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Year 2 Derm > The skin in systemic disease > Flashcards

The skin in systemic disease Flashcards

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1
Q

Why is the skin important in systemic disease?

A

Rashes may be more than skin deep
Comprehensive assessment coupled with dermatological diagnostic skills can:

  • Prevent or reduce internal organ damage by early diagnosis
  • Allow detection of internal malignancy
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2
Q

How can systemic disease manifest in the skin?

A

Skin targeted

Skin signs

‘Tell-tale’ skin conditions

Secondary systemic involvement

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3
Q

Give an example of skin targeted disease?

A

Multi-organ systemic disease targeting skin e.g. Sarcoidosis

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4
Q

Give an example of skin signs in disease?

A

Sign of internal disorder e.g. flushing in Carcinoid syndrome

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5
Q

Give an example of a ‘tell-tale’ skin condition?

A

Skin conditions suggestive of underlying condition e.g. Pyoderma gangrenosum in inflammatory bowel disease

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6
Q

Give an example of secondary systemic involvement?

A

Systemic disease secondary to skin disorder e.g. high output cardiac failure in erythroderma

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7
Q

What are the different diagnosis categories?

A 
Idiopathic
Neoplastic
Infection
Inflammatory
Drug-induced
Auto immune
Traumatic 
Metabolic 
Genetic
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8
Q

What are the two main types of lupus?

A

Systemic Lupus Erythematosus

Cutaneous (Discoid) Lupus Erythematosus

  • Overlap
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9
Q

What are the mucocutaneous manifestations of systemic lupus?

A 
Cutaneous lupus	- acute
Cutaneous lupus	- chronic		 Mucocutaneous
Oral ulcers
Alopecia
Synovitis
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10
Q

What are the other manifestations of systemic lupus?

A

Synovitis
Serositis (pleurisy or pericarditis)
Renal disorder
Neurological disorde

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11
Q

What are the haematological manifestations of systemic lupus?

A

Haemolytic anaemia
Thrombocytopenia
Leukopenia

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12
Q

What are the immunological criteria for systemic lupus diagnosis?

A 
ANA
Anti-dsDNA
Anti-Sm
Antiphospholipid			
Low Complement
Direct Coomb’s test
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13
Q

What are the cutaneous manifestations of systemic lupus?

A 
Photodistributed rash 
Cutaneous vasculitis
Chilblains 
Alopecia
Livedo reticularis
Cutaneous vasculitis 
Subacute cutaneous lupus 	(SCLE)
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14
Q

What are the cutaneous manifestations of cutaneous lupus?

A

Discoid lupus erythematosus SCLE

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15
Q

What is the test that must be conducted if neonatal lupus is suspected?

A

Test ECG

– risk of heart block (50% risk)

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16
Q

What is dermatomyositis?

A

Autoimmune connective tissue disease

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17
Q

What are the main features of dermatomyositis?

A

Proximal extensor inflammatory myopathy

Photo-distributed pink-violet rash favouring scalp, periocular regional and extensor surfaces

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18
Q

What are the subtypes of dermatomyositis?

A

Subtypes with clinical features that can be predicted by autoantibody profile

  • Malignancy
  • Interstitial lung disease
  • Digital ischaemia
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19
Q

What are the signs seen in dermatomyositis?

A 
Gottron's papules
Ragged cuticles
Shawl sign
Helitrope rash
Photosensitive erythema
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20
Q

What are the autoantibodies that can diagnose subtypes of dermatomyositis?

A

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

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21
Q

How do you diagnose dermatomyositis?

A 
ANA
CK
Skin biopsy
LFT (ALT often increased) 
EMG
Screening for internal malignancy
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22
Q

What are the different types of vasculitis?

A

Small
Small and medium
Medium
Large

Vessel

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23
Q

What is the classification of small vessel vasculitis?

A

Cutaneous small vessel (leukocytoclastic) vasculitis

  • Idiopathic
  • Infectious
  • Medication exposure
  • Inflammatory (connective tissue disease
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24
Q

What is the classification of small vessel vasculitis? (special types)

A
  • IgA Vasculitis (Henoch-Scholein)
  • Urticarial vasculitis
  • Acute haemorrhagic oedema of infacncy
  • Erythema elevatum diutinum
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25
Q

What is the classification of small and medium vessel vasculitis?

A

Cryoglobulinemia
- Type II & III

ANCA-associated

  • EGPA (Churg-Strauss)
  • Microscopic Polyangiitis,
  • GPA (Wegener)
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26
Q

What is the classification of medium vessel vasculitis?

A

Polyarteritis nodosa (PAN)

  • Benign cutaneous form
  • Systemic form
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27
Q

What is the classification of large vessel vasculitis?

A

Temporal arteritis

Tayakasu

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28
Q

What are the manifestation of small vessel vasculitis?

A

Purpura (macular / palpable)

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29
Q

What are the manifestation of medium vessel vasculitis?

A 
Digital necrosis 
Retiform purpura
Ulcers
Retiform purpura
Ulcers
Subcutaneous nodules along blood vessels
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30
Q

What are the main features of IgA Vasculitis?

A

Initial presentation often indistinguishable from other small vessel vasculitis

Abdominal pain, bleeding (65%), arthralgia / arthritis (64%)

IgA-associated glomerulonephritis (40%)

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31
Q

What are the main features of granulomatosis with polyangiitis?

A

Cough, dyspnoea, and chest pain:

Pulmonary infiltrates (70%)

Glomerulonephritis (85%)

Cutaneous findings (50%)

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32
Q

What are the small vessel manifestations of granulmatosis with polyangiitis?

A

Purpura (macular / flat or popular / palpable)

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33
Q

What are the medium vessel manifestations of granulmatosis with polyangiitis?

A
  • Retiform purpura
  • Digital necrosis
  • Livedo reticularis
  • Subcutaneous nodules distributed along blood vessels
  • Ulcers
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34
Q

What are the main features of 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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35
Q

What is DRESS?

A

Drug Reaction with Eosinophilia and Systemic Symptoms

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36
Q

What are the symptoms of DRESS?

A

Rash and systemic upset incorporating haematological and solid‐organ disturbances

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37
Q

What are the manifestations of DRESS?

A
  • Characteristic rash e.g. facial oedema
  • Widespread rash >50% BSA
    Fever ≥ 38.5°C
    Lymphadenopathy
    Peripheral eosinophilia >0.7 × 10^9
    Internal organ involvement (liver, kidneys, cardiac
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38
Q

What are the internal organ involvements 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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39
Q

What are the diagnostic criteria for DRESS?

A 
Fever
Lymphadenopathy  ⩾ 2 sites, > 1cm 
Circulating atypical lymphocytes
Peripheral hypereosinophilia
Internal organs involved
Negative ANA, Hepatitis / mycoplasma, chlamydia
Skin involvement
>50% BSA
Cutaneous eruption suggestive of DRESS e.g. facial oedema
Biopsy suggestive of DRESS
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40
Q

What causes DRESS?

A

Underlying mechanism not known
Starts 2-6 weeks after drug exposure
Fever and rash are most common symptoms
Face, upper trunk and extremities are initial sites of involvement

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41
Q

What drugs have been known to cause DRESS?

A

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

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42
Q

What is the morphology of DRESS rashes?

A

Urticated papular exanthem - widespread papules

  • Morbilliform eruption
  • Erythroderma / widespread exfoliative erythema
  • Head / neck oedema
  • Erythema multiforme-like
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43
Q

What is the treatment for DRESS?

A

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

44
Q

What is schnitzler syndrome?

A

Late‐onset acquired autoinflammatory syndrome
A recurrent urticarial rash is usually the first sign - non‐pruritic urticated macules, papules or plaques, particularly on the trunk, which resolve with brownish hyperpigmentation.

Usually manifests in adulthood

45
Q

What are the symptoms of schnitzler syndrome?

A

Raised monoclonal IgM
Recurrent fever above 40°C
Bone or joint pain (especially over the ilium or tibia)
Lymphadenopathy, hepatomegaly or splenomegaly,
Neutrophilia
Elevated acute phase reactants or abnormal bone imaging

46
Q

What is the treatment for schnitzler syndrome?

A

Mild – colchicine

Severe - anakinra

47
Q

What is BSA?

A

Body surface area

48
Q

How can you tell if a rash is drug related or graft vs. host disease?

A

Face involvement
Acral involvement
Diarrhoea
all indicate that GvHD more likely

49
Q

What are the main features of GvHD?

A

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

50
Q

What is affected in GvHD?

A
  • Skin
  • Liver
  • GI tract
51
Q

What are the two forms of GvHD?

A

Acute

Chronic

52
Q

What is suggestive of an internal cause?

A

Itching without rash suggestive of internal cause

53
Q

What are the haematological causes of pruritus?

A

lymphoma, polycythemia

54
Q

What are the other causes of pruritus?

A 
Uraemia
Cholestasis
Iron deficiency or iron overload
HIV / Hepatitis A / B / C
Cancer
Drugs (NB opiates / opioids) 
Psychogenic
Pruritus of old age
55
Q

What investigations should you do for pruritus?

A 
FBC, LDH
Renal profile
Liver function tests
Ferritin
XR Chest
HIV / Hepatitis A / B /C
56
Q

What is Nodular prurigo?

A

End of result of scratching

Skin trying to mount a defence

57
Q

What is systemic amyloidosis?

A

Manifestation of underlying plasma cell dyscrasia

Fibrils composed of AL protein (immunoglobulin light chains, usually λ chains)

58
Q

What are the presenting symptoms of systemic amyloidosis?

A

Weight loss

  • Fatigue
  • Paraesthesias
  • Dyspnoea
  • Syncopal attacks (orthostatic hypotension)
59
Q

What are the investigations for systemic amyloidosis?

A

Investigation: abdominal fat / rectal mucosa biopsy – SAP scintigraphy

60
Q

What is the treatment for systemic amyloidosis?

A

melphalan, autologous peripheral blood stem cell transplant, lenalidomide, bortezomib

61
Q

What are the cutaneous features of systemic amyloidosis?

A

Skin involvement in ~ 25%

Petechiae, purpura and ecchymoses - due to infiltration of vessel walls

Periorbital purpura (‘Raccoon sign’) precipitated by: coughing, Valsalva manoeuvre or pinching (pinch purpura)

Waxy, translucent or purpuric papules, nodules

Face, neck, and scalp, anogenital region, digits

62
Q

What are the main features of scurvy?

A

Vitamin C (ascorbic acid) deficiency

Spongy gingivae with bleeding and erosion

Petechiae, ecchymoses, follicular hyperkeratosis

Corkscrew hairs with perifollicular haemorrhage

63
Q

What are the systemic features of kwashiorkor?

A
  • Hepatomegaly
  • Bacterial / fungal infections
  • Diarrhoea
  • Loss of muscle mass
  • Oedema
  • Failure to thrive
64
Q

What causes kwashiorkor?

A

Protein deficiency

65
Q

What are the skin signs of Kwashiorkor?

A
  • Superficial dequamation large areas of erosion
  • Sparse, dry hair
  • Soft, thin nails
  • Cheilitis
66
Q

Why is zinc important?

A

Important role in 200 enzymes – regulation of lipid, protein, nucleic acid synthesis
Roles in wound healing, antioxidant

67
Q

What are the main features of zinc deficiency?

A

Deficiency: genetic (SLC39A4) or acquired

  • Triad of Dermatitis, Diarrhoea, Depression
68
Q

Where in particular are the cutaneous manifestations of zinc deficiency seen?

A

perioral, acral and perineal sites

69
Q

What are the cutaneous manifestations of zinc deficiency?

A
  • Erythema
  • Scale-crusts
  • Erosions
  • Alopecia
  • Stomatitis
  • Conjunctivitis
70
Q

Why is Vitamin B3 important?

A

Required for most cellular processes

71
Q

What are the main features of Vitamin B3 deficiency?

A

Dermatitis
Diarrhoea
Dementia
Death

72
Q

What are the cutaneous manifestations of Vitamin B3 deficiency?

A
  • Photodistributed erythema
  • ‘Casal’s necklace’
  • Painful fissures of the palms and soles
  • Peri-anal and perioral inflammation and erosions
  • Cheilitis and glossitis
  • Vaginitis with erosions
73
Q

What are the main features of Carcinoid syndrom?

A 
Signifies metastases of a malignant carcinoid tumour
5-HT secretion
Flushing in 25% of cases
Other symptoms: 
	- Diarrhoea 
	- Bronchospasm
	- Hypotension
74
Q

What condition constitutes a dermatological emergency?

A

Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis

75
Q

What are the main features of Stevens-Johnson syndrome?

A

Prodromal: flu-like sx
Abrupt onset of lesions on trunk > face/limbs
Macules, blisters, erythema – atypical targetoid
Blisters merge – sheets of skin detachment
Nikolsky +ve (touch extends the problem)

Extensive full thickness mucocutaneous necrosis <2-3 days

76
Q

What is the difference between Stevens-Johnson and Toxic epideraml necrolysis?

A

Same process

SJS less than 10% of body
TEN more than 10%

77
Q

What causes SJS/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

78
Q

What can SJS/TEN resemble?

A

Staphylococcal scalded skin syndrome (SSSS)
Thermal burns
Cutaneous graft versus host disease

79
Q

What score is used to assess severity of SJS/TEN?

A

SCORTEN

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

80
Q

What are the complications of SJS/TEN?

A

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

81
Q

What are the systemic manifestations of erythroderma?

A

manifestations reflect impairment in skin function:

  • Peripheral edema
  • Tachycardia
  • Loss of fluid and proteins
  • Disturbances in thermoregulation
  • Risk of sepsis
82
Q

What are the causes of erythroderma?

A
  • Drug reactions
  • Cutaneous T-cell lymphoma – Sézary syndrome
  • Psoriasis
  • Atopic eczema
  • Idiopathic (25-30%)
83
Q

How is Erythroderma managed?

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

84
Q

What means itch?

A

Excoriations

Prurigo

85
Q

What means dryness?

A

Xerosis

86
Q

What is Calciphylaxis?

A

Calcium blocks the arteries

87
Q

What are systemic manifestations of CKD?

A

Anaemia – mucosal pallor, hair thinning
Excoriations, prurigo
Calciphylaxis
Half and half nails

88
Q

What CKD signs are related to primary disease?

A
  • ANCA-associated vasculitis

- Systemic Lupus Erythematosus

89
Q

What CKD signs are related to immunosurpression?

A
  • Viral warts

- Skin cancer

90
Q

What indicates chronic liver disease?

A 
Excoriations, prurigo
Jaundice
Muehrcke’s lines of nails
Terry’s nails
Palmar erythema
Spider telangiectasia
Clubbing
91
Q

What are the main features of 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

92
Q

What are the manifestations of diabetes?

A 
Terry's nails
Granuloma annulare
Neuropathic ulcers
Acanthosis nigiricans
Xerosis
Xanthelesma & xanthomata 
Skin infections
93
Q

What are the manifestations of hyperlipideamia?

A

Eruptive xanthomas

94
Q

What are the Cutaneous Manifestations of Other Endocrinological Disorders?

A

Pre-tibial myxoedema (Grave’s disease)

Hyperpigmentation (Addison’s disease)

Acne (Acromegaly , cushing’s syndrome, polycystic ovarian syndrome

Cutis gyrata verticis (Acromegaly)

95
Q

What are the cutaneous signs seen in immunosurpession?

A 
Severe seborrhoeic dermatitis
CMV ulceration
Extensive viral warts
Norwegian scabies
Bacillary angiomatosis
96
Q

What are the cutaneous signs seen in HIV?

A

Penicillinosis
Cryptococcosis
Kaposi sarcome

97
Q

What are the variable nonspecific manifestations of HIV?

A
  • Morbilliform rash
  • Urticaria
  • Erythema multiforme
  • Oral / genital ulceration
  • NB Low threshold for testing
98
Q

What are signs of HIV?

A

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

99
Q

What are the cutaneous signs seen in GI systemic diseases?

A 
Panniculitis 
Pyoderma gangrenosum
Dermatitis herpetiformis
Oralfacial granulamotsis
Apthous ulceration
100
Q

What are the main features of hidradentitis suppuritiva?

A

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

101
Q

What are the main features of pyoderma gangrenosum?

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

102
Q

What is the cutaneous manifestation with coeliac disease?

A

Dermatitis herpetiformis

103
Q

What are the cutaneous signs of underlying malignancy?

A 
Peau d'orange
Leukemia cutis
Groin metastases
Metastatic bronchial carcinoma
Haemorrhagic nodules
Extramammary paget's disease
Acanthosis nigricans
Paraneoplastic pemphigus
Erytherma gyratum 
Chilblain like lesions
104
Q

What indicates peutz jegher syndrome?

A

Mucosal melanosis

105
Q

What indicates hereditary leimyomatosis and renal cell cancer?

A

Leiomyomas

106
Q

What are skin diseases associated with malignancy?

A
  • Sweet’s syndrome
  • Dermatomyositis
  • Erythema gyratum repens
  • Pyoderma gangrenosum - Paraneoplastic pemphigus

Year 2 Derm (3 decks)

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