The skin in systemic disease Flashcards
(106 cards)
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
2
Q
How can systemic disease manifest in the skin?
A
Skin targeted
Skin signs
‘Tell-tale’ skin conditions
Secondary systemic involvement
3
Q
Give an example of skin targeted disease?
A
Multi-organ systemic disease targeting skin e.g. Sarcoidosis
4
Q
Give an example of skin signs in disease?
A
Sign of internal disorder e.g. flushing in Carcinoid syndrome
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
6
Q
Give an example of secondary systemic involvement?
A
Systemic disease secondary to skin disorder e.g. high output cardiac failure in erythroderma
7
Q
What are the different diagnosis categories?
A
Idiopathic Neoplastic Infection Inflammatory Drug-induced Auto immune Traumatic Metabolic Genetic
8
Q
What are the two main types of lupus?
A
Systemic Lupus Erythematosus
Cutaneous (Discoid) Lupus Erythematosus
- Overlap
9
Q
What are the mucocutaneous manifestations of systemic lupus?
A
Cutaneous lupus - acute Cutaneous lupus - chronic Mucocutaneous Oral ulcers Alopecia Synovitis
10
Q
What are the other manifestations of systemic lupus?
A
Synovitis
Serositis (pleurisy or pericarditis)
Renal disorder
Neurological disorde
11
Q
What are the haematological manifestations of systemic lupus?
A
Haemolytic anaemia
Thrombocytopenia
Leukopenia
12
Q
What are the immunological criteria for systemic lupus diagnosis?
A
ANA Anti-dsDNA Anti-Sm Antiphospholipid Low Complement Direct Coomb’s test
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)
14
Q
What are the cutaneous manifestations of cutaneous lupus?
A
Discoid lupus erythematosus SCLE
15
Q
What is the test that must be conducted if neonatal lupus is suspected?
A
Test ECG
– risk of heart block (50% risk)
16
Q
What is dermatomyositis?
A
Autoimmune connective tissue disease
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
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
19
Q
What are the signs seen in dermatomyositis?
A
Gottron's papules Ragged cuticles Shawl sign Helitrope rash Photosensitive erythema
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
21
Q
How do you diagnose dermatomyositis?
A
ANA CK Skin biopsy LFT (ALT often increased) EMG Screening for internal malignancy
22
Q
What are the different types of vasculitis?
A
Small
Small and medium
Medium
Large
Vessel
23
Q
What is the classification of small vessel vasculitis?
A
Cutaneous small vessel (leukocytoclastic) vasculitis
- Idiopathic
- Infectious
- Medication exposure
- Inflammatory (connective tissue disease
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
25
What is the classification of small and medium vessel vasculitis?
Cryoglobulinemia
- Type II & III
ANCA-associated
- EGPA (Churg-Strauss)
- Microscopic Polyangiitis,
- GPA (Wegener)
26
What is the classification of medium vessel vasculitis?
Polyarteritis nodosa (PAN)
- Benign cutaneous form
- Systemic form
27
What is the classification of large vessel vasculitis?
Temporal arteritis
| Tayakasu
28
What are the manifestation of small vessel vasculitis?
Purpura (macular / palpable)
29
What are the manifestation of medium vessel vasculitis?
```
Digital necrosis
Retiform purpura
Ulcers
Retiform purpura
Ulcers
Subcutaneous nodules along blood vessels
```
30
What are the main features of IgA Vasculitis?
Initial presentation often indistinguishable from other small vessel vasculitis
Abdominal pain, bleeding (65%), arthralgia / arthritis (64%)
IgA-associated glomerulonephritis (40%)
31
What are the main features of granulomatosis with polyangiitis?
Cough, dyspnoea, and chest pain:
Pulmonary infiltrates (70%)
Glomerulonephritis (85%)
Cutaneous findings (50%)
32
What are the small vessel manifestations of granulmatosis with polyangiitis?
Purpura (macular / flat or popular / palpable)
33
What are the medium vessel manifestations of granulmatosis with polyangiitis?
- Retiform purpura
- Digital necrosis
- Livedo reticularis
- Subcutaneous nodules distributed along blood vessels
- Ulcers
34
What are the main features of sarcoidosis?
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
35
What is DRESS?
Drug Reaction with Eosinophilia and Systemic Symptoms
36
What are the symptoms of DRESS?
Rash and systemic upset incorporating haematological and solid‐organ disturbances
37
What are the manifestations of DRESS?
- 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
38
What are the internal organ involvements in DRESS?
Liver (hepatitis) - most frequent cause of death- Kidneys (interstitial nephritis) - Heart (myocarditis) - Brain - Thyroid (thyroiditis) - Lungs (interstitial pneumonitis)
39
What are the diagnostic criteria for DRESS?
```
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
```
40
What causes DRESS?
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
41
What drugs have been known to cause DRESS?
Sulfonamides, anti-epileptics (carbamazepine, phenytoin, lamotrigine), allopurinol, Antibiotics (vancomycin, amoxicillin, minocycline, piperacillin-tazobactam), ibuprofen are common triggers
42
What is the morphology of DRESS rashes?
Urticated papular exanthem - widespread papules
- Morbilliform eruption
- Erythroderma / widespread exfoliative erythema
- Head / neck oedema
- Erythema multiforme-like
43
What is the treatment for DRESS?
Withdrawal of culprit
Corticosteroids are first line treatment - may require months of treatment
Mortality 5-10%
44
What is schnitzler syndrome?
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
What are the symptoms of schnitzler syndrome?
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
What is the treatment for schnitzler syndrome?
Mild – colchicine
| Severe - anakinra
47
What is BSA?
Body surface area
48
How can you tell if a rash is drug related or graft vs. host disease?
Face involvement
Acral involvement
Diarrhoea
all indicate that GvHD more likely
49
What are the main features of GvHD?
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
What is affected in GvHD?
- Skin
- Liver
- GI tract
51
What are the two forms of GvHD?
Acute
| Chronic
52
What is suggestive of an internal cause?
Itching without rash suggestive of internal cause
53
What are the haematological causes of pruritus?
lymphoma, polycythemia
54
What are the other causes of pruritus?
```
Uraemia
Cholestasis
Iron deficiency or iron overload
HIV / Hepatitis A / B / C
Cancer
Drugs (NB opiates / opioids)
Psychogenic
Pruritus of old age
```
55
What investigations should you do for pruritus?
```
FBC, LDH
Renal profile
Liver function tests
Ferritin
XR Chest
HIV / Hepatitis A / B /C
```
56
What is Nodular prurigo?
End of result of scratching
| Skin trying to mount a defence
57
What is systemic amyloidosis?
Manifestation of underlying plasma cell dyscrasia
| Fibrils composed of AL protein (immunoglobulin light chains, usually λ chains)
58
What are the presenting symptoms of systemic amyloidosis?
Weight loss
- Fatigue
- Paraesthesias
- Dyspnoea
- Syncopal attacks (orthostatic hypotension)
59
What are the investigations for systemic amyloidosis?
Investigation: abdominal fat / rectal mucosa biopsy – SAP scintigraphy
60
What is the treatment for systemic amyloidosis?
melphalan, autologous peripheral blood stem cell transplant, lenalidomide, bortezomib
61
What are the cutaneous features of systemic amyloidosis?
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
What are the main features of scurvy?
Vitamin C (ascorbic acid) deficiency
Spongy gingivae with bleeding and erosion
Petechiae, ecchymoses, follicular hyperkeratosis
Corkscrew hairs with perifollicular haemorrhage
63
What are the systemic features of kwashiorkor?
- Hepatomegaly
- Bacterial / fungal infections
- Diarrhoea
- Loss of muscle mass
- Oedema
- Failure to thrive
64
What causes kwashiorkor?
Protein deficiency
65
What are the skin signs of Kwashiorkor?
- Superficial dequamation large areas of erosion
- Sparse, dry hair
- Soft, thin nails
- Cheilitis
66
Why is zinc important?
Important role in 200 enzymes – regulation of lipid, protein, nucleic acid synthesis
Roles in wound healing, antioxidant
67
What are the main features of zinc deficiency?
Deficiency: genetic (SLC39A4) or acquired
- Triad of Dermatitis, Diarrhoea, Depression
68
Where in particular are the cutaneous manifestations of zinc deficiency seen?
perioral, acral and perineal sites
69
What are the cutaneous manifestations of zinc deficiency?
- Erythema
- Scale-crusts
- Erosions
- Alopecia
- Stomatitis
- Conjunctivitis
70
Why is Vitamin B3 important?
Required for most cellular processes
71
What are the main features of Vitamin B3 deficiency?
Dermatitis
Diarrhoea
Dementia
Death
72
What are the cutaneous manifestations of Vitamin B3 deficiency?
- 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
What are the main features of Carcinoid syndrom?
```
Signifies metastases of a malignant carcinoid tumour
5-HT secretion
Flushing in 25% of cases
Other symptoms:
- Diarrhoea
- Bronchospasm
- Hypotension
```
74
What condition constitutes a dermatological emergency?
Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis
75
What are the main features of Stevens-Johnson syndrome?
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
What is the difference between Stevens-Johnson and Toxic epideraml necrolysis?
Same process
SJS less than 10% of body
TEN more than 10%
77
What causes SJS/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
78
What can SJS/TEN resemble?
Staphylococcal scalded skin syndrome (SSSS)
Thermal burns
Cutaneous graft versus host disease
79
What score is used to assess severity of SJS/TEN?
SCORTEN
| Criteria: age >40, HR, initial % epidermal detachment, serum urea + glucose + bicarbonate, presence of malignancy
80
What are the complications of SJS/TEN?
Death - Overall mortality 30%
Blindness, dehydration, hypothermia/hyperthermia, renal tubular necrosis, eroded GI tract, interstitial pneumonitis, neutropaenia, liver and heart failure
81
What are the systemic manifestations of erythroderma?
manifestations reflect impairment in skin function:
- Peripheral edema
- Tachycardia
- Loss of fluid and proteins
- Disturbances in thermoregulation
- Risk of sepsis
82
What are the causes of erythroderma?
- Drug reactions
- Cutaneous T-cell lymphoma – Sézary syndrome
- Psoriasis
- Atopic eczema
- Idiopathic (25-30%)
83
How is Erythroderma managed?
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
What means itch?
Excoriations
| Prurigo
85
What means dryness?
Xerosis
86
What is Calciphylaxis?
Calcium blocks the arteries
87
What are systemic manifestations of CKD?
Anaemia – mucosal pallor, hair thinning
Excoriations, prurigo
Calciphylaxis
Half and half nails
88
What CKD signs are related to primary disease?
- ANCA-associated vasculitis
| - Systemic Lupus Erythematosus
89
What CKD signs are related to immunosurpression?
- Viral warts
| - Skin cancer
90
What indicates chronic liver disease?
```
Excoriations, prurigo
Jaundice
Muehrcke’s lines of nails
Terry’s nails
Palmar erythema
Spider telangiectasia
Clubbing
```
91
What are the main features of 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
92
What are the manifestations of diabetes?
```
Terry's nails
Granuloma annulare
Neuropathic ulcers
Acanthosis nigiricans
Xerosis
Xanthelesma & xanthomata
Skin infections
```
93
What are the manifestations of hyperlipideamia?
Eruptive xanthomas
94
What are the Cutaneous Manifestations of Other Endocrinological Disorders?
Pre-tibial myxoedema (Grave’s disease)
Hyperpigmentation (Addison’s disease)
Acne (Acromegaly , cushing’s syndrome, polycystic ovarian syndrome
Cutis gyrata verticis (Acromegaly)
95
What are the cutaneous signs seen in immunosurpession?
```
Severe seborrhoeic dermatitis
CMV ulceration
Extensive viral warts
Norwegian scabies
Bacillary angiomatosis
```
96
What are the cutaneous signs seen in HIV?
Penicillinosis
Cryptococcosis
Kaposi sarcome
97
What are the variable nonspecific manifestations of HIV?
- Morbilliform rash
- Urticaria
- Erythema multiforme
- Oral / genital ulceration
- NB Low threshold for testing
98
What are signs of HIV?
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
What are the cutaneous signs seen in GI systemic diseases?
```
Panniculitis
Pyoderma gangrenosum
Dermatitis herpetiformis
Oralfacial granulamotsis
Apthous ulceration
```
100
What are the main features of hidradentitis suppuritiva?
Inflamed nodes, sterile abscess, sinus tracts, fistulae and hypertrophic scars
Favours intertriginous zones: especially axillary, anogenital and inframammary area
101
What are the main features of pyoderma gangrenosum?
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
What is the cutaneous manifestation with coeliac disease?
Dermatitis herpetiformis
103
What are the cutaneous signs of underlying malignancy?
```
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
What indicates peutz jegher syndrome?
Mucosal melanosis
105
What indicates hereditary leimyomatosis and renal cell cancer?
Leiomyomas
106
What are skin diseases associated with malignancy?
- Sweet’s syndrome
- Dermatomyositis
- Erythema gyratum repens
- Pyoderma gangrenosum - Paraneoplastic pemphigus
