The Skin in Connective Tissue Disease Flashcards
(113 cards)
1
Q
What causes Connective Tissue Diseases (CTDs)?
A
Problems with collagen deposition
CT diseases are characterized by inflammatory autoimmune conditions affecting the skin and other connective tissues.
2
Q
What is the pathogenesis of CTDs?
A
Variable and not completely understood
3
Q
Why are CTDs hard to distinguish?
A
Many CTDs share common signs and symptoms, making them hard to distinguish
This can complicate diagnosis.
4
Q
What is the primary method for diagnosing CTDs?
A
Clinical presentation, blood tests, and skin biopsy
5
Q
What further investigations (Ix) can be conducted for CTDs?
A
- Urine tests for hyaline casts, creatinine, protein, and blood
- Blood pressure measurement
- Chest x-ray
- Ultrasound
- CT scans
- MRI scans
- ECG
- Echocardiography
- Nerve and muscle testing
- Ophthalmological examination
- Endoscopy of the gastrointestinal tract
- Kidney biopsy
6
Q
Why is early diagnosis of CTDs important?
A
To reduce the risk of systemic complications
7
Q
What complication can occur if discoid lupus erythematosus is left untreated?
A
Scarring of facial skin
8
Q
What may subacute lupus erythematosus be associated with?
A
Drug-induced factors, especially in middle-aged or elderly individuals
9
Q
How can complications associated with systemic lupus erythematosus be managed?
A
By early intervention
10
Q
What effects can linear morphoea have?
A
- Affect limb growth
- Cause flexural contractions
11
Q
What is a notable association of subgroups of adult dermatomyositis?
A
Associated with malignancy
12
Q
What can overlap between different CTDs result in?
A
Mixed connective tissue disorder
13
Q
What are the characteristics of connective tissue disorders?
A
- Pathology in one or more organ systems
- Autoimmunity with autoantibody production or disordered cell-mediated immunity
- Vascular abnormalities such as Raynaud’s phenomenon
- Occlusive vascular disease and vasculitis
- Arthritis or arthralgia
- Skin disease
14
Q
List the most relevant connective tissue diseases (CTDs)
A
- Morphoea (scleroderma)
- Systemic sclerosis
- Lupus erythematosus (cutaneous and systemic)
- Dermatomyositis (and its subgroups)
- Sjögren’s syndrome
- Rheumatoid arthritis and Still’s disease
- Mixed connective tissue disorder
15
Q
What characterizes mixed connective tissue disorder?
A
- Very high titre of speckled antinuclear antibody (ANA)
- RNP antibodies (U1-RNP)
- Features of SLE, systemic sclerosis, dermatomyositis, and polymyositis
16
Q
What are common symptoms of mixed connective tissue disorder?
A
- Raynaud’s
- Arthritis/arthralgia
- Swollen-tight fingers
- Abnormal oesophageal motility
- Impaired pulmonary diffusing capacity
- Myositis
17
Q
What is the incidence of clinical renal disease in mixed connective tissue disorder?
A
5%
18
Q
What is the general response to treatments for mixed connective tissue disorder?
A
Generally good with corticosteroids, methotrexate, and hydroxychloroquine
19
Q
What is a special consideration regarding the severity of mixed connective tissue disorder?
A
Can be more severe in children
20
Q
What is granuloma faciale?
A
A rare skin disorder characterized by papules, plaques, or nodules, most often occurring on the face
Lesions can also appear on the scalp, trunk, or extremities, known as extrafacial granuloma faciale.
21
Q
What are the characteristics of lesions in granuloma faciale?
A
Defined borders, varying in color from skin-colored, red-brown, blue, or purple
Lesions are often asymptomatic but can be associated with itching.
22
Q
How is cutaneous lupus erythematosus (CLE) classified?
A
- Acute
- Subacute
- Chronic
It may be associated with systemic lupus erythematosus (SLE).
23
Q
Where do lesions of cutaneous lupus erythematosus usually occur?
A
Usually on sun-exposed areas such as the face, ears, scalp, and upper trunk
Lesions are erythematous and scaly.
24
Q
What factors may be associated with the pathogenesis of cutaneous lupus erythematosus?
A
- Genetic susceptibility
- Environmental factors
- Auto-antibodies
Environmental factors include smoking and sun exposure.
25
What is tinea faciei?
A fungal infection affecting the face
## Footnote
Characterized by round or oval erythematous, scaly patches.
26
What is cutaneous sarcoidosis?
A condition that may occur in patients with systemic sarcoidosis or in isolation, characterized by granulomas
## Footnote
Erythema nodosum is the most common non-specific finding.
27
What are the hallmark lesions of cutaneous sarcoidosis?
Granulomas on histology, including lupus pernio, papules, and plaques
## Footnote
Erythema nodosum presents as tender, subcutaneous erythematous nodules.
28
What is lichen planus?
A chronic inflammatory skin condition affecting the skin and mucosal surfaces
## Footnote
Classical presentation includes papules or plaques with overlaying white lines known as Wickham striae.
29
What are the common presentations of lichen planus lesions?
Can be scattered, clustered, linear, annular, or actinic
## Footnote
Most often appears on the wrists, lower back, and ankles.
30
What is dermatomyositis?
An idiopathic inflammatory myopathy characterized by skeletal muscle weakness and skin changes
## Footnote
Characteristic skin changes include heliotrope rash and Gottron sign.
31
List some characteristic skin changes in dermatomyositis.
* Heliotrope rash
* Gottron sign
* Gottron papules
* Shawl sign
* Photosensitivity
* Nail fold changes
* Scalp involvement
32
Fill in the blank: Tinea faciei is characterized by _____ patches.
erythematous, scaly
33
What is acute cutaneous lupus erythematosus (ACLE)?
Typically presents as transient erythematous patches associated with a flare of systemic lupus erythematosus (SLE)
## Footnote
ACLE can manifest as localized or generalized forms.
34
What characterizes localized acute cutaneous lupus erythematosus?
Malar ‘butterfly' rash and erythema and swelling over both cheeks, sparing the nasolabial folds
## Footnote
This rash can last from hours to days.
35
What is generalized acute cutaneous lupus erythematosus characterized by?
Diffuse or papular erythema of the face, upper limbs, and trunk resembling a morbilliform drug eruption or a viral exanthem
## Footnote
The upper limbs spare the knuckles.
36
What is subacute cutaneous lupus erythematosus (SCLE)?
Less commonly associated with SLE and skin changes are more persistent than those of ACLE
## Footnote
SCLE lesions are typically triggered or aggravated by sun exposure.
37
Where do skin lesions typically occur in subacute cutaneous lupus erythematosus?
On the trunk and upper limbs
## Footnote
Lesions present as a psoriasiform papulosquamous rash or annular, polycyclic plaques with central clearing.
38
What happens to skin lesions in subacute cutaneous lupus erythematosus after resolution?
They leave dyspigmentation and telangiectases, but no scarring
## Footnote
This indicates that while the lesions may resolve, some changes in skin pigmentation may persist.
39
What is chronic cutaneous lupus erythematosus (CCLE)?
The most common form of cutaneous lupus erythematosus
## Footnote
CCLE encompasses various types of skin lesions.
40
What is discoid lupus erythematosus (DLE)?
The most common form of chronic cutaneous lupus erythematosus
## Footnote
DLE is particularly prevalent and severe in patients with skin of color.
41
Where are skin lesions of discoid lupus erythematosus (DLE) most commonly located?
* Scalp
* Ears
* Cheeks
* Nose
* Lips
## Footnote
Involvement of the concha is common for ear lesions.
42
What do skin lesions of discoid lupus erythematosus present as?
Destructive scaly plaques with follicular prominence, which can result in scarring alopecia
## Footnote
These lesions heal slowly, leaving behind significant skin changes.
43
What do discoid lupus erythematosus lesions leave after healing?
Post-inflammatory dyspigmentation and scarring
## Footnote
This highlights the long-term effects of DLE on the skin.
44
What type of biopsy is recommended for cutaneous lupus erythematosus?
A skin biopsy for hematoxylin and eosin (H and E) staining
45
Which autoantibodies should be tested in blood for suspected cutaneous lupus?
* antinuclear antibody (ANA)
* extractable nuclear antigen (ENA)
* double-stranded deoxyribonucleic acid antibody (dsDNA)
* complement titers (C3/C4)
46
What percentage of patients with Discoid Lupus Erythematosus (DLE) have a positive antibody titer?
Approximately 50%
47
What tests are included in the additional workup for suspected cutaneous lupus?
* Full blood count (FBC)
* Renal function tests
* Liver function tests (LFT)
* Lipid profile
* HbA1C
* Thyroid function tests
* Erythrocyte sedimentation rate and/or C reactive protein (ESR/CRP)
* Vitamin D
48
What specific antibodies should be tested for antiphospholipid antibodies in suspected cutaneous lupus?
* lupus anticoagulant
* beta-2-glycoprotein
* cardiolipin antibody
49
What type of analysis is recommended for urinary assessment in suspected cutaneous lupus?
Urinalysis +/- urinary protein/creatinine ratio
50
What vital sign measurement is recommended in the additional workup for suspected cutaneous lupus?
Blood pressure
51
What are general measures to manage CLE?
* Lifestyle changes
* Vitamin D supplementation
* Discontinuation of potential causative drugs
* Consider systemic lupus erythematosus
* Optimize medication adherence
* Minimize oral steroid intake
* Assess cardiovascular and infection risk
* Contraception and pregnancy issues.
## Footnote
These measures aim to address both lifestyle and medical factors influencing CLE.
52
What lifestyle changes are recommended for CLE management?
Photoprotection and smoking cessation.
## Footnote
These changes help reduce skin damage and improve overall health.
53
What is a potential cause of subacute CLE that should be addressed?
Drug-induced CLE.
## Footnote
Discontinuing the causative drug may help alleviate symptoms.
54
What topical therapies are considered for CLE?
* Topical corticosteroids
* Topical calcineurin inhibitors
* Intralesional triamcinolone.
## Footnote
These therapies aim to reduce inflammation and manage lesions.
55
What is the first-line systemic therapy for CLE?
* Hydroxychloroquine
* Mepacrine
* Chloroquine
* Systemic corticosteroids.
## Footnote
Hydroxychloroquine is typically used at 200–400 mg daily.
56
What is the typical maintenance dose of hydroxychloroquine for CLE?
Less than 5 mg/kg daily.
## Footnote
This dosage helps maintain remission while minimizing side effects.
57
What are the options for second-line systemic therapy in CLE?
* Methotrexate
* Mycophenolate mofetil
* Dapsone
* Acitretin.
## Footnote
These therapies are considered when first-line treatments are ineffective.
58
What is a typical starting dose for mycophenolate mofetil in CLE?
0.5 g twice daily.
## Footnote
Dosage may be escalated based on patient response.
59
What are some third-line systemic therapy options for CLE?
Clofazimine, biologics (belimumab, rituximab), IVIg, thalidomide, lenalidomide.
## Footnote
These are considered for refractory cases or severe manifestations.
60
True or False: Mepacrine can be used alone or in combination with hydroxychloroquine.
True.
## Footnote
Mepacrine is an alternative option in systemic therapy.
61
Fill in the blank: The typical dose of dapsone in CLE starts at _______.
50 mg daily.
## Footnote
The dose can be increased based on effectiveness and tolerance.
62
What is the typical starting dose of acitretin for CLE resistant to other treatments?
25–50 mg daily.
## Footnote
This is used particularly in cases of hyperkeratotic DLE.
63
What should be considered in terms of medication adherence for CLE management?
Optimize medication adherence and minimize oral steroid intake.
## Footnote
This approach helps prevent flares and manage disease effectively.
64
What risks should be assessed in patients with CLE?
Cardiovascular, thrombosis, and infection risk.
## Footnote
These risks are important for comprehensive patient management.
65
What is the risk of developing SLE in localized cases of DLE?
5%
## Footnote
Localized cases of DLE refer to limited skin involvement.
66
What is the risk of developing SLE in generalized cases of DLE?
15%
## Footnote
Generalized cases refer to widespread skin involvement.
67
Which demographic groups of women with DLE are more likely to develop SLE?
Women of African or Asian ancestry
## Footnote
This highlights the influence of genetic and ethnic factors on disease progression.
68
Name two specific clinical features that increase the likelihood of a woman with DLE developing SLE.
Nail fold lesions and periungual telangiectasia
## Footnote
These features indicate more severe disease manifestation.
69
What are some systemic symptoms associated with a higher risk of developing SLE from DLE?
Fever and malaise
## Footnote
Systemic symptoms suggest a more severe systemic involvement.
70
True or False: The risk of progression to SLE is the same for all women with DLE.
False
## Footnote
Risk varies based on disease severity and other clinical features.
71
What is systemic lupus erythematosus (SLE)?
An autoimmune disease causing widespread inflammation and tissue damage in affected organs
## Footnote
SLE can affect joints, skin, brain, lungs, kidneys, and blood vessels.
72
What percentage of SLE patients have cutaneous findings?
Approximately 80%
## Footnote
Cutaneous findings in SLE can include various skin manifestations.
73
What is acute cutaneous lupus erythematosus (ACLE)?
Characterized by a malar rash affecting the central face that usually resolves without scarring.
74
List some associated features of SLE beyond the malar rash.
* Blistering
* Mucosal erosions and ulcerations
* Photosensitivity
* Diffuse hair loss
75
What is dermatomyositis?
An idiopathic inflammatory myopathy characterized by skeletal muscle weakness and skin changes.
76
Which muscle groups are typically affected in dermatomyositis?
Proximal muscle groups such as the triceps and quadriceps.
77
What are some characteristic skin changes in dermatomyositis?
* Heliotrope rash
* Gottron sign
* Gottron papules
* Shawl sign
* Photosensitivity
* Nail fold changes
* Scalp involvement
78
What is psoriasis?
A chronic inflammatory skin condition classified into various subtypes, with plaque psoriasis being the most common.
79
How does psoriasis typically present?
As symmetrically distributed, erythematous, scaly plaques with well-defined edges.
80
What is a photodrug eruption?
Drug-induced photosensitivity causing a phototoxic or photoallergic reaction resulting in sunburn or dermatitis on sun-exposed skin.
81
When do phototoxic skin reactions occur after exposure?
Minutes to hours after exposure to the causative agent and the sun.
82
What are the characteristics of phototoxic skin reactions?
Affected skin appears erythematous and edematous, with possible vesicles or blisters in severe reactions.
83
When do photoallergic reactions typically develop?
24–72 hours after exposure to the causative agent and the sun.
84
What do affected areas in photoallergic reactions look like?
Eczematous and may spread to areas not exposed to sunlight.
85
What is cutaneous T-cell lymphoma (CTCL)?
A type of primary cutaneous lymphoma and a form of non-Hodgkin lymphoma.
86
What is the most common form of cutaneous T-cell lymphoma?
Mycosis fungoides.
87
How may mycosis fungoides present initially?
As a chronic itch before clinical features develop.
88
What is the progression of lesions in mycosis fungoides?
Initially scaly patches on sun-exposed areas, developing into well-defined, itchy plaques, and then tumors.
89
What should be done for all patients with suspected dermatomyositis?
Refer to secondary care
## Footnote
This includes referrals to dermatology for cutaneous involvement, rheumatology for muscle involvement, and other specialties based on clinical findings.
90
When should a patient with dermatomyositis be referred to dermatology?
If there is cutaneous involvement
91
When should a patient with dermatomyositis be referred to rheumatology?
If there is evidence of muscle involvement
92
What are the general management strategies for dermatomyositis?
* Photoprotection and sun-avoidance
* Bedrest during acute flare
* Physical therapy and activity
93
What is a treatment option for cutaneous lesions in dermatomyositis?
* Potent topical corticosteroids
* Topical tacrolimus
* Hydroxychloroquine 200–400 mg daily orally
* Chloroquine 250 mg daily orally
* Quinacrine 100 mg daily orally
* Low-dose methotrexate 5–15 mg weekly
94
What is the first-line therapy for systemic disease in dermatomyositis?
* Prednisone 1 mg/kg daily orally, tapered to 50% over 6 months and to 0 over 2–3 years
* Start calcium and vitamin D supplementation
* Consider a proton pump inhibitor for gastric protection
* Assess the risk of osteoporosis using the FRAX tool
95
What is the second-line therapy for systemic disease in dermatomyositis?
* Methotrexate 5–25 mg orally or subcutaneously
* Azathioprine 2–3 mg/kg daily orally
96
True or False: Patients with dermatomyositis do not require sun protection.
False
97
Fill in the blank: The maintenance dose of hydroxychloroquine in dermatomyositis is < _____ mg/kg daily.
< 5 mg/kg
98
What should be assessed when a patient is on long-term oral steroids for dermatomyositis?
The risk of osteoporosis using the FRAX tool
99
What is small vessel vasculitis?
The most common form of vasculitis, affecting the arterioles and venules
## Footnote
It can be idiopathic or secondary to infections or drugs (hypersensitivity vasculitis), or disease.
100
What are the clinical features of hypersensitivity vasculitis?
* Prominent involvement of the lower legs with fewer lesions on proximal sites
* Palpable purpura, petechiae, ecchymoses, non-purpuric erythematous or urticaria-like wheals, macules, and papules
* Hemorrhagic bullae, necrosis, and superficial ulceration
* Local pruritus, burning, pain, and swelling
* Systemic symptoms can include fever, joint aches, lymphadenopathy, and gastrointestinal problems
101
What does medium vessel vasculitis affect?
Medium-sized arteries in the dermis and subcutaneous tissue
## Footnote
Examples include polyarteritis nodosa and Kawasaki disease.
102
What are the cutaneous signs of polyarteritis nodosa?
* Painful subcutaneous nodules
* Livedo reticularis
* Leg ulcers
* Post-inflammatory hyperpigmentation
103
What are the cutaneous signs of Kawasaki disease?
* Polymorphous exanthem typically on the trunk and proximal limbs
* Edema and erythema of the palms and soles of the feet
* Periungual desquamation
104
Is cutaneous involvement common in large vessel vasculitis?
No, it is uncommon as the vessels affected do not directly supply the skin
## Footnote
Examples include giant cell arteritis.
105
What are the symptoms of giant cell arteritis?
* Severe headache
* Fever
* Malaise
* Visual symptoms
* Can include cutaneous signs such as erythema, purpura, induration, and bullae
106
What are some cutaneous signs associated with giant cell arteritis?
* Tender nodules
* Ulcers
* Necrosis
* Periorbital ecchymosis
* Facial edema
* Hair loss
107
What is the first-line treatment of cutaneous small vessel vasculitis without evidence of systemic involvement?
Supportive care, evaluate for underlying cause and treat trigger, rest, compression stockings, leg elevation, paracetamol or NSAIDs for pain, bland emollient, potent topical steroid, dress any ulcers, treat secondary bacterial infection
## Footnote
These interventions aim to alleviate symptoms and address the underlying cause of vasculitis.
108
What should be done if cutaneous vasculitis is severe, ulcerated, chronic, or recurrent?
Systemic therapy may be required
## Footnote
This indicates a need for a more aggressive treatment approach.
109
List the treatment options for severe or chronic cutaneous vasculitis.
* Oral prednisolone tapered over 4–6 weeks
* Colchicine (0.6 mg 2–3 times daily)
* Dapsone (50–200 mg daily)
## Footnote
These treatments aim to reduce inflammation and control symptoms.
110
What might be required if vasculitis is refractory?
Immunosuppressive therapies such as methotrexate or azathioprine
## Footnote
These medications help to suppress the immune system and reduce inflammation.
111
What is the role of paracetamol or NSAIDs in the treatment of cutaneous small vessel vasculitis?
For pain relief
## Footnote
These medications help manage discomfort associated with the condition.
112
Fill in the blank: If any ulcers are present in cutaneous vasculitis, they should be _______.
dressed
## Footnote
Proper dressing is essential to prevent infection and promote healing.
113
What is a recommended non-pharmacological treatment for cutaneous vasculitis?
Leg elevation
## Footnote
Elevating the legs can help reduce swelling and discomfort.
