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Dermatology OAU > CONNECTIVE TISSUE DISORDERS > Flashcards

CONNECTIVE TISSUE DISORDERS Flashcards

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Dermatology Board Prep flashcards
1
Q

Mention 3 functions of connective tissues?

A

They give metabolic support to cells serving as the medium for diffusion of nutrients and waste products.
It gives the body’s tissues and organs strength, form and flexibility.
They also aid in the special functions of certain tissues; like the ability to move in the joints.

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2
Q

Connective tissue is made up mainly of what?

A

Ground substance

Fibres

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3
Q

Connective tissue is made up mainly of what?

A

Ground substance

Fibres

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4
Q

What are connective tissue disorders?

A

Connective tissue d/o comprise of a heterogeneous group of autoimmune responses that target of affect collagen or ground substance.

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5
Q

Mention the 2 types of connective tissue disorders?

A

Heritable CTD

Auto-immune CTD

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6
Q

Outline 5 examples of heritable CTD?

A

MARFAN SYNDROME

OSTEOGENESIS IMPERFECTA

STICKLER SYNDROME

EHLERS-DANLOS SYNDROME

PSEUDOXANTHOMA ELASTICUM

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7
Q

Itemize 4 autoimmune CTD with skin manifestations?

A

LUPUS ERYTHEMATOSUS
SCLERODERMA
DERMATOMYOSITIS
MIXED CONNECTIVE TISSUE DISEASE.

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8
Q

Mention 5 other autoimmune conditions you know?

A 
Rheumatoid Arthritis 
Behcet’s  Syndrome
Panniculitis
Polyarteritis Nodosa
Relapsing Polychronditis
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9
Q

cutaneous disease never occurs without systemic involvement in lupus Erythrmatosus (T/F)?

A

False!

cutaneous disease may occur with or without systemic involvement.

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10
Q

LE may manifest as a systemic disease or in purely cutaneous forms (T/F)?

A

True!

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11
Q

chronic skin lesions often equate with purely cutaneous disease in lupus Erythrmatosus (T/F)?

A

False!

chronic skin lesions DO NOT equate with purely cutaneous disease.

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12
Q

Some patients with LE may show features of more than one type (T/F) ?

A

True!

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13
Q

Classify the cutaneous manifestations seen in LE?

A

CHRONIC cutaneous LE
SUBACUTE cutaneous LE
ACUTE cutaneous LE

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14
Q

Itemize 6 subtypes of chronic cutaneous LE?

A 
A. Discoid Lupus Erythematosus
B. Verrucous (hypertrophic) LE 
C. Lupus erythematosus–lichen planus overlap
D. Chilblain Lupus Erythematosus
E. Tumid lupus
F. Lupus panniculitis (LE profundus)
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15
Q

Neonatal LE and Complement deficiency syndromes exhibit morphology similar to acute cutaneous LE (T/F) ?

A

False

It’s sub-acute cutaneous LE!

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16
Q

Outline 3 examples of sub-acute cutaneous LE?

A 
A. Papulosquamous
B. Annular
C. Syndromes commonly exhibiting similar morphology
*Neonatal LE
*Complement deficiency syndromes
*Drug induced.
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17
Q

Drug induced LE is a subtype of chronic cutaneous LE (T/F)?

A

False!

It’s sub-acute cutaneous LE!

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18
Q

Acute cutaneous LE is generally associated with Systemic Lupus Erythematosus (T/F)?

A

True!

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19
Q

localized or generalized erythema or bullae may be seen in acute cutaneous LE? (T/F)?

A

True!

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20
Q

What is the most common type of lupus erythematosus seen by dermatologists ?

A

Chronic DLE!

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21
Q

DLE is a very malignant skin condition (T/F)?

A

False!

This is a benign disorder of the skin

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22
Q

DLE It is an acute benign disorder of the skin (T/F)?

A

False

It’s a chronic disorder

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23
Q

DLE most frequently involves which body area?

A

The face!

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24
Q

How would you describe a DLE lesion?

A

DLE is characterized by well-defined erythematous scaly patches of variable size

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25
Q

DLE lesions rarely heal with scarring (T/F)?

A

False

It heals with atrophy, scarring, and pigmentary changes

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26
Q

What sex is mostly affected by DLE?

A

FEMALES!

greater than Male 2:1

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27
Q

What is the peak age of onset of DLE?

A

in the 4th decade of life

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28
Q

50% of DLE cases will go on to develop systemic lupus erythematosus (T/F)?

A

False!

Only 5% of cases will go on to develop systemic lupus erythematosus

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29
Q

Genetic and environmental factors have been implicated in the etiology of DLE (T/F)?

A

Very True!

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30
Q

What genes have been implicated in the etiology of DLE?

A 
Genetic:
HLA B7
HLA B8.       
HLACW7.   
HLADR2
HLADR3.  
HLADQw1

*A family history was found in some cases

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31
Q

Viral infections may have a role in the etiology of DLE (T/F)? What virus?

A

True!

Reovirus

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32
Q

Emotional factors, worry, anxiety may lead to exacerbation of DLE lesions (T/F)?

A

Very true!

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33
Q

Mention 10 environmental factors that can precipitate the onset of DLE lesions?

A 
Trauma
Mental stress
Pregnancy 
Sunburn
Infection
Exposure to cold
Laser therapy
psoralen and UVA (PUVA) therapy
Xrays
Diathermy 
Chemical burns
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34
Q

Laser therapy and psoralen and UVA (PUVA) therapy may be useful in the treatment of DLE lesions (T/F)?

A

Very false!

They precipitate DLE lesions

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35
Q

Hydropic degeneration of the basal cell layer of the epidermis and follicular epithelium is a characteristic finding seen in drug induced LE (T/F)?

A

False

It’s seen in DLE!

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36
Q

What characteristic finding is seen in the Epidermis of ptx with DLE?

A

effacement of the rete ridge pattern or irregular acanthosis.

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37
Q

Mention 3 Degenerative changes in the connective tissue seen in DLE? And where are they mostly markedly seen?

A

hyalinization
oedema
Fibrinoid change

most marked immediately below the epidermis.

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38
Q

“A patchy dermal lymphocytic infiltrate with a few plasma cells and histiocytes, particularly around the appendages, which may be atrophic” is the correct microscopic finding seen in what condition?

A

DLE!

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39
Q

Mention 3 microscopic skin changes seen in DLE?

A

Thinning and pallor of the epidermis with relative hyperkeratosis and plugging of the follicular orifices

Thickening of the basement membrane of the epidermis and sometimes of small vessels

Premature elastotic degeneration of collagen in light-exposed areas.

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40
Q

Parakeratosis is a characteristic finding seen in DLE (T/F)?

A

False!

It’s hyperkeratosis

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41
Q

Histological findings in DLE may also be seen in uninvolved skin (T/F)?

A

False

Do not occur in uninvolved skin, unlike majority of cases of SLE

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42
Q

State 2 Immunohistological findings seen in DLE?

A

Presence of immunoglobulins IgG, IgA, IgM and complement at the DEJ, in skin lesions present for 6 weeks or more in approximately 80% of patients.

Homogenous granular or thread pattern.

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43
Q

Homogenous granular or thread pattern is a characteristic immunohistological finding in drug induced LE (T/F)?

A

False

It’s seen in DLE

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44
Q

Outline the anti-cardiolipin antibodies seen in DLE? for how long can they be found within lesions? And in what % of ptx?

A

IgG, IgA, IgM and complement
for 6 weeks or more
in approximately 80% of patients.

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45
Q

DLE lesions usually begins as a Fixed irregular erythematous, violaceous, scaly macules or plaques of similar sizes (T/F)?

A

False

Lesions are usually well-defined and are if variable sizes!

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46
Q

DLE lesions are usually localized below the neck (T/F)?

A

False

ABOVE the neck!

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47
Q

What are the Favoured sites seen in DLE?

A 
Scalp
bridge of nose
malar areas
lower lip
ears(concha and external canal)
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48
Q

Itemize 5 cardinal features of DLE lesions?

A 
Erythema and telangiectasia
Adherent scales
Patulous hair follicles
hyperkeratotic plugging of the follicles
Scarring and atrophy
Post-inflammatory dyschromia (both hyperpigmentation and depigmentation)
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49
Q

langue au chat(cat’s tongue sign) is a characteristic finding seen in what condition? What does it describe?

A

Seen in DLE lesions!
Describes hyperkeratotic plugging of the hair follicles producing carpet tack-like spines on the undersurface of the scale

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50
Q

Scalp involvement in DLE leads to non cecatricial alopecia (T/F)?

A

False

Scalp involvement leads to scarring/cecatricial Alopecia!

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51
Q

ORAL INVOLVEMENT(erythematous patches or ulceration) occurs in 50% of cases (T/F)?

A

False!

25% of cases

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52
Q

Disseminated DLE is most commonly seen in what group of ptx?

A

Women who are usually cigarette smokers

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53
Q

Itching and tenderness are rare findings in DLE and may often be severe (T/F)?

A

False

Itching and tenderness are COMMON and may RARELY be severe.

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54
Q

erythematous patches or ulceration may be seen in ptx with DLE (T/F)?

A

True!

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55
Q

What is a rare complication seen in long-standing lesions of DLE ?

A

aggressive squamous cell carcinoma

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56
Q

Outline 3 Nail changes seen in DLE?

A

subungual hyperkeratosis
red-blue coloring of nail plate
longitudinal striae & crumbling nails

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57
Q

Nail changes may be successfully treated with what drug?

A

Chloroquine!

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58
Q

Calcifications may occur in plaques seen in DLE (T/F)?

A

True!

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59
Q

Follicular plugging is a characteristic finding seen in DLE (T/F)?

A

Very true!

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60
Q

The active stage of DLE lesions is usually associated with severe degree of scaling (T/F)?

A

True!

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61
Q

Hypopigmentation is a feature of post inflammatory DLE (T/F)?

A

True!

And hyperpigmentation too

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62
Q

What are the goals of management of DLE lesions?

A

to improve the patient’s appearance
to control existing lesions and limit scarring
to prevent the development of further lesions.

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63
Q

Blood tests are usually normal in DLE (T/F)?

A

True!

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64
Q

In what % of cases do serum of DLE ptx occasionally contain antinuclear antibodies?

A

in 30% of cases

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65
Q

How would you manage a ptx diagnosed with DLE?

A 
History taking and examination
Investigations 
*Skin biopsy
*Direct immunofluorescence.
*Blood tests
*other baseline investigations e.g PCV, FBC, EUC,  LFTs, ESR, TFTs etcTo R/o systemic involvement
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66
Q

How would you treat DLE?

A

General measures:

  • avoid precipitating factors
  • Sunscreens

TOPICAL STEROIDS

  1. 025% or 2% FLUOCINOLONE cream
  2. 1% BETAMETHASONE 17-valerate cream
  3. 2% FLUOCINOLONE
  4. 05% CLOBETASOL PROPIONATE cream

INTRALESIONAL CORTICOSTEROID INJECTIONS
5-10mg/ml TRIAMCINOLONE ACETONIDE at 6 weekly intervals.

  • IFN-alpha.
  • Painting small lesions with TRICHLOROACETIC ACID
  • CARBONDIOXIDE LASER and both the pulsed-dye and ARGON LASER may be valuable for telangiectasia lupus erythematosus.

ORAL THERAPY

Oral PREDNISOLONE
ANTIMALARIALS(hydroxyquinone, chloroquine, mepacrine)
AURANOFIN
ACITRETIN
ETRETINATE
ISOTRETINOIN
DAPSONE
oral METHOTREXATE 
oral THALIDOMIDE
CLOFAZIMINE(Lamprene)
SULFASALAZINE
  • 0.5mg/kg prednisolone rapidly tapered over 6weeks
  • antimalarials such as hydroxychloroquine 200mg twice daily then reduced to 200mg/day once a response is achieved.
  • isotretinoin 20-80mg/day
  • thalidomide have proved helpful in resistant cases
For long standing lesions
auranofin 6-9mg
Etretinate 1mg/kg/day combined with chloroquine in chronic hyperkeratotic lesions
Dapsone 100mg/day can be used
thalidomide
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67
Q

Sub-acute cutaneous LE is a fairly common condition. (T/F)?

A

False

It’s rare

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68
Q

Patients sub-acute cutaneous LE exhibit mainly systemic disease (T/F)?

A

False

Mainly cutaneous manifestations!

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69
Q

Sub-acute cutaneous LE is often associated with systemic disease (T/F)?

A

True

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70
Q

Sub-acute cutaneous LE has a poor prognosis (T/F)?

A

False

Good prognosis

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71
Q

Sub-acute cutaneous LE is is a dx of unknown etiology (T/F)?

A

True

It’s specific cause is unknown

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72
Q

What is almost a universal finding in sub-acute cutaneous LE?

A

antibodies to R₀/SS-A antigen

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73
Q

What sex is mostly affected by sub-acute cutaneous LE?

A

Most often women

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74
Q

What age group is most commonly affected in sub-acute cutaneous LE?

A

Age is between 15 – 40years

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75
Q

What % of the LE population constitutes sub-acute cutaneous LE?

A

Approximately 10-15%

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76
Q

What environmental factor may explain the pathogenesis of sub-acute cutaneous LE?

A

ultraviolet light exposure (photosensitive lupus)

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77
Q

The most common haplotype in SCLE is what?

A

HLA-DR3 with HLA-B8.

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78
Q

Itemize 5 drugs which have been reported to precipitate or exacerbate SCLE ?

A 
thiazide diuretics
griseofulvin
terbinafine
cinnazarine
calcium channel blockers
etanercept
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79
Q

Telangiectasia or dyspigmentation are characteristic findings seen in chronic DLE and sub-acute cutaneous LE (T/F)?

A

Very true!

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80
Q

SCLE lesions tend to be transient or migratory but may heal with scarring (T/F)?

A

False

Heals without scarring !

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81
Q

SCLE lesions rarely occur in sun-exposed areas (T/F)?

A

False

They occur in sun exposed areas

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82
Q

What are the preferred body areas affected by SCLE?

A 
Above the waist and particularly around the neck
on the trunk
on the outer aspects of the arms
Shoulders
Chest
back
extensor surfaces of the arms
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83
Q

SCLE lesions typically occur below the waist and particularly around the neck (T/F)?

A

False

ABOVE the waist and particularly around the neck

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84
Q

Itemize 3 morphological features of s SCLE lesion

A
  • Lesions are scaly and evolve as annular polycyclic lesions or psoriasiform plaques
  • Scale is thin and easily detached.
  • Lesions vary from red to pink with faint violet tones.
  • Borders may show vesiculation and crusting
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85
Q

Like DLE, facial involvement is common in SCLE (T/F)?

A

False

Facial involvement is uncommon!

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86
Q

Concomitant DLE is a common finding in sub-acute cutaneous LE (T/F)?

A

False

Present in 20% of cases!

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87
Q

Majority of patients with SCLE have arthralgia or arthritis (T/F)?

A

True

¾ of patients have arthralgia or arthritis.

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88
Q

Athralgia and arthritis is a common finding in chronic DLE (T/F)?

A

False

It’s sub-acute cutaneous LE!

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89
Q

Fever, malaise and central nervous system involvement and renal disease are common findings in SCLE (T/F)?

A

False

Renal disease is mild and infrequent.

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90
Q

Occasionally patient develop overt systemic lupus with severe visceral disease in chronic DLE (T/F)?

A

False

It’s sub-acute cutaneous LE!

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91
Q

How can SCLE be differentiated from chronic DLE ?

A
  • Presence of more epidermal atrophy
  • LESS – hyperkeratosis, basement membrane thickening, follicular plugging and inflammatory infiltration.
  • Colloid bodies and epidermal necrosis are present in more than 50% especially in those with R₀/SS-A antibodies.
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92
Q

The epidermis may show minor atrophy and vacuolar changes while the dermal lymphocytic infiltrate is usually sparse correctly describes what?

A

Sub-acute cutaneous LE!

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93
Q

How would you treat SCLE?

A

This condition is controlled by sunscreen in most patients.

Topical treatment is the same as that of discoid lupus erythematosus.

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94
Q

SLE most commonly affects?

A

the skin, joints and vasculature.

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95
Q

SLE is associated with certain immunological abnormalities (T/F)?

A

True!

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96
Q

Has a worldwide distribution (1 in 1000) and is equally found in blacks and white (T/F)?

A

False

appears to be three times more in blacks than whites (black>white – 3:1)

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97
Q

What sex is affected more in SLE?

A

Females!
F>M- 8:1
Occurs in early adult life

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98
Q

The Aetiology of SLE is generally unknown (T/F)?

A

True!

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99
Q

What is the mode of inheritance of SLE?

A

Multifactorial

  • genetic factors
  • environmental factors
  • hormonal factors
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100
Q

Itemize the hereditary factors that increase susceptibility to SLE?

A

hereditary factors

  • complement deficiency
  • certain HLA types —- (HLA-B8, HLADR3, HLA-A1 and HLA–DR2)
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101
Q

What constitutes the hallmark of SLE ?

A

Non organ specific humoral autoantibodies

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102
Q

Mention 2 antibodies that are specific for SLE?

A

Anti- Sm and anti-ds DNA

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103
Q

Mention 5 precipitating factors of SLE?

A 
Exposure to sunlight/ultraviolet radiations
Streptococcal 
Viral infections e.g EBV CMV, HIV
Pregnancy
Hormonal
Certain drugs
*hydralazine
*methyldopa
*procainamide
*minocycle
*anticonvulsants (carbamazepine, vaproate)
*D-penicillamine
*oral contraceptives
 Chemicals such as L-canavanine
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104
Q

Itemize 5 primary lesions seen in SLE?

A 
Fibrinoid necrosis
Collagen sclerosis
Necrosis 
Basophilic body formation
Vascular endothelial thickening
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105
Q

Immunohistological findings in SLE will reveal what?

A

Immunohistologically, IgG is predominant but less frequently IgA & IgM, together with complement (C1, C3) can be demonstrated at the DEJ.

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106
Q

What do s the predominant non-organ specific humoral antibody found in SLE?

A

IgG!

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107
Q

In SLE, Immunohistological findings is not seen in uninvolved skin (T/F)

A

False

It’s seen in uninvolved skin unlike chronic DLE

108
Q

In SLE, Immunohistological findings are commonly seen in lesions only (T/F)?

A

False

It’s also seen in sun exposed areas

109
Q

What are the Constitutional features seen in SLE?

A 
fatigue
fever
malaise
Myalgia
Arthralgia
weight loss
110
Q

Itemize 10 cutaneous features seen in SLE?

A 
Photosensitivity
Raynaud phenomenon
Butterfly rash
Chronic Urticaria
Non scarring Alopecia
Mouth Ulcerations
Chronic Discoid LE
Chilblain Lupus 
Scarring DLE alopecia
Cutaneous Vasculitis
Bullous eruptions 
Subacute Lupus Erythematous 
Facial oedema
Livedo reticularis
Episcleritis 
Cheilitis
111
Q

In what % of ptx with SLE is the butterfly malaria rash seen?

A

50%

112
Q

The characteristic cutaneous rash of SLE is commonly seen in what body areas?

A

forehead
periorbital area
sides of the neck
Cheek

113
Q

Scarring and non-scarring Alopecia are features of SLE (T/F)?

A

True!

114
Q

Outline 3 hands and feet lesions seen in SLE?

A

reticular telangiectasia
nail folds may show hyperkeratotic and rugged cuticles
splinter haemorrhages.

115
Q

What type of arthritis is seen in SLE?

A

inflammatory, symmetric, nonerosive!

116
Q

Itemize 4 haematologic changes seen in SLE?

A 
Anemia (may be hemolytic)
neutropenia
thrombocytopenia
lymphadenopathy
splenomegaly
venous or arterial thrombosis
117
Q

Itemize 4 cardiopulmonary changes seen in SLE?

A

pleuritis
pericarditis
myocarditis
endocarditis

118
Q

What gastrointestinal change is associated with SLE?

A

Peritonitis!

119
Q

Outline 3 neurological changes seen in SLE?

A

organic brain syndromes
seizures
psychosis

120
Q

Mention 4 systemic diseases associated with SLE?

A 
rheumatoid arthritis
systemic sclerosis
lichen sclerosus
morphea
sjogren syndrome
121
Q

State the 11 ACR CRITERIA required for the diagnosis of SLE?

A 
SOAP BRAIN MD* 
Serositis (pleurisy, Pericarditis) 
Oral/nasal ulcers (painless) 
Arthritis/Arthralgia(non-erosive)
Photosensitivity (skin) 
Blood (cytopenia)
Renal involvement 
ANA 
Immune (typical antibodies e.g. dsDNA, anti-Sm) Neurologic (e.g. Seizures, stroke) 
Malar rash 
Discoid rash
122
Q

How many out of the 11 ACR criteria is required to make a diagnosis of SLE?

A

4!

123
Q

In the MGT of SLE, what would you look for in the history?

A
  • Complete drug hx
  • Provocation/aggravation of skin eruptions through sunlight exposure
  • Chest pain
  • Joint pain
  • Numbness and pain in the fingers in the cold(sugg. of Raynaud Phenomenon)
  • Cvs symptoms-dypsnea
  • Seizures(indicating CNS involvement)
  • Menstrual abnormalities
  • proteinuria etc.
124
Q

What % of ptx with SLE have associated rheumatoid arthritis presenting as joint pain?

A

90%

125
Q

What would you be looking for in the physical examination of a ptx with SLE?

A

Classical ‘butterfly’ rash over nose and cheek(50%)
Appearance of erythematous rash in sun-exposed areas
Discoid lesions(occasionally)
Patchy alopecia
Other signs of internal diseases

126
Q

How would you investigate and ptx with SLE?

A 
HAEMATOLOGY 
Anaemia, Chk PCV
raised ESR
thrombocytopenia ( Do FBC)
decreased white cell count
  • Biopsy of skin lesions
  • Direct immunoflourescence
  • Immunological tests
  • Urinalysis
  • LFTs
  • Renal fxn tests/EUC
127
Q

What would skin biopsy in a ptx with SLE reveal?

A

some thinning of the epidermis
liquefaction degeneration of epidermal basal cells and
A mild Perivascular mononuclear cell infiltrate.

128
Q

What would direct immunoflourescence in SLE reveal?

A

IgG, IgM, IgA and C3 are found individually or together in a band-like pattern at the dermo-epidermal junction of involved skin and often uninvolved skin as well

129
Q

What would immunological tests in SLE reveal?

A 
Antinuclear antibody, 
Antibodies to double-stranded dna,
Low total complement level, 
Anti-sm antibody
Antiphospholipid antibody
130
Q

What would urinalysis in SLE reveal?

A

Proteinuria or haematuria, often with casts if kidneys involved

131
Q

The function of what organs must always be tested in suspected case of SLE?

A

Always test KIDNEY and LIVER function!

*Tests for function of other organs as indicated by history.

132
Q

Systemic steroid is the definitive cure for SLE (T/F)?

A

False

There’s no definitive cure for SLE!

133
Q

What is the aim of treatment in SLE?

A

Aim is to maintain optimal function with minimal therapy!

134
Q

What are the treatment options for SLE?

A

NON-PHARMACOLOGIC Tx
*Patients with photosensitivity should avoid sunlight and use high-factor sunscreens

PHARMACOLOGIC Tx
Systemic steroids are the mainstay of treatment!
oral corticosteroids
*PREDNISOLONE 60mg/d, tapered to 5-15mg/d.

INTRALESIONAL CORTICOSTEROIDS

  • 1% xylocaine
  • saline triamcinolone acetonide(kenalog)

NSAIDs (e.g., ibuprofen,aspirin)

IMMUNOSUPPRESSIVE AGENTS

  • azathioprine
  • cyclophosphamide
  • dapsone
  • other drugs (e.g. antihypertensive therapy or anticonvulsants) may also be needed.

ANTIMALARIAL DRUGS may help some patients with marked photosensitivity, as may sunscreens.

Long-term and regular follow-up is necessary!

135
Q

Mention 5 differentials of SLE?

A 
Malaria 
Polymyositis
Scleroderma
Mixed connective tissue disease
Rheumatoid Arthritis 
Sjogren’s syndrome
Lyme disease 
Antiphospholipid syndrome
136
Q

The appearance of circumscribed or diffuse, hard, smooth, ivory-colored areas that are immobile and give the appearance of “hidebound skin” is characteristically of what?

A

SCLERODERMA!

137
Q

Scleroderma is the thickening or hardening of the skin owing to what?

A

Abnormal dermal collagen!

138
Q

Scleroderma is not a diagnostic entity in itself (T/F)?

A

True

139
Q

In what 2 forms does scleroderma occur?

A

localized and systemic forms

140
Q

Outline the types of cutaneous scleroderma?

A

1) morphea
* localized
* generalized
* profunda
* atrophic
* pansclerotic types

2)linear scleroderma (with or without melorheostosis or hemiatrophy).

141
Q

Outline the 2 main types of systemic scleroderma?

A
  • Progressive systemic sclerosis

* CREST syndrome

142
Q

The CREST syndrome is otherwise called what?

A

Thibierge–Weissenbach syndrome

143
Q

SCLERODERMA is commoner in what sex?

A

Females!

Female:male ratio of 4:1

144
Q

What is the usual age of onset seen in scleroderma?

A

between the 3rd and 5th decade of life.

145
Q

The Aetiology of scleroderma is generally unknown (T/F)?

A

True!

146
Q

Classify scleroderma based on the degree and location of the skin involvement ?

A

SYSTEMIC

  • Limited cutaneous scleroderma-70%
  • Diffuse cutaneous scleroderma-30%

LOCALIZED

  • Localized scleroderma
  • Morphea
  • Systemic sclerosis sine scleroderma
  • Overlap syndrome with additional features of SLE ,RA, or inflammatory muscle disorder.
147
Q

Limited cutaneous scleroderma accounts for 30% of systemic sclerosis seen (T/F)?

A

False

It’s 70%

148
Q

Skin involvement in limited systemic sclerosis is limited to what body areas?

A

hands
face
feet
forearms.

149
Q

Limited systemic sclerosis often produces flexion deformities of the fingers (T/F)?

A

Very true!

The skin is tight over the fingers and often produces flexion deformities of the fingers

150
Q

characteristic ‘beak’-like nose and a small mouth (microstomia) is a distinct feature of diffuse systemic sclerosis (T/F)?

A

False!

It’s seen in Involvement of the skin of the face in limited systemic sclerosis!

151
Q

Skin changes in limited systemic sclerosis are usually followed by raynaud phenomenon (T/F)?

A

Very false!

Limited systemic sclerosis usually starts with Raynaud’s phenomenon many years (up to 15) before any skin changes.

152
Q

Painless digital ulcers and telangiectasia with dilated nail-fold capillary loops are seen in limited systemic sclerosis (T/F)?

A

False!

The digital ulcers are painful!

153
Q

Digital ischaemia and gangrene may be seen in limited systemic sclerosis (T/F)?

A

Very true!

154
Q

Gastrointestinal tract involvement is less common in limited systemic sclerosis (T/F)?

A

False

It’s common!

155
Q

Pulmonary hypertension develops in what % of ptx with Limited systemic sclerosis?

A

in 10-15% of this group

and pulmonary interstitial disease may occur.

156
Q

What does CREST syndrome stand for?

A 
The CREST syndrome
C-calcinosis
R-raynaud’s phenomenon
E-esophageal involvement
S-sclerodactyly
T-telangiectasia
157
Q

What are the cardinal features seen in diffuse systemic sclerosis?

A
  • Initially oedematous in onset
  • skin sclerosis rapidly follows.
  • Raynaud’s phenomenon usually starts just before or concomitant with the oedema.
  • weight loss.
158
Q

What does morphoea mean?

A

Localised sclerosis of the skin

159
Q

What age group of individuals are mostly affected by morphoea?

A

Can affect individuals of any age

160
Q

What is the peak incidence of morphoea?

A

between 20-40years.

161
Q

75%% of ptx with morphoea begin below the age of 10 (T/F)?

A

False

It’s 15%

162
Q

What sex is more affected by morphoea?

A

Females!

F : M 3:1

163
Q

Morphoea Can be divided into several subtypes based on clinical criteria which includes?

A

LOCALIZED MORPHEA

  • guttate
  • bullous
  • keloidal

GENERALIZED MORPHEA

  • Morphea profunda
  • Linear morphea
    • -Linear scleroderma
    • -morphea en coup de sabre ECDS
  • Pansclerotic morphea
  • Mixed forms
164
Q

What is the cause of morphoea?

A

It’s unknown!

165
Q

Mention 4 factors that have been implicated in the pathogenesis of morphoea?

A
  • Trauma
  • Borrelia burgdorferi
  • Phenylketonuria
  • Drugs like bromocriptine, pentazocine, penicillamine
166
Q

Where is the most favoured site of morphoea?

A

Trunk is the most favoured site!

167
Q

How would lesions from morphoea appear morphologically?

A

Lesions appear as bluish red plaques which progress to induration and then central white atrophy.

Plaque lesions which appear immobile, shiny and hypopigmented skin surrounded by a violaceous hue (lilac) or brown border

168
Q

Hairs are typically absent around morphoea lesions and the area ceases to sweat (T/F)?

A

Very true!

169
Q

What microscopic finding are seen in the early stages of morphoea?

A

In early stages,
Consist of an inflammatory infiltrate of lymphocytes, histiocytes and plasma cells found primarily in the subcutaneous tissue

170
Q

What microscopic findings are seen in the later stages of morphoea?

A

Later, the subcutaneous tissue is replaced by hyalinized connective tissue which is responsible for the induration of the skin

171
Q

A ptx presents with Plaque lesions which appear immobile, shiny and hypopigmented skin surrounded by a violaceous hue (lilac) or brown border. Hairs are noticed to be absent and the area ceases to sweat. This correctly describes what?

A

Morphoea!

172
Q

Outline the subtypes of morphoea?

A 
Localized 
Generalized 
Linear
Profunda
Pansclerotic
173
Q

Scarring Alopecia is a typical feature of localized morphoea (T/F)?

A

False

It’s generalized!

174
Q

There is usually no visceral involvement in generalized morphoea (T/F)?

A

True!

175
Q

Localised morphoea most commonly affects what body area?

A

Most common in TRUNKS but can also affect extremities.

176
Q

Muscle atrophy is a known feature of localized morphoea (T/F)?

A

False

It’s generalized morphoea!

177
Q

Muscle atrophy is a known feature of localized morphoea (T/F)?

A

False

It’s generalized morphoea!

178
Q

lesions of localized morphoea may extend the length of arm or leg and Often begins in the first decade of life (T/F)?

A

False

It’s linear morphoea!

179
Q

What usually presents as an indurated area on the forehead that may spread onto the scalp (with associated hair loss) or the face, with facial hemiatrophy in some patients.

A

Frontoparietal morphea(en coup de sabre):

180
Q

What is the Parry-Romberg syndrome?

A

Progressive hemifacial atrophy
Epilepsy
Exophthalmos
Alopecia

181
Q

Generalized morphoea may present as the parry-romberg syndrome (T/F)?

A

False

It’s linear morphoea!

182
Q

Generalized morphoea may present as the parry-romberg syndrome (T/F)?

A

False

It’s linear morphoea!

183
Q

Outline 4 clinical features associated with linear morphoea involving the Lower limbs ?

A

associated spina bifida
faulty limb development
hemiatrophy or flexion contractures. melorheostosis may be seen on radiography.

184
Q

Loss of wrinkles as a result of firmness and contraction of skin and Scarring alopecia are typical features of what?

A

Generalized morphoea!

185
Q

Linear morphoea may be associated with scarring Alopecia esp the “en coup de sabre” type (T/F)

A

False

Alopecia is not scarring

186
Q

What type of morphoea is associated with scarring Alopecia?

A

Generalized morphoea

187
Q

Morphoea characterised by widespread involvement of indurated plaques with pigmentary change is known as?

A

Generalized morphoea!

188
Q

A ptx presents with smooth, hard, somewhat depressed yellowish white or ivory lesions with margins surrounded by a lilac border or by telangiectasia which began initially as rose or violaceous macule correctly describes what lesion?

A

Localised morphoea!

189
Q

Linear morphoea lesions are usually single and unilateral (T/F)?

A

True!

190
Q

What form of morphoea involves deep subcutaneous tissue, including fascia?

A

Profunda sub-type

And also pansclerotic sub-type

191
Q

What form of morphoea is associated with sclerosis of the dermis, panniculus,fascia, muscle and at times the bone ?

A

Pansclerotic sub-type

192
Q

A well known complication of pansclerotic morphoea is what?

A

Ptx has disabling limitation of joint movement.

193
Q

What does Scleroderma sine scleroderma mean?

A

Refers to when Systemic features occur without skin involvement.

194
Q

What is the most common sub-type of morphoea?

A

Localised morphoea

195
Q

for linear morphoea, there is spontaneous resolution in about 1.5-4 years (T/F)?

A

False

It’s localized morphoea

196
Q

Secondary changes seen in morphoea e.g pigmentary, facial deformity are usually temporary (T/F)?

A

False

They are more permanent!

197
Q

Occasionally, ulceration may develop in old lesions of morphea (T/F)?

A

Very true!

198
Q

Some patients with localised morphea may survive for decades (T/F)?

A

False

It’s generalised morphoea!

199
Q

Itemize 3 theories which may explain the etiology of systemic sclerosis ?

A
  • Abnormal responsiveness of blood vessels
  • Augmented synthesis of collagen by dermal fibroblasts
  • Inappropriate cellular immune responsiveness
200
Q

Itemize 3 theories which may explain the etiology of systemic sclerosis ?

A
  • Abnormal responsiveness of blood vessels
  • Augmented synthesis of collagen by dermal fibroblasts
  • Inappropriate cellular immune responsiveness
201
Q

The kidneys are involved early in systemic sclerosis, but effects are usually mild (T/F)?

A

Very false!!

The kidneys are involved late, but this has a grave prognosis from MALIGNANT HYPERTENSION

202
Q

Mention 10 features of systemic involvement in systemic sclerosis?

A

most have abnormalities of the Gut including

  • dysphagia,
  • oesophagitis,
  • constipation,
  • diarrhoea
  • malabsorption.
  • Fibrosis of the lungs leads to dyspnoea
  • Fibrosis of the heart to congestive failure.
203
Q

“The nose becomes beak-like and wrinkles radiate around the mouth” this statement correctly describes features of what?

A

Systemic sclerosis!

204
Q

Outline the 3 stages of evolution of skin manifestations in scleroderma?

A

Edematous
Indurative
Atrophic

205
Q

loss of normal skin wrinkles, the follicular orifices becoming unduly prominent describes what stage of scleroderma skin manifestation?

A

Edematous stage

206
Q

Peau d’orange skin appearance describes what stage of scleroderma skin manifestation?

A

Edematous stage

207
Q

The edematous stage of scleroderma skin manifestation may last for how long?

A

several weeks to month.

208
Q

How does the skin appear in the Edematous stage of scleroderma?

A

The skin simply appear stretched and taut and pits on pressure (more prominent on the face and dorsi of the hands)

209
Q

When it becomes impossible to pickup the skin between the thumb and index finger, what stage of scleroderma skin manifestation is this?

A

Indurative stage

210
Q

How does the skin appear in the indurative stage of scleroderma skin manifestation?

A

The skin becomes hard and bound down to the underlying fascia

211
Q

What body areas is the Indurative stage of scleroderma skin manifestation most prominent ?

A

on the face
dorsi of the hands
fore-arms and the fingers

212
Q

The characteristic “pinched-mask expressionless facies” is a feature of what stage of scleroderma skin manifestation?

A

Atrophic stage

213
Q

tip of the nose becomes pinched and pointed in what stage of scleroderma skin manifestation?

A

Attophic stage

214
Q

progressive loss of hair follicles, thinning of the lips, puckering and narrowing of the oral aperture are characteristic features of Indurative stage of skin manifestation (T/F)?

A

False!

It’s the Atrophic stage

215
Q

Mention 3 finger nail changes seen in systemic sclerosis?

A 
Loss of pulp substance 
Periungual telangiectasiaa
Painful digital ulcers 
Scerotic skin (flecks of calcium(chalk white substance) extruding) 
Claw hand deformity
216
Q

The diagnosis of systemic sclerosis is usually made how? And why is this so?

A
  • The diagnosis is made clinically

* because histological abnormalities are seldom present until the physical signs are well established.

217
Q

The Thibierge-Weissenbach syndrome is comprised of what?

A

calcinosis + scleroderma

218
Q

Outline the diagnostic criteria for systemic sclerosis?

A

ONE MAJOR CRITERION
*scleromatous skin changes proximal to the metacarpal-phalangeal joints

TWO OF THREE MINOR CRITERIA:

  • sclerodactyly,
  • digital pitting scars
  • bibasilar pulmonary fibrosis on chest radiograph.
219
Q

Rheumatoid factor is positive in 70% of cases in systemic sclerosis (T/F)?

A

False!

It’s ANA!

220
Q

What investigations would you carry out in a suspected case of systemic sclerosis?

A 
FBC
E &U
Urinalysis
Imaging tech
Auto antibodies
Rheumatoid factor is positive in 30%
ANA is positive in 70%
ESR- elevated
Cryoglobulins - elevated
Hypergammaglobulinemia
221
Q

Mention 4 auto-antibodies associated with systemic sclerosis ?

A

Anticentromere abs –LcSc
Antitopoisomerase 1 –DcSc
RF in 30%
Antinuclear Abs ANA -70%

222
Q

What name is given to the characteristic appearance seen on upper GI endoscopy in systemic sclerosis?

A

“Water Melon stomach” which represents dilated submucosal capillaries in the stomach

223
Q

What is the characteristic feature seen on x-ray of the hand in a ptx with systemic sclerosis?

A

calcium deposits around fingers with erosion & absorption of the tufts of the fingers, called OSTEONECROLYSIS

224
Q

What characteristic appearance is seen on CXR in a ptx with systemic sclerosis?

A

Honeycomb appearance and basal fibrosis

225
Q

What imaging modalities would you use in the MGT of a ptx with systemic sclerosis?

A 
Xray of hands
CXR
Lung Fxn Tests
High resolution CT of the chest
Barium swallow
mannometry
upper GI endoscopy
226
Q

What are the treatment options for systemic sclerosis?

A

treatment depends upon which organs are involved!
but systemic steroids are usually needed
LOCALIZED LESIONS
*topical corticosteroids or calcipotriol
The safest class of systemic agents – ANTIMALARIALS
2nd line agents — RETINOIDS
3rd line –SYSTEMIC IMMUNOSUPPRESSIVE AGENTS
*Thalidomide is effective but use is limited

FOR ACUTE FLARES
*oral prednisolone

OTHERS
penicillamine
d-penicillamine
colchicine
cyclophospamide
methotrexate
steroids – low dose daily
ace inhibitors
calcium channel blockers
ultraviolet A
interferon
227
Q

management of morphoea is relatively easy (T/F)?

A

False

It’s difficult!

228
Q

Systemic steroids are usually needed in the mgtbof systemic sclerosis (T/F)?

A

Very true!

229
Q

What’s the 1st line agent used in the mgt of systemic sclerosis?

A

Antimalarials

230
Q

How is raynaud phenomenon in systemic sclerosis managed?

A

Control vasospasm
Dress warmly
avoid smoking

AVOID

  • amphetamine
  • ergotamine
  • ß-blocker

VASODLATORS

  • Angiotensin receptor blockers
  • Nitroglycerin paste
  • Sildenafil
  • Ketanserin
  • Fluoxetine
  • IV prostagladins
  • prostacyclin analogs.
231
Q

What inflammatory myopathy is characterised by a distinctive skin rash which may accompany or preceed the onset of muscle disease?

A

Dermatomyositis!

232
Q

A scaling erythematous eruption or dusky red patches over the knuckles, elbows,and knees seen in Dermatomyositis is known as?

A

grotton lesions.

233
Q

What group of muscles are affected first in Dermatomyositis?

A

Proximal muscles!

234
Q

Proximal myopathy in Dermatomyositis is bilaterally symmetrical (T/F)?

A

True!

235
Q

What tasks become difficult for a ptx with Dermatomyositis?

A

Getting up from a chair

Climbing stairs

236
Q

The heart is commonly affected in Dermatomyositis (T/F)?

A

False

Less common

237
Q

What body parts are commonly affected in Dermatomyositis?

A

The joints
Esophagus
Lungs

238
Q

What are the types of Dermatomyositis?

A

Adult type

Juvenile type

239
Q

What’s the incidence of Dermatomyositis?

A

1:100,000

240
Q

What sex group are more commonly affected by Dermatomyositis?

A

Females

F:M (2:1)

241
Q

Dermatomyositis usually occurs in association with systemic sclerosis and mixed connective tissue disease (T/F)?

A

False!

usually occurs Alone but sometimes can be associated with systemic sclerosis and mixed connective tissue disease.

242
Q

What age group are commonly affected by Dermatomyositis in children?

A

10-15yrs

243
Q

What age group are commonly affected by Dermatomyositis in adults?

A

45-60yrs

244
Q

The cause of Dermatomyositis is largely unknown (T/F)?

A

True

245
Q

Dermatomyositis may be considered as an auto-immune disorder (T/F)?

A

True!

246
Q

The etiology of Dermatomyositis may be of infectious origin (T/F)?

A

True!

Infections caused by bacteria, parasites or viruses are known to cause inflammatory myopathies

247
Q

but in most cases of Dermatomyositis no preceeding infection has been identified (T/F)

A

Very true!

248
Q

Genetic susceptibility plays no role in Dermatomyositis (T/F)?

A

False

It may play a role

249
Q

Itemize 5 agents that have been implicated in the etiology of Dermatomyositis

A 
Hydroxyurea
Penicillamine
Statin drugs
Quinidine
Phenylbutazone
250
Q

Why may ptx with CML or essential thrombocytosis present with Dermatomyositis like skin changes?

A

Bcoz they are treated with hydroxyurea

251
Q

After what age would Dermatomyositis signal internal malignancy?

A

Age 40

252
Q

Auto-antibodies to smooth muscles have been found in Dermatomyositis (T/F)?

A

False

It’s striated muscles

253
Q

Outline 3 pathognomonic clinical features of Dermatomyositis?

A
  • A violaceous periorbital eruption (helitrope rash)
  • scaly papules over the knuckles (Gottron’s papules)
  • Weakness of the proximal muscles is found in most cases.
254
Q

What are the clinical features of Dermatomyositis?

A

This weakness is symmetrical and causes *difficulty in getting out of chair

  • difficulty in climbing stairs
  • difficulty in brushing hairs
  • dysphagia
  • muscle pain
  • fatigue
  • fever
  • weight loss.
255
Q

Skin and mucous membrane involvement in Dermatomyositis have a predilection for sun exposed areas (T/F)?

A

Very true!

256
Q

What is known as SCLERODERMOMYOSITIS?

A

A finding In some children with Dermatomyositis in Whc the skin may become thick and hard in a way similar to scleroderma.

257
Q

Skin involvement in Dermatomyositis affects what body areas commonly?

A 
the face
neck
shoulders (shawl sign)
elbows
forearms
hands.
258
Q

Rash of Dermatomyositis tends to be exacerbated by sunlight (T/F)?

A

True!

259
Q

What is the shawl sign?

A

Erythema over the shoulders and neck in dermatomyositis

260
Q

How is diagnosis of Dermatomyositis made?

A

Diagnosis depends on Clinical presentation! e.g skin rash

DIAGNISTIC CRITERIA:

1) proximal muscle weakness,
2) Elevated serum creatinine kinase,
3) Myopathic changes on electromyography
4) Muscle biopsy with evidence of a lymphocytic inflammation.

*Diagnosis is Definitive with all four criteria, probable with three, and possible with two.

261
Q

What investigations should be done in a suspected case of Dermatomyositis?

A

Electromyography
Blood analysis
Muscle enzymes like creatine kinase and aldolase.
Detection of autoantibodies
Muscle biopsy to show inflammatory evidence in the muscle.
Skin biopsy
MRI which may help to detect inflammation.

262
Q

There is no cure for dermatomyositis (T/F)?

A

True!

263
Q

What is the rationale for treatment in Dermatomyositis?

A

treatment can improve the skin, muscle strength and function.

264
Q

What are the treatment options for Dermatomyositis?

A 
High dose corticosteroids like PREDNISOLONE which is tapered as the signs and symptoms improve.
Topical corticosteroids.
Calcium and vitamin D
Immunosuppressants
*azathioprine
*methotrexate
*cyclophosphamide
*cyclosporine

Antimalarials – for persistent rash.

  • chloroquine
  • hydroxychloroquine

Physical therapy to improve muscle strength and function.
Surgery to remove painful calcium deposit.
Pain relievers
*NSAID; paracetamol and codeine

265
Q

Topical corticosteroid therapy in Dermatomyositis is often needed for years (T/F)?

A

True!

266
Q

Mention 5 complications of Dermatomyositis?

A 
Dysphagia
Weight loss 
Malnutrition
Dyspnea
Calcinosis
Myocarditis
CCF and arrythmias
Cancer
267
Q

Dermatomyositis in adult has been linked to increase likelihood of cancer particularly in what organs?

A

lungs
breasts
ovaries

Dermatology OAU (10 decks)

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