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Dermatology Diploma > Connective Tissue Disease > Flashcards

Connective Tissue Disease Flashcards

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1
Q

In SLE, who are more commonly affected, men or women?

A

Women (8:1)

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2
Q

What is the peak age of onset in SLE?

A

Mid-thirties

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3
Q

What drugs can cause SLE?

A

Hydrazine and Isoniazid

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4
Q

What is the Mnemonic for remembering how SLE manifests?

A

SOAP BRAIN MD

  • Arthritis
  • ANA Positive
  • DS DNA Positive
  • Seizures
  • Pleuritis Percarditis
  • Malar Rash
  • Discoid Rash
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5
Q

If a patient is photosensitive, what should you always do?

A

Test for ANA to exclude lupus

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6
Q

What are some cutaneous signs of lupus?

A
  • Butterfly rash - photosensitivity over cheeks and nose.
  • Urticarial lesions that are not itchy that last more than 24 hours.
  • Vasculitis - if so, always screen for SLE.
  • Nail fold telangectasia and splinter haemorrhages.
  • Non-scarring hair loss - 50% of cases.
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7
Q

What investigations should be done if SLE is suspected?

A
  • Urine - test for blood and protein
  • FBC - thrombocytopenia
  • ESR - raised in active disease
  • U&Es - renal involvement
  • LFTs - hepatitis
  • Lupus serology - ANA, dsDNA & ENA
  • Complement - low C3 or C4 suggests active disease.
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8
Q

With serological markers, what is quite indicative of Lupus?

A

Both Positive dsDNA and positive ANA.

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9
Q

What is the diagnostic criteria of SLE?

A

The presence of any 4 of the following:

  • Malar rash
  • Discoid rash
  • Abnormal photosensitivity
  • Oral ulceration
  • Non-erosive arthritis
  • Serositis (pleurisy or pericarditis)
  • Renal disease (proteinuria or renal casts)
  • Neurological disease (epilepsy or psychosis)
  • Haematological disorder (anaemia, leucopenia, lymphopenia or thrombocytopenia)
  • Abnormal serology; ANA, dsDNA
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10
Q

What is the treatment for SLE?

A
  • Immunosupression is the main treatment - this depends on the overall degree of involvement.
  • Oral steroids

These all usually are initiated and monitored by a specialist.

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11
Q

What are the 2 main types of lupus?

A
  • Subacute Cutaneous Lupus
  • Discoid Lupus
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12
Q

What % of patients with either form of cutaneous lupus go on to get systemic symptoms?

A

5%

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13
Q

How does subacute cutaneous lupus present?

A
  • Rash on sunexposed areas.
  • Often annular
  • Some scaling
  • Lasts weeks or months.
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14
Q

What does the rash of discoid lupus look like?

What is the cardinal sign?

A

it is red and scaly.

The cardinal sign is atrophy and scarring.

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15
Q

Can lupus cause scarring?

A

yes

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16
Q

What are the investigations for cutaneous lupus?

A
  • Biopsy - remember to look for Immunofluorescence
    • On IMF - they will see the LUPUS BAND, IgG and C3 in the dermoepidermal junction.
  • Autoantibodies
    • Subacute cutaneous lupus - Usually ENA positive.
    • Discoid lupus - negative for autoantibodies
    • Screen with ANA, dsDNA & Complement. If positive, these patients are more likely to develop Systemic Lupus.
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17
Q

What are some complications of cutaneous lupus?

A
  • Discoid lupus - can progress to SCC.
  • SCLE - if Ro antibody positive - this antibody can cross the placenta causing neonatal heart block and SCLE in the neonate.
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18
Q

What is the treatment of cutaneous lupus?

A
  • Avoid UV exposure
  • Oral prednisolone (10-40mg OD) - short term
  • Hydroxycholorquine Oral - 200-400mg daily over summer months.
    • This with sunblock helps to reduce lesions in 80% of patients.
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19
Q

What are the most common drugs to cause lupus?

A
  • Hydralazine
  • Procainamide
  • Quinidine
  • Isoniazid
  • Diltiazem
  • Sulphasalazine
  • Minocycline
20
Q

How long does drug induced lupus last when the drug is stopped?

A

The symptoms usually clear within weeks.

21
Q

What are the symptoms of drug induced lupus?

A
  • Muscle and joint pain
  • Swelling
  • Flu-like symptoms such as fatigue and fever
  • Serositis
  • Skin rash
  • Raynaud’s phenomenon
  • Positive ANA test results
22
Q

What antibodies are present in drug induced lupus?

A
  • Positive ANA
  • Negative Anti-ds DNA
  • Anti-histone antibodies. (In 75% but not specific)
23
Q

What is this?

A

Neonatal Lupus

24
Q

What is this?

A

Lupus Profundus

  • (Lupus that affects the deep paniniculus)
  • Usually affects the face.
25
Q

What is this?

A

Lupus Tumidus

  • Cherry pink dermal inflammatory plaques and patches.
  • Stimulated by sunshine.
  • Disappears in the winter.
26
Q

In dermatomyositis, there are two peak ages. What are they?

A
  1. Childhood type - before the age of 10 years - benign.
  2. Adult (age 40-60 years) - associated with paraneoplasms.
27
Q

If someone developed dermatomyositis in adulthood,

what else should you do?

A

Seek an underlying malignancy

28
Q

What rash is seen around the eyes/on the face in dermatomyositis?

A

Heliotropic rash

Purplish rash around the eyes, forehead, temples and cheeks.

Oedema can also occur around the eyes and chest.

29
Q

What is this in dermatomyositis?

A

Gottron’s Papules

A purplish-red rash on the dorsum of the hands.

30
Q

What is dermatomyositis sensitive to?

A

The Sun

The rash gets worse with the sun.

31
Q

What is another feature of dermatomyositis apart from the cutaneous symptoms?

A

Musle Weakness

  • Usually proximal muscles
  • If severe, patient can be laryngeal weakness and difficulty breathing.
32
Q

What investigations should be done in Dermatomyositis?

A
  • Blood tests
    • Autoantibodies
      • Do DsDNA and Lupus Anticoagulant (To Exclude Lupus
      • ANA - Positive in 80%
      • Anti-Ro & Anti-Jo
    • FBC, U&Es and LFTs
    • CRP & CK should be raised.
  • Biopsy with H&E - is usually unremarkable.
  • EMG - to confirm myopathy.
  • Muscle Biopsy
  • MRI - may show proximal muscle oedema.
33
Q

What are 2 important associated conditions in dermatomyositis?

A
  1. Malignancy in 50% of adult patients with dermatomyositis
    • Breast and Ovarian in women.
    • Prostatic, Lung and Colon Cancer in Men.
  2. Systemic Lupus or Mixed Connective Tissue Disease
34
Q

What is the treatment for dermatomyositis?

A
  • Oral Prednisolone and then switch to:
  • Methotrexate, Azathioprine or cyclosporin for maintenance.
35
Q

Once you treat an underlying malignancy in deramtomyositis, what happens?

A

The symptoms of dermatomyositis settle

36
Q

What is the treatment for cutaneous disease in Dermatomyositis?

A

200-400mg of hydroxychloroquine daily.

37
Q

Scleroderma can be a spectrum of conditions. How do you break it down?

A

Scleroderma

  • Vasculopathy - Raynaud’s phenomenon
  • Systemic
    • Limited systemic sclerosis
    • Limited Cutaneous Systemic Sclerosis
  • Localised
    • Morphoea - localised and generalised
    • Scleroderma
38
Q

What is this?

Tell me a bit about it?

A

Morphoea

  • Localised plaques of collagen
  • It is due to a build up of collagen by fibroblasts underneath the skin.
  • Females > Males
  • Any age
  • Woody and atrophic feeling to the skin.
39
Q

What is this?

A

Linear Morphoea

  • More common in childhood
  • Usually as indurated lesions in the extremeties.
  • Treat aggresively otherwise it spreads to bones, muscle and soft tissue.
40
Q

What is this?

A

En Coup De Sabre Morphoea

41
Q

What is the treatment for Morphoea?

A
  • Initially: Superpotent topical steroids
  • Narrow band UVB if extensive
  • Linear Moprhoea - refer to consultant dermatologist.
    • They may beed immunosuppresive therapy - methotrexate etc.
  • Consider Borrelia Burgdorferi serology (Lyme’s disease) as Morphoea can occur after this.
42
Q

What is CREST Syndrome?

A

It is a limited form of systemic sclerosis involving:

  • Calcinosis
  • Raynaudsd
  • Eosophageal Dysmotility
  • Sclerodactyly
  • Telangiectasia
43
Q

In CREST Syndrome, what is often the first sign to appear?

A

Raynauds Syndrome

(Often by Years)

44
Q

What autoantibody is positive in CREST?

A

Anticentromere antibody

45
Q

Is linear morphoea symmetrical or unliateral?

A

Unilateral

46
Q

What can happen in linear morphoea if untreated?

A

It can lead to limb contractures.

Dermatology Diploma (39 decks)

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