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BRS Phase 1b > Skin in Systemic Disease > Flashcards

Skin in Systemic Disease Flashcards

1
Q

Why is the skin important in systemic disease?

A

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 underlying condition, e.g. Pyoderma gangrenosum in inflammatory bowel disease.

Secondary systemic involvement - Systemic disease secondary to skin disorder, e.g. high output cardiac failure in erythroderma.

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2
Q

What is an example of a test used to look at skin cells?

A

Punch biopsy

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3
Q

What are the 2 main groups of Lupus Erythematosus?

A

Systemic Lupus Erythematosus

Cutaneous (Discoid) Lupus Erythematosus

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4
Q

What are the Diagnostic criteria for Systemic Lupus Erythematosus?

A

Mucocutaneous - Cutaneous lupus - acute/chronic, Oral ulcers, Alopecia.
Synovitis, serostisis, renal disorder
Chilblains
Photodistributed erythematous rash
Neurological disorder
Haematological - Haemolytic anaemia, thrombocytopenia, leukopenia.
Immunological - ANA, Anti-dsDNA, Anti-Sm, Antiphospholipid, Low Complement, Direct Coomb’s test.

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5
Q

What are the presentations for Systemic Lupus Erythematosus?

A 
Photodistributed rash 
	Cutaneous vasculitis
	Chilblains 
	Alopecia
	Livedo reticularis
	Cutaneous vasculitis 
	Subacute cutaneous lupus 	(SCLE)
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6
Q

What are the presentations for Cutaneous (Discoid) Lupus Erythematosus?

A

Discoid lupus erythematosus
SCLE
Scarring

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7
Q

What is positive in Neonatal Lupus?

A

Ro positive antibody

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8
Q

What is the test that is essential for neonatal lupus?

A

ECG

50% have heart block

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9
Q

What is Dermatomyositis?

A

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

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10
Q

What are the signs of Dermatomyositis?

A 
Gottron's papules
Ragged cuticles
Shawl's sign
Heliotrope rash
Photosensitive
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11
Q

What are some important antibodies associated with subtypes of Dermatomyositis?

A

Anti-p155 - associated with malignancy

Anti-MDA5 - interstitial lung disease, digital ulcers/ischaemia

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12
Q

What are the tests you should do if you suspect Dermatomyositis?

A 
Anti-Nuclear Antibody - positive
Creatine kinase
Skin biopsy
LFT (ALT often increased) 
EMG
Screening for internal malignancy
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13
Q

What are the subclassifications of Cutaneous small vessel vasculitis?

A

Idiopathic
Infectious
Medication exposure
Inflammatory

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14
Q

What are the subclassifications of Small vessel vasculitis?

A

IgA vasculitis - Henoch Scholein
Urticarial vasculitis
Acute haemorrhagic oedema of infancy
Erythema elevatumdiutinum.

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15
Q

What are the subclassifications of small and medium vessel vasculitis?

A 
Type 2 & 3 Cryogolulinemia
ANCA- Associated;
EGPA (Churg Strauss)
Microscopic Polyangiitis
GPA(Wegener)
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16
Q

What are the subclassifications of medium vessel vasculitis?

A

Polyarteritis Nodosa
Benign Cutaneous form
Systemic form

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17
Q

What are the Classifications of Large vessel vasculitis?

A

Temporal arteritis

Tayakasu

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18
Q

What are the manifestations of small vessel vasculitis?

A

Purpura (macular/palpable)

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19
Q

What are the manifestations of medium vessel vasculitis?

A

Digital necrosis
Retiform purpura ulcers
Subcutaneous nodules along blood vessels

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20
Q

What is Sarcoidosis?

A

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 – NB
Ulcerative
Scar sarcoid
Erythema nodosum
Histology–non-caseating epithelioid granulomas
Diagnosis of exclusion
Requires evaluation for internal organ involvement

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21
Q

How does Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) present?

A

Rash and systemic upset incorporating haematological and solid‐organ disturbances

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22
Q

What is the diagnostic criteria for DRESS?

A

Diagnosis is based on scoring criteria including:
Fever ≥ 38.5°C
Lymphadenopathy ⩾ 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% BSA - body surface involvement
Cutaneous eruption suggestive of DRESS e.g. facial oedema
Biopsy suggestive of DRESS

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23
Q

What is the Internal organ involvement in DRESS?

A 
Liver (hepatitis)- most frequent cause of death
Kidneys (interstitial nephritis)
Heart (myocarditis)
Brain
Thyroid (thyroiditis)
Lungs (interstitial pneumonitis)
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24
Q

What is the Underlying mechanism in DRESS?

A

Underlying mechanism not known
Starts 2-6 weeks after drug exposure
Liver most common internal organ involved – majority of deaths associated with this

25
What are some common triggers of DRESS?
Sulfonamides, anti-epileptics (carbamazepine, phenytoin, lamotrigine), allopurinol, Antibiotics (vancomycin, amoxicillin, minocycline, piperacillin-tazobactam), ibuprofen are common triggers
26
How does the Rash appear in DRESS?
Rash morphologies: - Urticated papular exanthem - widespread papules - Maculopapular (morbilliform) eruption - Widespread erythema (Erythroderma) - Head / neck oedema - Erythema multiforme-like
27
What is the treatment for DRESS?
Withdrawal of culprit Corticosteroids are first line treatment - may require months of treatment Mortality 5-10%
28
How can you tell if a rash is caused by a drug or Graft versus Host Disease?
Face involvement Acral involvement Diarrhoea all indicate that GvHD more likely
29
What is Graft versus Host disease?
Multiple-organ disease | Affects ~10-80% of allogenic haematopoetic stem cell transplants (HSCT
30
What is the Pathogenesis of Graft versus Host Disease?
Pathogenesis: donor-derived T-lymphocyte activity against antigens in an immunocompromised recipient
31
What does Graft versus Host Disease mostly affect?
- Skin - Liver - GI tract
32
What is Pruritis suggestive of?
``` Itching without rash suggestive of internal cause: 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 ```
33
What investigations should be done for Pruritis?
``` FBC, LDH Renal profile Liver function tests Ferritin XR Chest HIV / Hepatitis A / B /C ```
34
What does the patient develop if they are left scratching?
Nodular prurigo
35
What happens in Scurvy?
Vitamin C (ascorbic acid) deficiency Spongy gingivae with bleeding and erosion Petechiae, ecchymoses, follicular hyperkeratosis Corkscrew hairs with perifollicular haemorrhage
36
What happens in Kwashiorkor?
``` Protein deficiency Systemic features: - Hepatomegaly - Bacterial / fungal infections - Diarrhoea - Loss of muscle mass - Oedema - Failure to thrive Skin signs: - Superficial dequamation large areas of erosion - Sparse, dry hair - Soft, thin nails - Cheilitis ```
37
What happens in Zinc deficiency?
Important role in 200 enzymes – regulation of lipid, protein, nucleic acid synthesis Roles in wound healing, antioxidant Deficiency: genetic (SLC39A4) or acquired - Triad of Dermatitis | Diarrhoea | Depression Perioral, acral and perineal skin in particular is affected with scaly erosive erythema
38
What happens in vitamin B3 deficiency?
Required for most cellular processes 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
39
What happens in Carcinoid syndrome?
``` Signifies metastases of a malignant carcinoid tumour 5-HT secretion Flushing in 25% of cases Other symptoms: - Diarrhoea - Bronchospasm - Hypotension ```
40
What is Stevens-Johnson syndrome/Toxic Epidermal Necrolysis(SGS/TEN)?
Derm emergency! (Rare) Prodromal: flu-like sx 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
41
What classifies SGS/TEN?
10-30% BSA Detatchment
42
What happens in SGS/TEN
Cell-mediated cytotoxic reaction against epidermal cells Drugs cause >80% of cases May be started up to 3 weeks prior to onset of rash DDx: Staphylococcal scalded skin syndrome (SSSS) Thermal burns Cutaneous graft versus host disease
43
What can be a cause of SGS/TEN?
Antibiotics Anti-Epileptics NSAIDs
44
How do you determine severity in SGS/TEN?
SCORTEN – score used to help assess severity | Criteria: age >40, HR, initial % epidermal detachment, serum urea + glucose + bicarbonate, presence of malignancy
45
What are the complications in SGS/TEN?
Death - Overall mortality 30% Blindness, dehydration, hypothermia/hyperthermia, renal tubular necrosis, eroded GI tract, interstitial pneumonitis, neutropaenia, liver and heart failure
46
What is Erythroderma?
``` 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 Multiple etiologies: - Drug reactions - Cutaneous T-cell lymphoma – Sézary syndrome - Psoriasis - Atopic eczema - Idiopathic (25-30%) ```
47
What is the management for 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
48
What are the signs of Chronic Kidney disease?
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
49
What are the signs of Chronic Liver Disease?
``` Excoriations, prurigo Jaundice Muehrcke’s lines of nails Terry’s nails Palmar erythema Spider telangiectasia Clubbing ```
50
What is Necrobiosis Lipoidica?
20-65% of cases occur in setting of Diabetes Mellitus Plaques with red-brown raised edge with yellow-brown atrophic centre Treatment: topical / intralesional steroids
51
What are some manifestations of Diabetes?
``` Terry's nails Granuloma annulare Neuropathic ulcers Acanthosis nigricans Xerosis Xanthelesma & Xanthomata Skin infections ```
52
What can be manifestations of HIV?
``` Severe seborrhoea dermatitis Extensive viral warts Norwegian scabies CMV Ulceration Kaposi sarcoma Eosinophilic folliculitis Bacillary angiomatosis ```
53
What are some other manifestations of HIV?
Seroconversion – variable nonspecific manifestations: - Morbilliform rash - Urticaria - Erythema multiforme - Oral / genital ulceration - NB Low threshold for testing Persistent or atypical manifestations or common infections Opportunistic infections Severe manifestations of common dermatoses (e.g. psoriasis, seborrheic dermatitis) Itch Suggestive dermatoses e.g. eosinophilic folliculitis
54
What are dermal presentations of GI Disorders?
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
55
What happens in Hidradentitis suppuritiva?
Inflamed nodes, sterile abscess, sinus tracts, fistulae and hypertrophic scars Favours intertriginous zones: especially axillary, anogenital and inframammary area
56
What happens in Pyoderma Gangronesum?
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
57
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 - Pyoderma gangrenosum - Paraneoplastic pemphigus Non-specific skin disease - Pruritus - Vasculitis - Urticaria ```
58
What is a sign of metastatic pancreatic carcinoma?
Haemorrhagic nodules

BRS Phase 1b (80 decks)

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