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systemic skin disease Flashcards

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Dermatology Board Prep 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
Q

How can vasculitis be classified based on affected vessels?

A

Small vessel
Small and medium vessel
Medium vessel
Large vessel

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26
Q

Give two examples for large vessel vasculitis

A

Temporal arteritis
Tayakasu

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27
Q

Give an example of medium vessel vasculitis and name its two forms

A

Polyarteritis nodosa (PAN)
-benign cutaneous form
systemic form

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28
Q

Give two classifications for small and medium vessel vasculitis

A

Cryoglobulinemia
- type II and III

ANCA- associated
-GPA (Wegener)
-EGPA (Churg-Strauss)
-microscopic polyangitis

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29
Q

What are some possible causes for cutaneous small vessel vasculitis?

A

Idiopathic
Infectious
Inflammatory
Medication exposure

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30
Q

Name 4 special types of small vessel vasculitis

A

IgA Vasculitis (Henoch-Schonlein)
Urticarial vasculitis
Acute haemorrhagic oedema of infancy
Erythema elevatum diutinum

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31
Q

What are small vessel manifestations of vasculitis?

A

Purpura (macular or papular)

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32
Q

Name some medium vessel manifestations of vasculitis

A

Digital necrosis
Retiform purpura
Ulcers
Subcutaneous nodules along blood vessels

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33
Q

What are some systemic symptoms of IgA vasculitis?

A

Abdominal pain, GI bleeding
Arthritis, arthralgia
IgA-associated glomerulonephritis -MONITOR URINE

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34
Q

What type of disorder is sarcoidosis?

A

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

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35
Q

What are the cutaneous manifestations of sarcoidosis?

A

Red-brown to violaceous papules on face, lips, neck, upper back and extremities
Lupus pernio
Ulcers
Scar sarcoid
Erythema nodosum

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36
Q

What does histological investigation show in sarcoidosis?

A

Non-caseating epithelioid granulomas

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37
Q

What are the features of Drug Reaction with Eosinophilic and Systemic Symtoms (DRESS)

A

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

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38
Q

What internal organs are involved in DRESS

A

Liver (hepatitis- most frequent cause of death)
Kidney- interstitial nephritis
Heart- myocarditis
Lungs- interstitial pneumonitis
Brain
Thyroid- thyroiditis

39
Q

What is the underlying mechanism for DRESS?

A

Unknown

40
Q

How soon after drug exposure does DRESS start?

A

2-6 weeks

41
Q

What are some common drug triggers for DRESS?

A

Ibuprofen
Antibiotics (sulphonamides, vancomycin, amoxicillin, minocycline, piperacillin tazobactam)
Allopurinol
Anti-epileptics (phenytoin, carbamazepine, lamotrigine)

42
Q

What are the rash morphologies in DRESS?

A

Morbilliform eruption
Erythema multiforme-like
Erythroderma/ widespread exfoliative erythema
Head/ neck oedema
Urticated papular exanthem

43
Q

Treatment for DRESS?

A

Withdrawal of culprit
Corticosteroids are first line treatment- may require months of treatment

44
Q

DRESS mortality?

A

5-10%

45
Q

What are indications that a rash is more likely caused by GvHD than drug reaction?

A

Facial involvement
Acral involvement
Diarrhoea

46
Q

Pathogenesis of GvHD?

A

Donor derived T-lymphocyte activity against antigens in an immunocompromised recipient

47
Q

What organs are mainly affected by GvHD?

A

Skin
Liver
GI tract

48
Q

Two major forms of GvHD?

A

Acute
Chronic

49
Q

What does itching without rash suggest?

A

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
Q

Investigations carried out in itching without rash?

A

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

51
Q

What skin symptom can chronic itching cause?

A

Nodular prurigo

52
Q

Features of Vitamin C (ascorbic acid) deficiency

A

Spongy gingivae with bleeding and erosion
Petechiae, ecchymoses, follicular hyperkeratosis
Corkscrew hairs with perifollicular haemorrhage

53
Q

What deficiency causes Kwashiorkor?

A

Protein deficiency

54
Q

Skin signs of kwashiorkor?

A

Superficial desquamation with large areas of erosion
Sparse, dry hair
Soft, thin nails
Cheilitis

55
Q

What is zinc important for?

A

Important role in 200 enzymes- regulation of lipid, protein and nucleic acid synthesis

56
Q

What are the causes of zinc deficiency?

A

Genetic (SLC39A4)
Acquired

57
Q

What are the symptoms of zinc deficiency?

A

Triad of dermatitis, diarrhoea, depression

Cutaneous manifestations- scaly erosive erythema (particularly in perioral, acral and perineal sites)

58
Q

What causes carcinoid syndrome?

A

Significant metastases of a malignant carcinoid tumor

5-HT secretion

59
Q

What are symptoms of carcinoid syndrome?

A

Flushing in 25% of cases
Diarrhoea
Bronchospasm
Hypotension

60
Q

What are symptoms of Stevens-Johnson syndrome/ toxic epidermal necrolysis?

A

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
Q

What is the BSA detahcment percentage for
a)SJS
b)SJS/ TEN
c)TEN

A

a) <10
b) 10-30
c) >30

62
Q

What is the mortality % for
a) SJS
b)TEN

A

a) <=10
b)>=30

63
Q

What kind of cellular reaction mediates SJS/TEN?

A

Cell-mediated cytotoxic reaction against epidermal cells

64
Q

What causes >80% cases of SJS/TEN?

A

Drugs
-antibiotics (sulphonamides, beta-lactams)
-allopurinol
-anti-epileptics (phenytoin, carbamazepine, lamotrigine)
-NSAIDs

65
Q

What are differential diagnoses for symptoms of SJS/TEN?

A

Staphylococcal scalded skin syndrome (SSSS)
Thermal burns
cutaneous GvHD

66
Q

What scoring system is used to assess severity in SJS/TEN?

A

SCORETEN
Criteria
age>40
Heart rate
Initial % epidermal detachment
serum urea +glucose+bicarbonate
presence of malignancy

67
Q

What are complications of SJS/TEN?

A

Death
Blindness
Dehydration
Hypothermia/ hyperthermia
Renal tubular necrosis
Eroded GI tract
neutropenia
Interstitial pneumonitis
Liver failure
Heart Failure

68
Q

What is erythroderma?

A

Generalised erythema affecting >90% BSA

69
Q

What are the systemic manifestations of erythroderma?

A

Loss of fluid and proteins
peripheral oedema
disturbances in thermoregulation
risk of sepsis
tachycardia

70
Q

What are some of the multiple aetiologies of erythroderma?

A

Idiopathic
Psoriasis
Atopic eczema
Drug reactions
Cutaneous T cell lymphoma- Sézary syndrome

71
Q

How is erythroderma managed?

A

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
Q

What are some cutaneous signs of CKD?

A

Anaemia (mucosal pallor, hair thinning)
Excoriations/ prurigo
Calciphylaxis
Half and half nails

73
Q

What are cutaneous signs of CKD related to immunosuppression?

A

Viral warts
Skin cancer

74
Q

CKD cutaneous signs related to primary disease?

A

ANCA associated vasculitis
SLE

75
Q

What are cutaneous signs of chronic liver disease?

A

Excoriations/ prurigo
Jaundice
Muehrcke’s lines of nails
Terry’s nails
Clubbing
Palmar erythema
Spider telangiectasia

76
Q

In what setting does 20-65% of necrobiosis lipoidica occur?

A

Diabetes mellitus

77
Q

What kind of skin markings are seen in necrobiosis lipoidica?

A

Plaques with red-brown raised edges with yellow-brown atrophic centre

78
Q

What is the treatment for necrobiosis lipoidica?

A

Topical/ intralesional steroids

79
Q

What are the cutaneous manifestations of diabetes mellitus?

A

Necrobiosis lipoidica
Neuropathic ulcers
Skin infections
Xerosis
Acanthus nigricans
Xanthom and xanthelesma
Granuloma annulare
Terry’s nails

80
Q

Cutaneous manifestations of hyperlipidemia?

A

Eruptive xanthoma

81
Q

Cutaneous manifestation of Graves’ disease?

A

Pretibial myxoedema

82
Q

Hyperpigmentation is a cutaneous manifestation of what endocrinological disorder?

A

Addison’s disease

83
Q

In what endocrine disorders is acne a possible cutaneous disorder?

A

Acromegaly, Cushing’s, PCOS

84
Q

What are two cutaneous manifestations of acromegaly?

A

Acne
Cutis vertices gyrata

85
Q

What are cutaneous manifestations of HIV seroconversion?

A

Morbilliform rash, erythema multiform-like, urticaria, ulceration (oral/genital)

86
Q

features of hidradenitis suppuritiva?

A

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
Q

Describe the cutaneous features of pyoderma gangrenosum

A

Pustules on erythematous base- ulcerates and extends with a necrotic undermined border

Painful

88
Q

What diseases is pyoderma gangrenous associated with?

A

Inflammatory bowel disease
Leukemia
Seronegative arthritis in 50-70% of cases

89
Q

What cutaneous diseases are associated with inflammatory bowel disease?

A

Pyoderma gangrenosum
Panniculitis
Orofacial granulomatosis
Aphthous ulceration
Associated with psoriasis, pemphigoid

90
Q

Name a cutaneous manifestation of celiac disease

A

Dermatitis herpetiformis

91
Q

Name a malignancy reflecting an internal malignancy

A

Extramammary Paget’s diseases

92
Q

Genetic conditions leading to predisposition to internal cancer and skin lesions?

A

Hereditary leiomyomatosis and renal cell cancer
Peutz-Jeghers syndrome

93
Q

Skin disease associated with malignancy?

A

Pyoderma gangrenosum
Paraneoplastic pemphigus
Erythema gyratum repens
Dermatomyositis

94
Q

non specific skin disease associated with internal malignancy?

A

Pruritis
vasculitis
urticaria

derm 2 (3 decks)

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