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Yr2 lily BRS Derm > the skin in systemic disease > Flashcards

the skin in systemic disease Flashcards

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1
Q

why is it important to look at the skin?

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

in what ways can the skin play a role in systemic disease?

A

skin targeted -in diseases such as sarcoidosis

skin signs - may be present in internal disorders, such as flushing in carcinoid syndrome

tell-tale skin conditions - Skin conditions suggestive of underlying condition e.g. Pyoderma gangrenosum in inflammatory bowel disease

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

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3
Q

what is a punch biopsy?

A

skin investigation taken under local anaesthetic

can be sent for histology - cells and inflammatory patterns and cellular abnormalities

checks for neoplasia or invasive cancer

can also be sent for immuno flourescencing, in which auto antibodies can be detected

they can also be sent for tissue culture

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4
Q

what is lupus erythematosus?

A

Two main groups:
Systemic Lupus Erythematosus
Cutaneous (Discoid) Lupus Erythematosus
- Overlap

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5
Q

what is the diagnostic criteria for systemic lupus erythematosus?

A

Cutaneous lupus - acute
Cutaneous lupus - chronic {Mucocutaneous}
Oral ulcers
Alopecia

Synovitis (joints)
Serositis (pleurisy or pericarditis)
Renal disorder
Neurological disorder

Haemolytic anaemia
Thrombocytopenia {Haematological}
Leukopenia

ANA
Anti-dsDNA
Anti-Sm
Antiphospholipid			{Immunological}
Low Complement
Direct Coomb’s test
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6
Q

what are some symptoms (visible) of SLE?

A

Photodistributed rash (on areas exposed to the sun)
Cutaneous vasculitis
Chilblains
Alopecia
Livedo reticularis (nettle like erythema)
palpable purpura
Cutaneous vasculitis
Subacute cutaneous lupus (SCLE) (ring like/annular plaque) (also seen in cutaneous lupus erythemqtosus)

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7
Q

what are some symptoms of cutaneous lupus erythematosus?

A

in 95% of cases, no other organ involves - cutaneous only

distinct scarring (eg. on scalp and cheek)

also subacute cutaneous lupus (SCLE)

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8
Q

what is neonatal lupus?

A

one exception where you can make a spot diagnosis (dont need differential)

neonatal lupus (Ro positive)

Test ECG
– comes with arisk of heart block (50% risk)

shows up with annular/ring like plaques - subacute cutaneous lupus (SCLE)

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9
Q

what is dermatomyositis?

A

Autoimmune connective tissue disease

Proximal extensor inflammatory myopathy (muscle weakness)

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

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10
Q

what are some distinct signs of dermatomyositis?

A

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

Gottron’s papules - metacarpophylangeal joints and distal interphylangeal joints, vereacious plaques

Ragged cuticles - of nails

Shawl sign - redness on upper back/trunk

Heliotrope rash - erythema of the eyelids

muscle weakness

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11
Q

what are the subtypes of dermatomyositis?

A

Subtypes with clinical features that can be predicted by autoantibody profile

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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12
Q

what investigations are done for dermatomyositis?

A 
ANA (antinuclear antibodies)
CK (creatine kinase) (muscles)
Skin biopsy
LFT (ALT often increased) 
EMG (electromyography)
Screening for internal malignancy
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13
Q

what is IgA vasculitis?

A

vasculitis effecting the small blood vessels

Abdominal pain, bleeding, arthralgia, arthritis, IgA- associated, macula purpura (flat)
glomerulonephritis- may develop later

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14
Q

how does small vessel vasculitis present?

A

Purpura (macular / palpable)

macular is flat, papular is raised

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15
Q

how does medium vessel vasculitis present?

A

Digital necrosis

Subcutaneous nodules along blood vessels

Retiform purpura Ulcers

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16
Q

what is ANCA associated vasculitis?

A

small and medium vessel

can get nose saddle deformities

cough, dyspnoea, chest pain

ulcerative rash

stuffy nose and nosebleeds

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17
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 (nothing to do with lupus, redness on face) (often on nose ears and lips, often shiny, blue-red)
    Ulcerative
    Scar sarcoid
    Erythema nodosum (lower legs, fat inflammation)

Histology – non-caseating epithelioid granulomas (no necrosis)

Diagnosis of exclusion

Requires evaluation for internal organ involvement

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18
Q

what is DRESS?

A

Drug Reaction with Eosinophilia and Systemic Symptoms

Rash and systemic upset incorporating haematological and solid‐organ disturbances

Underlying mechanism not known

Starts 2-6 weeks after drug exposure

Liver most common internal organ involved – majority of deaths associated with this

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

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19
Q

what are 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
Cutaneous eruption suggestive of DRESS e.g. facial oedema
Biopsy suggestive of DRESS

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20
Q

what internal organ involvement is present 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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21
Q

what are the rash morphologies associated with DRESS?

Study These Flashcards
A
  • Urticated papular exanthem - widespread papules
  • Maculopapular (morbilliform) eruption
  • Widespread erythema (Erythroderma)
  • Head / neck oedema
  • Erythema multiforme-like (target like lesions)
22
Q

how is DRESS treated?

Study These Flashcards
A

Withdrawal of culprit

Corticosteroids are first line treatment - may require months of treatment

Mortality 5-10%

23
Q

what is graft versus host disease?

Study These Flashcards
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

Mainly affects:

- Skin 
- Liver
- GI tract
24
Q

how can you tell if as rash is caused by DRESS or graft versus host disease?

Study These Flashcards
A

Face involvement
Acral (didtal limbs) involvement
Diarrhoea

all indicate that GvHD more likely

25
what is pruritus?
``` 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 ``` may cause Nodular prurigo a defence mechanism, thickening of the skin
26
what investigations are done if someone has pruritus?
``` FBC, LDH Renal profile Liver function tests Ferritin XR Chest HIV / Hepatitis A / B /C ```
27
what is scurvy?
Vitamin C (ascorbic acid) deficiency symptoms: Spongy gingivae with bleeding and erosion Petechiae, ecchymoses, follicular hyperkeratosis Corkscrew hairs with perifollicular haemorrhage
28
what is kwashiorkor?
``` Protein deficiency Systemic features: - Hepatomegaly - Bacterial / fungal infections - Diarrhoea - Loss of muscle mass - Oedema - Failure to thrive Skin signs: - Superficial desquamation large areas of erosion - Sparse, dry hair - Soft, thin nails - Cheilitis ```
29
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 (dietary) - Triad of Dermatitis | Diarrhoea | Depression Perioral, acral and perineal skin in particular is affected with scaly erosive erythema
30
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
31
what is carcinoid syndrome?
Signifies metastases of a malignant carcinoid tumour 5-HT secretion (serotonin) (only happens when the tumour has metastasised) ``` Flushing in 25% of cases Other symptoms: - Diarrhoea - Bronchospasm - Hypotension ``` (flushing, with wheezing, light headedness and diarrhoea is strongly suggestive)
32
what is stevens-johnson syndrome (aka toxic epidermal necrolysis)?
Derm emergency! (Rare) Prodromal: flu-like symptoms 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 Complications Death - Overall mortality 30% Blindness, dehydration, hypothermia/hyperthermia, renal tubular necrosis, eroded GI tract, interstitial pneumonitis, neutropaenia, liver and heart failure
33
what is the pathway from stevens johnson syndrome to toxic epidermal necrolysis?
BSA Detachment (%): SJS- <10 SJS/TEN- 10-30 TEN- >30 ``` Mortality (%) -SCORTEN -ABCD-10: SJS- ≤10 TEN- ≥30 ```
34
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 ``` DDx: Staphylococcal scalded skin syndrome (SSSS) Thermal burns Cutaneous graft versus host disease erythema multiforme ``` causes: antibiotics (Beta-lactams Sulphonamides), antiepileptics (Phenytoin Carbamazepine Lamotrigine), allopurinol, NSAIDs
35
how is the severity and prognosis of SJS/TEN determined?
SCORTEN – score used to help assess severity Criteria: age >40, HR, initial % epidermal detachment, serum urea + glucose + bicarbonate, presence of malignancy Complications Death - Overall mortality 30% Blindness, dehydration, hypothermia/hyperthermia, renal tubular necrosis, eroded GI tract, interstitial pneumonitis, neutropaenia, liver and heart failure
36
what is erythroderma?
Generalized erythema affecting >90% BSA (body surface area) Systemic manifestations reflect impairment in skin function: - Peripheral edema - Tachycardia - Loss of fluid and proteins - Disturbances in thermoregulation - Risk of sepsis
37
what may cause erythroderma?
Multiple etiologies: - Drug reactions - Cutaneous T-cell lymphoma – Sézary syndrome - Psoriasis - Atopic eczema - Idiopathic (25-30%)
38
how is erythroderma managed?
support the skin function 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
39
what are some cutaneous signs of systemic disease?
excoriation/prurigo xerosis (dry skin) half and half nails calciphylaxis (big black crustyness) muehrckes lines (white stripes on nails) jaundice spider telalngiectasia (spider veins) terrys nails palmar erythema Porphyria cutaneous tarda clubbing
40
how does chronic kidney disease effect the skin?
Anaemia – mucosal pallor, hair thinning Excoriations, prurigo Calciphylaxis Half and half nails Signs related to primary disease that caused the kidney disease - ANCA-associated vasculitis - Systemic Lupus Erythematosus Signs related to immunosuppression - Viral warts - Skin cancer
41
how does chronic liver disease effect the skin?
``` Excoriations, prurigo Jaundice Muehrcke’s lines of nails Terry’s nails Palmar erythema Spider telangiectasia Clubbing ```
42
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
43
how might diabetes mellitus effect the skin?
Terry’s nails Granuloma annulare Neuropathic ulcers Acanthosis nigricans Xerosis Xanthelesma & xanthomata Skin infections
44
what are some ways in which endocrine diseases can effect the skin?
Pre-tibial myxoedema (Grave’s disease) Hyperpigmentation (Addison’s disease) Acne (Acromegaly , cushing’s syndrome, polycystic ovarian syndrome Cutis gyrata verticis (Acromegaly) (weird head wrinkles)
45
how might HIV effect the skin in general?
may cause sever forms of common conditions ``` such as severe: Severe seborrhoeic dermatitis Extensive viral warts Norwegian scabies Severe psoriasis CMV ulceration Eosinophilic folliculitis Kaposi sarcoma Bacillary angiomatosis ```
46
what are the effects 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
47
how do GI disorders effect the skin?
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
48
what is hidradenitis suppuritiva
Inflamed nodes, sterile abscess, sinus tracts, fistulae and hypertrophic scars Favours intertriginous zones: especially axillary, anogenital and inframammary area devastating condition seen in people with high BMA
49
what is 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
50
what are the 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
51
what are some specific skin conditions in different cancers?
Peau d’orange – breast carcinoma Groin metastases (prostatic carcinoma Metastatic bronchial carcinoma (skin on shoulders) Metastatic oesophageal carcinoma (skin on fingers) Leukemia cutis (blackening of arms) Haemorrhagic nodules (metastatic pancreatic carcinoma Extramammary Paget’s disease (groin) Paget’s disease of the nipple Acanthosis nigricans

Yr2 lily BRS Derm (2 decks)

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