erythroderma reflect?
impairment in skin function
What are the systemic manifestations of erythroderma?
- Peripheral edema
- Tachycardia
- Loss of fluid and proteins
- Disturbances in thermoregulation
- Risk of sepsis (loss of barrier)
What are the etiologies of erythroderma?
- Drug reactions
- Cutaneous T-cell lymphoma – Sézary syndrome
- Psoriasis
- Atopic eczema
- Idiopathic (25-30%)
What is the management of erythroderma?
Underlying cause (e.g. treat psoriasis, withdraw drug if drug cause, etc)
Hospitalisation if systemically unwell
Restore fluid and electrolyte balance, circulatory status and manage body temperature.
Emollients to support skin barrier
+/- Topical steroids
+/- Antibiotics
