Derm the skin in systemic disease Flashcards
What can effective dermatological diagnosis help with?
- preventing/reducing internal organ damage via early diagnosis
- detection of internal malignancy
example of disease where skin is just one of many targetted organs
sarcoidosis
example of disease where skin is a sign of internal disorder
flushing in carcinoid syndrome
example of disease where skin condition is telltale sign of underlying condition
pyoderma gangrenosum in IBD
example of disease where skin leads to 2ndary organ involvement
high output cardiac failure in erythroderma
things for dermatomyositis investigation
- anti-nuclear antibody (positive)
- creatine kinase (look at muscle activity)
- Skin biopsy
- LFT
- electromyography
- Screening for internal malignancy
What is the criteria for drug reaction with eosinophilia and systemic symptoms (DRESS)?
Fever ≥ 38.5°C
Lymphadenopathy ⩾ 2 sites, > 1cm
Circulating atypical lymphocytes
Peripheral hypereosinophilia >0.7 × 10^9
Internal organs involved - (liver, kidneys, cardiac)
Negative ANA, Hepatitis / mycoplasma, chlamydia
Skin involvement
- >50% BSA
- Cutaneous eruption suggestive of DRESS e.g. facial oedema
- Biopsy suggestive of DRESS
what organs are commonly affected in DRESS?
- Liver (hepatitis)- most frequent cause of death
- Kidneys (interstitial nephritis)
- Heart (myocarditis)
- Brain
- Thyroid (thyroiditis)
- Lungs (interstitial pneumonitis)
how to treat DRESS
- WITHDRAWAL OF CAUSATIVE AGENT
- corticosteroids are 1st line treatment: may require for months
- mortality is 5-10%
What is graft versus host disease?
Multiple-organ disease
Affects ~10-80% of allogenic haematopoetic stem cell transplants (HSCT)
Pathogenesis: donor-derived T-lymphocyte activity against antigens in an immunocompromised recipient
how is sarcoidosis diagnosed?
highly variable presentation = diagnosis of exclusion. Investigations required for diagnosis and to investigate extracutaneous involvement
What is scurvy and what are key symptoms?
Vitamin C (ascorbic acid) deficiency
- Spongy gingivae (gums) with bleeding and erosion
- Petechiae, ecchymoses, follicular hyperkeratosis
- Corkscrew hairs with perifollicular haemorrhage
What do we call protein deficiency and what are key features?
kwashiorkor Systemic features: - Hepatomegaly - Bacterial / fungal infections - Diarrhoea - Loss of muscle mass - Oedema - Failure to thrive Skin signs: - Superficial desquamation (large areas of erosion) - Sparse, dry hair - Soft, thin nails - Cheilitis (lip inflammation)
What are symptoms of vitamin B3 deficiency (niacin)?
Deficiency:
- Dermatitis | Diarrhoea | Dementia | Death
Cutaneous manifestations:
- Photodistributed erythema
- ‘Casal’s necklace’
- Painful fissures of the palms and soles
- Peri-anal, genital and perioral inflammation and erosions
What is carcinoid syndrome and what are symptoms?
Signifies metastases of a malignant carcinoid tumour Due to 5-HT secretion Flushing in 25% of cases Other symptoms: - Diarrhoea - Bronchospasm - Hypotension
What is Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis and what are symptoms?
Derm emergency! (Rare)
Prodromal: flu-like symptoms
Abrupt onset of lesions on trunk > face/limbs
Macules, blisters, erythema – atypical targetoid
Blisters merge – sheets of skin detachment ‘like wet wallpaper’
Extensive full thickness mucocutaneous (epidermal) necrosis <2-3 days
Where does the name Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis come from?
There is a spectrum:
SJS IF:
BSA detachment <10%
Mortality = 10%
SJS/TEN
BSA detachment 10-30%
TEN
BSA detachment >30%
Mortality >30%
What causes Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis?
Cell-mediated cytotoxic reaction against epidermal cells
Drugs cause >80% of cases
May be started up to 3 weeks prior to onset of rash
What drugs can cause Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis? (9)
Antibiotics Beta-lactams Sulphonamides Allopurinol Anti-Epileptic Drugs Phenytoin Carbamazepine Lamotrigine NSAIDs
How can we assess the severity of Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis?
SCORTEN Criteria: age >40 HR >120 initial % epidermal detachment serum urea + glucose + bicarbonate presence of malignancy
What are 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 zinc important for?
Important role in 200 enzymes – regulation of lipid, protein, nucleic acid synthesis
Roles in wound healing, antioxidant
What are causes and symptoms of zinc deficiency?
Deficiency: genetic (SLC39A4) or acquired
- Triad of Dermatitis | Diarrhoea | Depression
Perioral, acral and perineal skin in particular is affected with scaly erosive erythema
cutaneous manifestation of niacin (vit B3) deficiency
- photodistributed erythema
- casal necklace
- panful fissures of the palms and soles
- chellitis and glossitis
- vaginitis with erosions
New onset of itching without a rash may be a manifestation of an underlying cancer - true/false
true
What illness presents as Flu-like illness, sore eyes and oral ulceration.
- Followed by extensive painful rash
What disease is this?
Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis
Treatment for Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis
1st line: withdrawal of causative agent and urgent evaluation (SCORTEN)
Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis complications
Death - Overall mortality 30%
Blindness, dehydration, hypothermia/hyperthermia, renal tubular necrosis, eroded GI tract, interstitial pneumonitis, neutropaenia, liver and heart failure
criteria for Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis
Criteria: age >40, HR, initial % epidermal detachment, serum urea + glucose + bicarbonate, presence of malignancy
management of erythroderma
Treat 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
Chronic liver disease presentation?
Excoriations, prurigo Jaundice Muehrcke’s lines of nails Terry’s nails (white nails with red band at tip) Palmar erythema Spider telangiectasia Clubbing
What is a dermal manifestation of diabetes?
How does it present?
How to treat?
Necrobiosis Lipoidica
20-65% happen in diabetes
Plaques with red-brown raised edge with yellow-brown atrophic centre
-> treat with topical/intralesional steroids
Hidradenitis suppuritiva features
Inflamed nodes, sterile abscess, sinus tracts, fistulae and hypertrophic scars
Favours intertriginous zones: especially axillary, anogenital and inframammary area
Pyoderma gangrenosum features
Pustule on an erythematous base –ulcerates and extends with necrotic undermined border
Painful
Associated with inflammatory bowel disease, leukemia, seronegative arthritis in 50-70% of cases
Cutaneous diseases associated with inflammatory bowel disease:
Pyoderma gangrenosum Orofacial granulomatosis Panniculitis (erythema nodosum) Aphthous ulceration Association with psoriasis, pemphigoid
Cutaneous manifestation of celiac disease
- Dermatitis herpetiformis
Peau d’orange is a sign of what?
breast carcinoma
what might you see on the inner lips in Peutz-Jegher syndrome
mucosal melanosis
What are differentials for a skin condition? (9)
Idiopathic Neoplastic Infection Inflammatory Drug-induced Autoimmune Traumatic Metabolic Genetic
How is a skin biopsy taken?
Punch biopsy: taken under local anesthetic
Taken for histology to:
- examine cells
- see inflammatory patterns
- see cellular abnormalities
- check for cancer
Histology can also be taken for immunofluorescence to detect antibodies
What are the 2 types of Lupus Erythematosus?
Systemic Lupus Erythematosus
Cutaneous (Discoid) Lupus Erythematosus
What is the diagnostic criteria for systemic lupus erythematosus?
Mucocutaneous: Cutaneous lupus - acute Cutaneous lupus - chronic Oral ulcers Alopecia
Other: Synovitis Serositis (pleurisy or pericarditis) Renal disorder Neurological disorder
Haematological:
Haemolytic anaemia
Thrombocytopenia
Leukopenia
Immunological: ANA Anti-dsDNA Anti-Sm Antiphospholipid Low Complement Direct Coomb’s test
What are symptoms of SLE?
Photodistributed rash Cutaneous vasculitis Chilblains Alopecia Livedo reticularis Subacute cutaneous lupus (SCLE)
What are symptoms of cutaneous (discoid) lupus erythematosus?
Discoid lupus erythematosus
Subacute cutaneous lupus (SCLE)- overlap with SLE
You get distinct scaring
What makes cutaneous lupus erythematosus unique?
Has distinct scarring
What is neonatal lupus and what must you do with a baby with NL?
Lupus Erythematosus in newborns
Autoimmune
Theyre Ro positive
MUST DO AN ECG- risk of heart block is 50%
How would a sun exposed/ photo rash present?
Rash on face, chest, upper back and dorsal hands ( body in contact with sun)
What is dermatomyositis and what are some key symptoms?
Autoimmune connective tissue disease
Proximal extensor inflammatory myopathy
Photo-distributed pink-violet rash favouring scalp, periocular regional and extensor surfaces
What are some signs of dermatomyositis?
Gottron's papules on metacarpal and interphalangeal joints Ragged cuticles Shawl sign (red upper trunk) Heliotrope rash (red around eyes) Photosensitive erythema
What are the different autoantibodies and associated symptoms which can cause dermatomyositis?
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
What is IgA vasculitis?
Vasculitis which affects small vessels (AKA Henoch-Schonlein purpura)
What are symptoms of IgA vasculitis?
Purpura (macula/ palpable) Abdominal pain Bleeding Arthralgia (joint stiffness) Arthritis IgA- associated glomerulonephritis
How does vasculitis vary for different sized vessels (Classifications and subclassifications)?
Small
Cutaneous small vessel (leukocytoclastic) vasculitis - Idiopathic - Infectious - Medication exposure - Inflammatory (connective tissue disease Small vessel vasculitis - special types - IgA Vasculitis (Henoch-Scholein) - Urticarial vasculitis - Acute haemorrhagic oedema of infacncy - Erythema elevatum diutinum
Small and medium
Cryoglobulinemia - Type II & III ANCA-associated -EGPA (Churg-Strauss) -Microscopic Polyangiitis, - GPA (Wegener)
Medium
Polyarteritis nodosa (PAN)
- Benign cutaneous form
- Systemic form
Large
Temporal arteritis
Tayakasu
What is sarcoidosis and what are symptoms?
Systemic granulomatous disorder of unknown origin
Can affect multiple organs: most commonly lungs
Cutaneous manifestations in ~33%
- Highly variable – ‘the great mimicker’
- Red-brown to violaceous papules and face, lips, upper back, neck, and extremities
Lupus pernio – involvement of face (not related to SLE)
Ulcerative
Scar sarcoid
Erythema nodosum
Histology–non-caseating epithelioid granulomas
What drugs can cause DRESS?
Sulfonamides, anti-epileptics (carbamazepine, phenytoin, lamotrigine), allopurinol, Antibiotics (vancomycin, amoxicillin, minocycline, piperacillin-tazobactam), ibuprofen are common triggers
How do rashes present in DRESS?
Urticated papular exanthem - raised papule rash Maculopapular (morbilliform) eruption Widespread erythema (Erythroderma) Head / neck oedema Erythema multiforme-like
How can you differentiate a rash caused by drugs or a rash caused by graft vs host disease?
Face involvement
Acral involvement
Diarrhoea
ALL INDICATE GvHS
What does GvHD mainly affect?
Skin
Liver
GI tract
What are causes of pruritus?
Haematological causes: lymphoma, polycythemia Uraemia Cholestasis Iron deficiency or iron overload HIV / Hepatitis A / B / C Cancer Drugs (NB opiates / opioids) Psychogenic Pruritus of old age
What investigations are carried out for pruritus?
FBC, LDH Renal profile Liver function tests Ferritin XR Chest HIV / Hepatitis A / B /C
What happens if you continuously itch?
You develop nodular prurigo
The skin thickens as a defensive mechanism but this causes more itchiness
What is erythroderma and what can it lead to?
Generalized erythema affecting >90% BSA Systemic manifestations reflect impairment in skin function: - Peripheral edema - Tachycardia - Loss of fluid and proteins - Disturbances in thermoregulation - Risk of sepsis
What are causes of erythroderma?
Drug reactions Cutaneous T-cell lymphoma – Sézary syndrome Psoriasis Atopic eczema Idiopathic (25-30%)
What is pruritis?
Itching without rash suggestive of internal cause
What is a cutaneous manifestation of celiac disease?
Dermatitis herpetiformis
What are manifestations of HIV?
Non-specific: Morbilliform rash Urticaria Erythema multiforme Oral / genital ulceration NB Low threshold for testing
Itch
Opportunistic infection
Severe manifestations of common dermatosis e.g. psoriasis, seborrheic dermatitis)
Suggestive dermatoses e.g. eosinophilic folliculitis
How may chronic kidney disease present and what are key signs related to primary disease and immunosuppression?
Anaemia – mucosal pallor, hair thinning
Excoriations, prurigo
Calciphylaxis
Half and half nails
Signs related to primary disease
- ANCA-associated vasculitis - Systemic Lupus Erythematosus
Signs related to immunosuppression
- Viral warts - Skin cancer
What are cutaneous signs of internal malignancy?
Cutaneous metastases
Malignancy reflecting internal malignancy
- Extramammary Paget’s disease
Genetic condition predisposing to internal cancer and skin lesions
- Hereditary leiomyomatosis and renal cell cancer - Peutz–Jeghers syndrome
Skin disease associated with malignancy
- Dermatomyositis - Erythema gyratum repens (2o to bronchial carcinoma) - Pyoderma gangrenosum - Paraneoplastic pemphigus
Non-specific skin disease
- Pruritus - Vasculitis - Urticaria
