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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
Q

What are some common triggers of DRESS?

A

Sulfonamides, anti-epileptics (carbamazepine, phenytoin, lamotrigine), allopurinol, Antibiotics (vancomycin, amoxicillin, minocycline, piperacillin-tazobactam), ibuprofen are common triggers

26
Q

How does the Rash appear in DRESS?

A

Rash morphologies:

 - Urticated papular exanthem - widespread papules
  - Maculopapular (morbilliform) eruption 
  - Widespread erythema (Erythroderma)
  - Head / neck oedema
  - Erythema multiforme-like
27
Q

What is the treatment for DRESS?

A

Withdrawal of culprit
Corticosteroids are first line treatment - may require months of treatment
Mortality 5-10%

28
Q

How can you tell if a rash is caused by a drug or Graft versus Host Disease?

A

Face involvement
Acral involvement
Diarrhoea
all indicate that GvHD more likely

29
Q

What is Graft versus Host disease?

A

Multiple-organ disease

Affects ~10-80% of allogenic haematopoetic stem cell transplants (HSCT

30
Q

What is the Pathogenesis of Graft versus Host Disease?

A

Pathogenesis: donor-derived T-lymphocyte activity against antigens in an immunocompromised recipient

31
Q

What does Graft versus Host Disease mostly affect?

A
  • Skin
    • Liver
    • GI tract
32
Q

What is Pruritis suggestive of?

A 
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
Q

What investigations should be done for Pruritis?

A 
FBC, LDH
Renal profile
Liver function tests
Ferritin
XR Chest
HIV / Hepatitis A / B /C
34
Q

What does the patient develop if they are left scratching?

A

Nodular prurigo

35
Q

What happens in Scurvy?

A

Vitamin C (ascorbic acid) deficiency
Spongy gingivae with bleeding and erosion
Petechiae, ecchymoses, follicular hyperkeratosis
Corkscrew hairs with perifollicular haemorrhage

36
Q

What happens in Kwashiorkor?

A 
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
Q

What happens in Zinc deficiency?

A

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
Q

What happens in vitamin B3 deficiency?

A

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
Q

What happens in Carcinoid syndrome?

A 
Signifies metastases of a malignant carcinoid tumour
5-HT secretion
Flushing in 25% of cases
Other symptoms: 
	- Diarrhoea 
	- Bronchospasm
	- Hypotension
40
Q

What is Stevens-Johnson syndrome/Toxic Epidermal Necrolysis(SGS/TEN)?

A

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
Q

What classifies SGS/TEN?

A

10-30% BSA Detatchment

42
Q

What happens in SGS/TEN

A

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
Q

What can be a cause of SGS/TEN?

A

Antibiotics
Anti-Epileptics
NSAIDs

44
Q

How do you determine severity in SGS/TEN?

A

SCORTEN – score used to help assess severity

Criteria: age >40, HR, initial % epidermal detachment, serum urea + glucose + bicarbonate, presence of malignancy

45
Q

What are the complications in SGS/TEN?

A

Death - Overall mortality 30%
Blindness, dehydration, hypothermia/hyperthermia, renal tubular necrosis, eroded GI tract, interstitial pneumonitis, neutropaenia, liver and heart failure

46
Q

What is Erythroderma?

A 
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
Q

What is the management for Erythroderma?

A

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
Q

What are the signs of Chronic Kidney disease?

A

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
Q

What are the signs of Chronic Liver Disease?

A 
Excoriations, prurigo
Jaundice
Muehrcke’s lines of nails
Terry’s nails
Palmar erythema
Spider telangiectasia
Clubbing
50
Q

What is Necrobiosis Lipoidica?

A

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
Q

What are some manifestations of Diabetes?

A 
Terry's nails
Granuloma annulare
Neuropathic ulcers
Acanthosis nigricans
Xerosis
Xanthelesma & Xanthomata
Skin infections
52
Q

What can be manifestations of HIV?

A 
Severe seborrhoea dermatitis
Extensive viral warts
Norwegian scabies
CMV Ulceration
Kaposi sarcoma
Eosinophilic folliculitis
Bacillary angiomatosis
53
Q

What are some other manifestations of HIV?

A

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
Q

What are dermal presentations of GI Disorders?

A

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
Q

What happens in Hidradentitis suppuritiva?

A

Inflamed nodes, sterile abscess, sinus tracts, fistulae and hypertrophic scars
Favours intertriginous zones: especially axillary, anogenital and inframammary area

56
Q

What happens in Pyoderma Gangronesum?

A

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
Q

What are Cutaneous signs of internal malignancy?

A 
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
Q

What is a sign of metastatic pancreatic carcinoma?

A

Haemorrhagic nodules

BRS Phase 1b (80 decks)

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