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systemic skin disease Flashcards

1
Q

What are the differential diagnoses for skin rash

A

Idiopathic, infection, inflammatory, autoimmune, drug-induced, traumatic, metabolic, genetic, neoplastic

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2
Q

What are different investigations carried out for skin rash?

A

Blood tests, microbiology, imaging, skin biopsy, specific

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3
Q

What blood tests are carried out in investigation?

A

Full blood count, renal profile, liver function tests, inflammatory markers, autoimmune serology

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4
Q

What microbiological tests are carried out in investigation?

A

Viral/ Bacterial serology
Swabs for bacterial C and S
Viral PCR
Tissue culture/ PCR

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5
Q

What would imaging be expected to show?

A

Internal organ involvement
Vascular supply

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6
Q

What techniques are used for skin biopsy?

A

Microscopy

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7
Q

What specific investigations are carried out?

A

Urinalysis
Nerve conduction studies
Endocrine investigations

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8
Q

What are the 2 main groups of lupus erythematosus?

A

Systemic lupus erythematosus
Cutaneous (discoid) lupus erythematosus

(overlap)

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9
Q

What are the three main categories of diagnostic criteria for SLE?

A

Mucocutaneous
Haematological
Immunological

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10
Q

What are the mucocutaneous diagnostic criteria for SLE?

A

Cutaneous lupus (acute)
Cutaneous lupus (chronic)
Oral ulcers
Alopecia

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11
Q

What are the haematological diagnostic criteria for SLE?

A

Haemolytic anaemia
Thrombocytopenia
Leukopenia

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12
Q

What are the immunological diagnostic criteria for SLE?

A

ANA
anti-dsDNA
anti-SM
anti-phospholipids
Low complement
Direct Coombe’s test

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13
Q

What are additional systemic diagnostic criteria for SLE?

A

Synovitis
Serositis (pleurites/ pericarditis)
Renal disorder
Neurological disorder

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14
Q

What are the symptoms of SLE?

A

Photodistributed rash
Chillblains
Alopecia
Cutaneous vasculitis
Livedo reticularis
Subacute cutaneous lupus (SCLE)- annular pattern

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15
Q

What are the two ways in which cutaneous lupus erythematosus can manifest?

A

Discoid lupus erythematosus- characteristically causes scarring
SCLE
(overlap)

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16
Q

Neonatal lupus shows positivity for what antibody?

A

Ro

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17
Q

What test is necessary to carry out in cases of neonatal lupus?

A

ECG (50% risk of heart block)

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18
Q

What type of disease is dermatomyositis?

A

Autoimmune connective tissue disease

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19
Q

What type of myopathy occurs in dermatomyositis?

A

Proximal extensor inflammatory myositis?

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20
Q

What can systems review show in cases of dermatomyositis?

A

Weight loss
Fatigue
Weakness

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21
Q

Describe the type of rash you might expect to see in dermatomyositis.

A

Photodistributed pink violet rash, favouring scalp, periocular regions and extensor surfaces

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22
Q

What are some distinctive symptoms of dermatomyositis?

A

Gottron’s papules
Ragged cuticles
Heliotrope rash
Shawl sign
Photosensitive erythema

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23
Q

What can be used to predict the different clinical features of dermatomyositis subtypes?

A

Antibody profile

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24
Q

What investigations are done for dermatomyositis?

A

CK
Liver functions test (ALT often increased)
ANA
Skin biopsy
EMG
Screening for internal malignancy

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25
How can vasculitis be classified based on affected vessels?
Small vessel Small and medium vessel Medium vessel Large vessel
26
Give two examples for large vessel vasculitis
Temporal arteritis Tayakasu
27
Give an example of medium vessel vasculitis and name its two forms
Polyarteritis nodosa (PAN) -benign cutaneous form systemic form
28
Give two classifications for small and medium vessel vasculitis
Cryoglobulinemia - type II and III ANCA- associated -GPA (Wegener) -EGPA (Churg-Strauss) -microscopic polyangitis
29
What are some possible causes for cutaneous small vessel vasculitis?
Idiopathic Infectious Inflammatory Medication exposure
30
Name 4 special types of small vessel vasculitis
IgA Vasculitis (Henoch-Schonlein) Urticarial vasculitis Acute haemorrhagic oedema of infancy Erythema elevatum diutinum
31
What are small vessel manifestations of vasculitis?
Purpura (macular or papular)
32
Name some medium vessel manifestations of vasculitis
Digital necrosis Retiform purpura Ulcers Subcutaneous nodules along blood vessels
33
What are some systemic symptoms of IgA vasculitis?
Abdominal pain, GI bleeding Arthritis, arthralgia IgA-associated glomerulonephritis -MONITOR URINE
34
What type of disorder is sarcoidosis?
Systemic granulomatous disorder of unknown origin Can affect multiple organs (most commonly lungs) Requires evaluation for internal organ involvement Cutaneous manifestations in 33% Highly variable- the great mimicker
35
What are the cutaneous manifestations of sarcoidosis?
Red-brown to violaceous papules on face, lips, neck, upper back and extremities *Lupus pernio* Ulcers Scar sarcoid Erythema nodosum
36
What does histological investigation show in sarcoidosis?
Non-caseating epithelioid granulomas
37
What are the features of Drug Reaction with Eosinophilic and Systemic Symtoms (DRESS)
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 Biopsy suggestive of DRESS
38
What internal organs are involved in DRESS
Liver (hepatitis- most frequent cause of death) Kidney- interstitial nephritis Heart- myocarditis Lungs- interstitial pneumonitis Brain Thyroid- thyroiditis
39
What is the underlying mechanism for DRESS?
Unknown
40
How soon after drug exposure does DRESS start?
2-6 weeks
41
What are some common drug triggers for DRESS?
Ibuprofen Antibiotics (sulphonamides, vancomycin, amoxicillin, minocycline, piperacillin tazobactam) Allopurinol Anti-epileptics (phenytoin, carbamazepine, lamotrigine)
42
What are the rash morphologies in DRESS?
Morbilliform eruption Erythema multiforme-like Erythroderma/ widespread exfoliative erythema Head/ neck oedema Urticated papular exanthem
43
Treatment for DRESS?
Withdrawal of culprit Corticosteroids are first line treatment- may require months of treatment
44
DRESS mortality?
5-10%
45
What are indications that a rash is more likely caused by GvHD than drug reaction?
Facial involvement Acral involvement Diarrhoea
46
Pathogenesis of GvHD?
Donor derived T-lymphocyte activity against antigens in an immunocompromised recipient
47
What organs are mainly affected by GvHD?
Skin Liver GI tract
48
Two major forms of GvHD?
Acute Chronic
49
What does itching without rash suggest?
Internal cause: -Haematological cause (lymphoma, polycythaemia) -Iron deficiency/ iron overload -Uraemia -Cholestasis -HIV/ Hepatitis A/B/C -Cancer -Drugs (Opiates/ opioids) -Psychogenic -Pruritis of old age
50
Investigations carried out in itching without rash?
FBC, LDH Ferritin Renal profile Liver function tests HIV/ Hepatitis A/B/C XR chest
51
What skin symptom can chronic itching cause?
Nodular prurigo
52
Features of Vitamin C (ascorbic acid) deficiency
Spongy gingivae with bleeding and erosion Petechiae, ecchymoses, follicular hyperkeratosis Corkscrew hairs with perifollicular haemorrhage
53
What deficiency causes Kwashiorkor?
Protein deficiency
54
Skin signs of kwashiorkor?
Superficial desquamation with large areas of erosion Sparse, dry hair Soft, thin nails Cheilitis
55
What is zinc important for?
Important role in 200 enzymes- regulation of lipid, protein and nucleic acid synthesis
56
What are the causes of zinc deficiency?
Genetic (SLC39A4) Acquired
57
What are the symptoms of zinc deficiency?
Triad of dermatitis, diarrhoea, depression Cutaneous manifestations- scaly erosive erythema (particularly in perioral, acral and perineal sites)
58
What causes carcinoid syndrome?
Significant metastases of a malignant carcinoid tumor 5-HT secretion
59
What are symptoms of carcinoid syndrome?
Flushing in 25% of cases Diarrhoea Bronchospasm Hypotension
60
What are symptoms of Stevens-Johnson syndrome/ toxic epidermal necrolysis?
Prodromal: flu-like symptoms Abrupt onset of lesions on trunk> face/ limbs Macules, blisters, erythema- atypical targeted blisters merge- sheets of skin detachment (wet wallpaper) Extensive full mucocutaneous necrosis <2-3 days
61
What is the BSA detahcment percentage for a)SJS b)SJS/ TEN c)TEN
a) <10 b) 10-30 c) >30
62
What is the mortality % for a) SJS b)TEN
a) <=10 b)>=30
63
What kind of cellular reaction mediates SJS/TEN?
Cell-mediated cytotoxic reaction against epidermal cells
64
What causes >80% cases of SJS/TEN?
Drugs -antibiotics (sulphonamides, beta-lactams) -allopurinol -anti-epileptics (phenytoin, carbamazepine, lamotrigine) -NSAIDs
65
What are differential diagnoses for symptoms of SJS/TEN?
Staphylococcal scalded skin syndrome (SSSS) Thermal burns cutaneous GvHD
66
What scoring system is used to assess severity in SJS/TEN?
SCORETEN Criteria age>40 Heart rate Initial % epidermal detachment serum urea +glucose+bicarbonate presence of malignancy
67
What are complications of SJS/TEN?
Death Blindness Dehydration Hypothermia/ hyperthermia Renal tubular necrosis Eroded GI tract neutropenia Interstitial pneumonitis Liver failure Heart Failure
68
What is erythroderma?
Generalised erythema affecting >90% BSA
69
What are the systemic manifestations of erythroderma?
Loss of fluid and proteins peripheral oedema disturbances in thermoregulation risk of sepsis tachycardia
70
What are some of the multiple aetiologies of erythroderma?
Idiopathic Psoriasis Atopic eczema Drug reactions Cutaneous T cell lymphoma- Sézary syndrome
71
How is erythroderma managed?
Treat underlying cause (Treat psoriasis, withdraw drug if cause etc.) Hospitalise if systemically unwell Restore fluid and electrolyte balance, circulatory status and manage body temperature Emollient to support skin barrier +/- topical steroids +/- ABx
72
What are some cutaneous signs of CKD?
Anaemia (mucosal pallor, hair thinning) Excoriations/ prurigo Calciphylaxis Half and half nails
73
What are cutaneous signs of CKD related to immunosuppression?
Viral warts Skin cancer
74
CKD cutaneous signs related to primary disease?
ANCA associated vasculitis SLE
75
What are cutaneous signs of chronic liver disease?
Excoriations/ prurigo Jaundice Muehrcke's lines of nails Terry's nails Clubbing Palmar erythema Spider telangiectasia
76
In what setting does 20-65% of necrobiosis lipoidica occur?
Diabetes mellitus
77
What kind of skin markings are seen in necrobiosis lipoidica?
Plaques with red-brown raised edges with yellow-brown atrophic centre
78
What is the treatment for necrobiosis lipoidica?
Topical/ intralesional steroids
79
What are the cutaneous manifestations of diabetes mellitus?
Necrobiosis lipoidica Neuropathic ulcers Skin infections Xerosis Acanthus nigricans Xanthom and xanthelesma Granuloma annulare Terry's nails
80
Cutaneous manifestations of hyperlipidemia?
Eruptive xanthoma
81
Cutaneous manifestation of Graves' disease?
Pretibial myxoedema
82
Hyperpigmentation is a cutaneous manifestation of what endocrinological disorder?
Addison's disease
83
In what endocrine disorders is acne a possible cutaneous disorder?
Acromegaly, Cushing's, PCOS
84
What are two cutaneous manifestations of acromegaly?
Acne Cutis vertices gyrata
85
What are cutaneous manifestations of HIV seroconversion?
Morbilliform rash, erythema multiform-like, urticaria, ulceration (oral/genital)
86
features of hidradenitis suppuritiva?
Inflamed nodes, sterile abscess, sinus tracts, fistulae and hypertrophic scars favours intertriginous zones: especially axillary, anogenital and inframammry areas associated with smoking, obesity and IBS
87
Describe the cutaneous features of pyoderma gangrenosum
Pustules on erythematous base- ulcerates and extends with a necrotic undermined border Painful
88
What diseases is pyoderma gangrenous associated with?
Inflammatory bowel disease Leukemia Seronegative arthritis in 50-70% of cases
89
What cutaneous diseases are associated with inflammatory bowel disease?
Pyoderma gangrenosum Panniculitis Orofacial granulomatosis Aphthous ulceration Associated with psoriasis, pemphigoid
90
Name a cutaneous manifestation of celiac disease
Dermatitis herpetiformis
91
Name a malignancy reflecting an internal malignancy
Extramammary Paget's diseases
92
Genetic conditions leading to predisposition to internal cancer and skin lesions?
Hereditary leiomyomatosis and renal cell cancer Peutz-Jeghers syndrome
93
Skin disease associated with malignancy?
Pyoderma gangrenosum Paraneoplastic pemphigus Erythema gyratum repens Dermatomyositis
94
non specific skin disease associated with internal malignancy?
Pruritis vasculitis urticaria

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