Derm - Skin in Systemic Disease Flashcards
(109 cards)
1
Q
Why is learning about skin in systemic disease important?
A
→ rash may be more than skin deep
→ prevent or reduce internal organ damage by early diagnosis
2
Q
What are the 4 ways in which the skin can be involved in systemic disease?
A
→ skin is targeted by disease
→ skin provides signs of internal disorders
→ tell-tale skin conditions
→ systemic disease can be secondary to the skin disorder
3
Q
What is a punch biopsy?
A
→ skin investigation done under local anaesthetic
→ sent for histology for analysis, etc.
4
Q
What is Lupus Erythematosus?
A
autoimmune disease in which the immune system attacks its own tissues, causing widespread inflammation and tissue damage in the affected organs
5
Q
What are the 2 main types of LE? Is there an overlap between them?
A
→ systemic LE
→ cutaneous (discoid) LE
→ yes, there is some overlap
6
Q
What is the mucocutaneous diagnostic criteria of LE?
A
→ cutaneous lupus (acute) (ring-like plaques) → cutaneous lupus (chronic) → oral ulcers → alopecia (baldness) → chilblains → photo distributed (sun-exposed) rash → livedo reticularis → palpable purpura
7
Q
What is the systemic or internal diagnostic criteria of LE
A
→ synovitis (inflamed joints)
→ serositis (serous tissue inflammation) (pleurisy or pericarditis)
→ renal disorder
→ neurological disorder
8
Q
What is the haematological diagnostic criteria for LE?
A
→ haemolytic anemia
→ thrombocytopenia
→ leukopenia
9
Q
What are the immunological findings in LE?
A
main : auto-antibodies → ANA → anti-dsDNA → anti-Sm → antiphospholipid → low complement → Direct Coomb's test
10
Q
What is the significant presenting differences between the 2 types of LE?
A
→ Cutaneous / Discoid LE has more evident scarring
→ Systemic LE has more ring like plaques, etc.
11
Q
What is neonatal lupus?
A
autoimmune disorder that is congenital
12
Q
What are the presenting signs of neonatal lupus in a newborn?
A
→ ring-like rash / plaques
→ likely to be positive for Ro antibodies
13
Q
What test should immediately be done for newborns w neonatal lupus? Why?
A
→ ECG
→ 50% risk of heart block
→ might need a pacemaker
14
Q
What is dermatomyositis?
A
→ autoimmune connective tissue disorder
→ proximal extensor inflammatory myopathy
15
Q
What are the presenting signs of dermatomyositis?
A
→ Gottron’s papules (plaques on the metacarpal + phalanges)
→ ragged cuticles
→ Shawl Sign (redness of upper back)
→ Heliotrope rash (erythema of the eyelids)
→ photosensitive erythema
16
Q
What are the different subtypes of dermatomyositis classified by?
A
clinical features the can be predicted by autoantibody profile
17
Q
What DM subtype presents with fever, myositis + Gottron’s papules?
A
Anti Jo-1
18
Q
What DM subtype presents with necrotising myopathy?
A
Anti-SRP
19
Q
What DM subtype presents with mild muscle disease?
A
Anti Mi-2
20
Q
What DM subtype is associated with malignancy in adults?
A
Anti-p155
21
Q
What DM subtype is juvenile and usually presents with calcinosis?
A
Anti-p140
22
Q
What DM subtype presents with amyopathia (lack of muscle weakness)?
A
Anti-SAE
23
Q
What DM subtype presents with interstitial lung disease, digital ulcers, ischaemia?
A
Anti-MDA5
24
Q
What diagnostic tests need to be done for dermatomyositis?
A
→ antinuclear antibody test → creatine kinase (looking for increased CK) → skin biopsy → EMG → LFT (looking for increased ALT) → screening for internal malignancy
25
What are the symptoms of IgA vasculitis?
```
→ abdominal pain
→ bleeding
→ Henoch-Schonlein purpura
→ arthralgia
→ arthritis
→ IgA associated glomerulonephritis
```
26
What type of vasculitis presents systemically in the skin?
→ cutaneous small vessel vasculitis
| → benign cutaneous form of Polyartertis Nodosa (medium vessel vasculitis)
27
What are the different causes of cutaneous small vessel vasculitis?
```
→ idiopathic
→ malignancy
→ LE
→ infectious
→ medication exposure
→ inflammatory
→ IgA vasculitis
→ Urticarial vasculitis
→ acute haemorrhagic oedema of infancy
```
28
How do the small vessel manifestations of vasculitis usually present on skin?
purpura (macular or palpable)
29
How do the medium vessel manifestations of vasculitis usually present in skin?
→ digital necrosis
→ retiform purpura ulcers
→ subcutaneous nodules along blood vessels
30
What is sarcoidosis?
systemic granulomatous disorder of unknown origin
can affect multiple organs
most commonly affects lungs
31
How many have cutaneous manifestations of sarcoidosis?
33%
32
How does sarcoidosis present cutaneously?
→ highly variable
→ red-brown to violaceous papules on the face, lips, upper back, neck + extremities
→ can look like lupus pernio (nothing to do with lupus)
→ ulcerative
→ scar sarcoid
→ erythema nodosum (on the legs)
33
What does histology show for sarcoidosis?
non-caseating epthelioid granulomas
34
What is DRESS?
→ Drug reaction with Eosinophilia + Systemic Symptoms
→ rash + systemic upset incorporating haematological + solid-organ disturbances
→ underlying mechanism unknown
35
What is the diagnostic criteria for DRESS?
based on scoring criteria:
→ fever > 38.5
→ lymphadenopathy > 2 sites + more than 1 cm
→ circulating atypical lymphocytes
→ peripheral hypereosinophilia > 0.7 x 10 to the power of 9
→ internal organs involved
→ negative ANA, hepatitis, mycoplasma, chlamydia
→ skin involvement >
36
What internal organs can be involved in DRESS ?
```
→ liver (hepatitis)(most frequent)
→ kidneys (interstitial nephritis)
→ heart (myocarditis)
→ brain
→ thyroid
→ lungs (interstitial pneumonitis)
```
37
What is the onset of DRESS?
2-6 weeks after drug exposure
38
What drugs can trigger DRESS?
```
→ sulfoamides
→ anti-epileptics
→ allopurinol
→ Antibiotics
→ minocycline
→ ibuprofen
```
39
What is the cutaneous manifestation of DRESS?
```
rash:
→ urticated papular exanthem, (widespread papules)
→ maculopapular eruption
→ widespread erythema
→ head / neck oedema
→ erythema multiform-like
```
40
What is the treatment for DRESS?
→ withdrawal of drug
| → corticosteroids are first line
41
What is Graft versus Host Disease?
→ multiple organ disease
| → affects 10% of allogenic haematopoetic stem cell transplants
42
How can you differentiate between GvHD and rash induced by a drug?
→ face involvement
→ acral involvement
→ diarrhoea
43
What is pathogenesis of GvHD?
donor-derived T-lymphocyte activity against antigens in an immunocompromised recipient
44
What parts of the body does GvHD mainly affect?
→ skin
→ liver
→ GI tract
45
What is pruritus?
itching without a rash
46
What does pruritus suggest?
```
suggestive if internal cause:
→ haematological causes (lymphoma, polycythemia)
→ uraemia
→ cholestasis
→ iron deficiency
→ HIV or hepatitis A/B/C
→ cancer
→ drugs (opiates or opioids)
→ psychogenic
→ pruritus of old age
```
47
What investigations need to be carried for pruritus?
```
→ FBC
→ LDH
→ renal profile
→ liverfunction tetss
→ ferritin
→ chest x-ray
→ HIV
→ hepatitis A B C
```
48
What can develop as a result of pruritus?
nodular prurigo
49
What is nodular prurigo?
skin thickening as a defence mechanism due to excessive scratching
50
What is scurvy?
vitamin C deficiency
51
What are the cutaneous signs of scurvy?
→ spongy gingival with bleeding + erosion
→ petechiae, ecchymoses, follicular hyperkeratosis
→ corkscrew hairs with perifollicular haemorrhage
52
What is Kwashiorkor?
protein deficiency
53
What are the systemic features of Kwashiorkor?
```
→ hepatomegaly
→ bacterial or fungal infections
→ diarrhoea
→ loss of muscle mass
→ oedema
→ failure to thrive
```
54
What are the skin signs of Kwashiorkor?
→ superficial desquamation with large areas of erosion
→ sparse + dry hair
→ soft + thin nails
→ cheilitis (inflammation of lips)
55
What is the importance of zinc?
→ important role in 200 enzymes
| → regulates lips, proteins, nucleic acid synthesis
56
What are the causes of zinc deficiency?
→ congenital due to genetic (SLC39A4)
| → acquired
57
What is the Zinc deficiency triad?
→ Dermatitis
→ Depression
→ Diarrhoea
58
How does zinc deficiency manifest in the skin?
perioral + aural + perineal skin in particular is affected with scaly erosive erythema
59
What is the importance of Vitamin B3 in the body?
required for most cellular processes
60
What are the 4 D's of Vitamin B3 (niacin) deficiency?
→ dermatitis
→ dementia
→ diarrhoea
→ death
61
What are the cutaneous manifestations of Vitamin B3 deficiency?
→ photo-distributed erythema
→ Casal's necklace
→ painful fissures of the palms + soles
→ peri-anal, genital + perioral inflammation + erosions
62
What is carcinoid syndrome?
→ metastases of a malignant carcinoid tumour
| → results in 5-HT secretion
63
What are the main symptoms of the carcinoid syndrome?
→ hypotension
→ flushing
→ diarrhoea
→ bronchospasm
64
In how many cases of carcinoid syndrome do you see flushing?
25%
65
What is SJS/TEN?
→ Stevens-Johnson syndrome / Toxic Epidermal Necrolysis
| → Derm emergency
66
What are the symptoms + progression of SJS/TEN?
→ prodromal : flu-like symptoms
→ abrupt onset of lesions on trunk > face/limbs
→ macules, blisters, erythema - atypical taretoid
→ blisters merge to form sheets of skin detachment "like wet wallpaper"
67
Why is SJS/TEN considered an emergency?
extensive full thickness mucocutaneous (epidermal) necrosis (2-3 days)
68
Why is it called SJS/TEN?
it's disease progression:
→ SJS = 10% BSA detachment
→ SJS / TEN = 1-30% BS detachment
→ TEN = more than 30%
69
What causes SJS/TEN?
→ cell-mediated cytotoxic reaction against epidermal cells
→ drugs cause >80% of cases
→ drugs must've started up to 3 weeks prior to onset
70
What drugs can cause SJS/TEN?
```
→ antibiotics
→ beta-lactams
→ sulphonamides
→ allopurinol
→ anti-epileptic drugs
→ phenytoin
→ carbamazepine
→ lamotrigine
```
71
What other diseases can SJS/TEN resemble?
→ staphylococcal scalded skin syndrome
→ thermal burns
→ cutaneous graft versus host disease
72
What is the diagnostic score criteria for SJS/TEN?
```
to help assess severity:
→ age > 40
→ HR
→ initial % epidermal detachment
→ serum urea + glucose + bicarbonate
→ presence of malignancy
```
73
What are the complications of SJS/TEN?
```
→ death (overall mortality fo 30%)
→ blindness
→ dehydration
→ hypo or hyper thermia
→ renal tubular necrosis
→ eroded GI tract
→ interstitial pneumonitis
→ neutropaenia
→ liver
→ heart failure
```
74
What is erythroderma?
generalised erythema affecting >90% BSA
75
What are some of the systematic manifestations of erythroderma?
```
→ peripheral oedema
→ tachycardia
→ loss of fluids + proteins
→ disturbances in thermoregulation
→ risk of sepsis
```
76
What can cause erythroderma?
```
→ drug reactions
→ cutaneous T-cell lymphoma
→ psoriasis
→ atopic eczema
→ idiopathic
```
77
How can erythroderma be managed?
→ underlying cause (e.g. treat psoriasis, withdraw drug if drug is the cause etc.)
→ hospitalisation is necessary
→ restore fluid + electrolyte balance, circulatory status, body temp
→ emollients to support skin barrier
→ topical steroids
→ antibiotics
78
What are some of the skin manifestations of CKD?
→ excoriations
→ prurigo
→ xerosis (dryness)
→ half and half nails
→ calciphylaxis
→ cutaneous signs related to primary disease ( e.g. vasculitis, SLE)
→ cutaneous signs related to immunosuppression (e.g. viral warts, skin cancer)
79
What are some other signs of CKD?
→ anaemia
→ mucosal pallor
→ hair thinning
80
What are the skin manifestations of Chronic Liver Disease?
```
→ excoriations
→ prurigo
→ Clubbing
→ Jaundice
→ Spider Telangiectasia
→ Terry's nails
→ Meuhrcke's lines on nails
→ Palmar erythema
→ Porphyria cutaneous tarda
```
81
What is necrobiosis lipoidica?
plaques with red-brown raised edge with yellow-brown atrophic centre
82
What is the relationship between necrobiosis lipoidica and DM?
20-65% of cases occur in setting of Diabetes Mellitus
83
What is the treatment for necrobiosis lipoidica?
topical or intralesional steroids
84
What are other skin manifestations of Diabetes mellitus?
```
→ Terry's nails
→ Granuloma annulare
→ Neuropathic ulcers
→ Acanthosis Nigricans
→ xerosis (dryness)
→ xanthelesma or xanthomata
→ skin infections
```
85
How does Grave's disease manifest in the skin?
pre-tibial myxoedema
86
How does Addison's disease manifest in the skin?
hyper pigmentation
87
What endocrine disorders can have acne as a symptom?
→ acromegaly
→ Cushing's syndrome
→ PCOS
88
What skin signs can acromegaly have?
cutis gyrata verticis
89
What are some skin manifestations of HIV?
```
→ severe seborrhoea dermatitis
→ extensive viral warts
→ CMV ulceration
→ eosinophilic folliculitis
→ bacillary angiomatosis
→ Norwegian scabies
→ severe psoriasis
→ morbilliform rash
→ urticaria
→ erythema multiforme
→ oral / genital ulceration
→ itch with no rash
```
90
What cutaneous diseases are associated with IBD?
```
→ Pyoderma gangrenosum
→ Orofacial granulomatosis
→ Panniculitis (erythema nodosum)
→ Aphthous ulceration
→ Association with psoriasis, pemphigoid
```
91
What cutaneous diseases are associated with celiac disease?
dermatitis herpetiformis
92
What is hidrandenitis suppuritiva?
causes small, painful lumps to form under the skin
93
What are the symptoms of hidrandenitis suppurtiva?
```
→ Inflamed nodes
→ sterile abscess
→ sinus tracts
→ fistulae
→ hypertrophic scars
```
94
What areas does hidradenitis suppurtiva usually occur in?
Favours intertriginous zones (areas that rub together) : especially axillary, anogenital and inframammary area
95
What are other consequences of hidradenitis suppurtiva?
→ pain
| → bad body odour
96
What are risk factors for HS?
→ high Dmi
→ diabetes
→ IBD
97
What is pyoderma gangrenous?
→ causes large, painful sores (ulcers) to develop on your skin
→ pustule on an erythematous base - ulcerates + extends with necrotic undermined border
98
What systemic disease is pyoderma gangrenous associated with?
in 50-70% of cases:
→ IBD
→ leukaemia
→ seronegative arthritis
99
What are some of the ways in which internal malignancy can present cutaneously?
→ cutaneous metastases
→ malignancy reflecting internal malignancy
→ genetic condition predisposing to internal cancer + skin lesions
→ skin disease associated with malignancy
→ non-specific skin disease
100
What are some non-specific skin disease that could mean internal malignancy?
→ pruritus
→ vasculitis
→ urticaria (hives)
101
What is urticaria?
hives
102
What skin disease are associated with malignancy?
→ dermatomyositis
→ erythema gyratum repens
→ pyoderma gangrenosum
→ paraneoplastic pemphigus
103
What is paraneoplastic pemphigus?
fatal autoimmune blistering disease associated with malignancy
104
What is pyoderma gangrenosum?
large, painful sores (ulcers) to develop on your skin, most often on your legs
105
What is erythema gyratum repens?
rare disease characterized by the cutaneous eruption of migratory concentric erythematous bands
106
What is an example of cutaneous malignant reflection of internal malignancy?
extramammary Paget's disease
107
What are some genetic conditions that predispose to internal cancer + skin lesions?
→ hereditary leiomyomatosis
→ renal cell cancer
→ Peutz-Jeghers syndrome
108
What is Paget' disease?
disease of bone interferes with your body's normal recycling process, in which new bone tissue gradually replaces old bone tissue
109
What is acanthuses nigricans?
skin condition that causes a dark discoloration in body folds and creases
