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Yr 3 - Core Conditions > Dermatology - Autoimmune Disorders > Flashcards

Dermatology - Autoimmune Disorders Flashcards

1
Q

What is vitiligo?

A

An autoimmune dermatological condition in which there is loss of melanocytes in the epidermis, resulting in a loss of pigmentation.

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2
Q

Give some autoimmune diseases associated with vitiligo

A
  • Addison’s disease
  • Thyroid disease
  • T1 diabetes
  • Polyendocrine genetic conditions
  • Rheumatoid arthritis
  • Myasthenia gravis
  • Pernicious anaemia
  • Autoimmune hepatitis
  • Alopecia areata
  • Psoriasis
  • Lichen planus
  • Lichen noticeable
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3
Q

Describe the lesions in vitiligo

A
  • Well-demarcated
  • Smooth, flat patches of depigmentation
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4
Q

The lesions in vitiligo may be segmental or non-segmental. What is the different?

A

Segmental:

  • Unilateral, following one or more dermatomes
  • Tends to affect children
  • Rapid onset
  • Not accompanied by other autoimmune disease

Non-segmental:

  • Localised or generalised
  • Tends to affect adults
  • Is progressive with noticeable ‘flares’
  • More often associated with other autoimmune disease
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5
Q

Does segmental vitiligo tend to be associated with children or adults?

A

Children

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6
Q

What is the Koebner phenomenon?

A

The appearance of new skin lesions of a pre-existing dermatosis on areas of cutaneous injury in otherwise healthy skin.

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7
Q

Location of lesions in vitiligo?

A

Common in areas of increased friction (Koebner phenomenon) i.e. fingers, beneath watch strap, genitals

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8
Q

How does body hair appear in areas of vitiligo?

A

White

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9
Q

Management of vitiligo?

A
  • Strongly advise wearing sunscreen
  • Topical steroid cream may induce re-pigmentation
  • UVB or PUVA treatment in those with widespread disease and psychological impact
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10
Q

What is alopecia areata?

A

Describes loss of hair from areas where hair normally grows.

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11
Q

What are the 2 types of alopecia?

A
  1. Scarring
  2. Non-scarring
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12
Q

What happens in scarring alopecia?

A
  • Hair follicles destroyed
  • Skin becomes scarred
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13
Q

Is scarring alopecia reversible?

A

No

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14
Q

What happens in non-scarring alopecia?

A
  • Hair follicles NOT destroyed
  • Skin does NOT scar
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15
Q

Is scarring or non-scarring alopecia more common?

A

Non scarring

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16
Q

Is non-scarring alopecia reversible?

A

Yes

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17
Q

Is alopecia areata a type of scarring or non-scarring alopecia?

A

Non-scarring

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18
Q

Pathophysiology of alopecia areata?

A

Autoimmune

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19
Q

Presentation of alopecia areata?

A
  • Well-defined patches of hair loss
  • Surrounding hairs are narrower near base – ‘exclamation mark’ hairs
  • Usually reverses spontaneously after a few months
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20
Q

What are exclamation mark hairs?

A

Exclamation point hairs are short, broken hairs that can be extracted with minimal traction and where the proximal end of the hair is narrower than the distal end.

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21
Q

Management of alopecia areata?

A
  • No reliable cure
  • Topical steroids
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22
Q

What is SLE?

A

Systemic lupus erythematosus (SLE) is an autoimmune disease that causes inflammation in connective tissues (e.g. cartilage, lining of blood vessels).

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23
Q

Pathophysiology of SLE?

A

Immune complex deposition

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24
Q

What are the 3 different forms of cutaneous lupus?

A
  1. Acute cutaneous LE
  2. Subacute cutaneous LE
  3. Chronic cutaneous LE
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25
Q

Who does acute cutaneous LE affect?

A

People with active disease

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26
Q

What is the characteristic cutaneous feature of acute cutaneous LE?

A
  • Malar rash (butterfly rash) → erythema of the malar eminences and across the bridge of the nose, classically the nasolabial folds are spared
  • Photosensitivity
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27
Q

Define malar

A

Involving the cheeks

28
Q

Describe the presentation of subacute cutaneous LE

A

Results in widespread, psoriasis-like papulosquamous rash or annular plaques with clear centre

29
Q

What is the commonest type of cutaneous lupus?

A

Chronic cutaneous LE

30
Q

Who does chronic cutaneous LE affect?

A

Affects people without active disease

31
Q

Describe the presentation of chronic cutaneous LE

A
  • Discoid, erythematous scaly plaques
  • Scarring and atrophy occur later
32
Q

What is dermatomyositis?

A

A rare condition that consists of skin signs and myositis.

33
Q

What is myositis?

A

Myositis is the name for a group of rare conditions that can cause muscles to become weak, tired and painful. The word myositis simply means inflammation in muscles.

34
Q

Describe the muscle presentation of dermatomyositis

A
  • Proximal muscle weakness and myalgia
  • Difficulty rising from a chair, climbing stairs, lifting objects and raising arms (e.g. to comb hair)
35
Q

Are the proximal or distal muscles affected in dermatomyositis?

A

Proximal

36
Q

Describe the skin signs in detmatomyositis

A
  • Helicotrope rash
  • Gottron’s papules
  • Shawl rash
  • Nailfold erythema
37
Q

What is a helicotrope rash?

A

A heliotrope rash is a reddish purple rash on or around the eyelids.

38
Q

What is a shawl rash?

A

erythema across the upper back and shoulders

39
Q

What are Gottron’s papules?

A

red papules on dorsal aspect of finger joints

40
Q

What is the diagnostic investigation in dermatomyositis?

A

A muscle biopsy

41
Q

What may an autoantibody screen show in dermatomyositis?

A

May show anti-Mi2 and anti-Jo1 antibodies

42
Q

What bloods may be useful in dermatomyositis?

A

Plasma muscles enzymes (CK, ALT, AST, LDH)

43
Q

What is the 1st line management of dermatomyositis?

A

Oral corticosteroids

44
Q

What should you always screen for in dermatomyositis?

A

Screen for malignancy!!! Dermatomyositis can be a para-neoplastic phenomenon

45
Q

What is vasculitis?

A

A group of autoimmune disorders in which there are inflamed blood vessels in the skin

46
Q

How are vasculitis classified?

A

Categorised based on whether they affect small, medium or large vessels.

47
Q

What types of vasculitis affect small vessels?

A
  • Henoch-Schonlein purpura
  • Eosinophilic Granulomatous with Polyangiitis (Churg-Strauss syndrome)
  • Microscopic polyangiitis
  • Granulomatosis with polyangiitis (Wegener’s granulomatosis)
48
Q

What types of vasculitis affect medium vessels?

A
  • Polyarteritis nodosa
  • Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
  • Kawasaki disease
49
Q

What types of vasculitis affect large vessels?

A
  • Giant cell arteritis
  • Takayasu’s arteritis
50
Q

Give some general clinical features that apply to most types of vasculitis

A
  • Purpura
  • Joint muscle & pain
  • Peripheral neuropathy
  • Renal impairment
  • GI disturbance (diarrhoea, abdo pain, bleeding)
  • Anterior uveitis and scleritis
  • Hypertension
  • Systemic manifestations - fever, fatigue, weight loss, anaemia
51
Q

What is the classic dermatological feature of vasculitis

A

Purpura - purple-coloured non-blanching spots caused by blood leaking into the skin

52
Q

Which autoantibody indicates vasculitis?

A

ANCA (anti-neutrophil cystoplasmic antibodies)

53
Q

What are the 2 types of ANCA? What does each indicate?

A
  • p-ANCA (also called anti-MPO) → microscopic polyangiitis, Churg-Strauss syndrome
  • c-ANCA (also called anti-PR3) → Wegener’s granulomatosis
54
Q

Pharmacological management of vasculitis?

A

Involves a combination of steroids and immunosuppressants.

  • Steroids:
    • Oral (prednisolone)
    • IV (i.e. hydrocortisone)
    • Nasal sprays for nasal symptoms
    • Inhaled for lung involvement (e.g. Churg-Strauss syndrome)
  • Immunosuppressants:
    • Cyclophosphamide
    • Methotrexate
    • Azathioprine
    • Rituximab and other monoclonal Abs
55
Q

What is Henoch-Schonlein Purpura?

A

Small vessel vasculitis

56
Q

What is the most common vasculitis seen in children aged 3-15?

A

Henoch-Schonlein Purpura

57
Q

Pathophysiology of Henoch-Schonlein Purpura?

A

Small vessel leukocytoclastic vasculitis involving the deposition of IgA immune complexes in affected organs

58
Q

What is Henoch-Schonlein Purpura thought to be triggered by?

A

A viral URTI

59
Q

What tetrad of symptoms does Henoch-Schönlein purpura (HSP) usually present with?

A
  1. Purpuric rash (over extensor surfaces of lower limb)
  2. Arthralgia/arthritis
  3. Abdominal pain
  4. Renal disease (IgA nephritis)
60
Q

Investigations for Henoch-Schonlein Purpura?

A
  • Urinalysis → presence of blood/protein (renal involvement)
  • BP (can be elevated in nephritic syndrome)
61
Q

Management of HSP?

A
  • Most cases resolve spontaneously and treatment not required
  • Symptomatic treatment
  • Monitor for signs of renal disease
  • Renal involvement → treat with corticosteroids
62
Q

What is Granulomatosis with Polyangiitis (Wegener’s Granulomatosis)?

A

Small vessel vasculitis that affects the respiratory tract and kidneys.

63
Q

Granulomatosis with Polyangiitis commonly affects the nose. How does it present?

A
  • Nose bleeds (epistaxis) and crust nasal secretions
  • A classic sign in exams is the saddle shaped nose due to a perforated nasal septum which causes a dip halfway down the nose
64
Q

What is kawasaki disease?

A

Kawasaki disease is an acute systemic vasculitis that affects young children.

65
Q

Classic symptom of Kawasaki disease?

A

Strawberry tongue

Yr 3 - Core Conditions (33 decks)

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