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Inflammatory Connective Tissue Disease > 28 Localized Scleroderma > Flashcards

28 Localized Scleroderma Flashcards

1
Q

T/F Compared to SSc, LS is usually unilateral and less extensive

A

T

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2
Q

T/F SSc has a different pattern of extracutaneous involvement compared to LS

A

T

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3
Q

T/F SSc has a GENERALLY much better prognosis than LS

A

F

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4
Q

PReS classification of LS

A

1) Circumscribed (superficial or deep) 2) Linear (trunk/limb or head) 3) Generalized 4) Pansclerotic 5) Mixed

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5
Q

T/F CM lesions occur most commonly in the trunk and less often in the extremities

A

T

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6
Q

T/F CM lesions often spare the face

A

T

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7
Q

4 or more individual lesions, typically >3cm diameter, involving at least 2 of 7 anatomic sites

A

GM

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8
Q

7 anatomical sites of affectation in morphea

A

Head-neck, RUE, LUE, RLE, LLE, anterior trunk, posterior trunk

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9
Q

MC type of LS in pediatrics

A

Linear scleroderma

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10
Q

T/F Linear scleroderma typically affect the trunk

A

F, upper or lower ext and face

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11
Q

Distribution of linear scleroderma typically follow this embryonic pattern

A

Blaschko’s lines

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12
Q

T/F Linear scleroderma are confined to the superficial layers of the skin

A

F, may involve underlying tissues up to bone

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13
Q

What are en coup de sabre

A

Linear lesions of the face or scalp

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14
Q

T/F Scalp involvement of linear scleroderma results in non-scarring alopecia

A

F, SCARRING and often irreversible

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15
Q

Form of linear Scl of the face and head that presents as progressive hemifacial atrophy due to loss of tissue on 1 side of the face

A

Parry-Romberg syndrome

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16
Q

LS described as having a lilac ring

A

Circumscribed morphea

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17
Q

2 forms of linear Scl of the head

A

ECDS (en coup de sabre) and PRS (parry romberg syndrome)

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18
Q

Circumferential involvement of limbs affecting skin up to bone

A

Pansclerotic morphea

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19
Q

Extremely rare in children; most severe subtype of LS with potential of auto-amputation of the affected limb

A

Disabling pansclerotic morphea

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20
Q

Disabling pansclerotic morphea is associated with what CA

A

Sq cell CA

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21
Q

Skin disorder associated with LS that presents as violaceous discoloration, progressing to shiny, white superficial plaques, typically along anogenital area and over wrists and ankles

A

Lichen sclerosus et atrophicus

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22
Q

Skin disorder associated with LS that can create a peau d’ orange appearance, tends to involve the hands and feet of children, and can be mistaken as DPM

A

Eosinophilic fasciitis

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23
Q

Differentiate EF from DPM (disabling pansclerotic morphea)

A

EF: Spares face and trunk, high peripheral blood eosinophil counts, and thickened, inflamed fascia

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24
Q

T/F Most patients with LS present at the 1st decade of life

A

T

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25
Q

T/F Skin biopsies of LS and SSc are often indistinguishable

A

T

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26
Q

Proposed initiating event in SSc and LS

A

Endothelial cell/vascular injury

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27
Q

Potential environmental triggers for LS

A

Trauma, IM injections, meds, radiation, pregnancy, infection

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28
Q

Bacteria evaluated as potential etiology for LS-like lesions in many studies

A

Borrelia

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29
Q

Reported triggers for EF

A

Muscle trauma (more in adults), infections ( more in children, medications, radiotherapy, burns

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30
Q

Predominant cells found in skin lesions of patients with LS

A

T cells, predominantly T helper cells, with reduction in functional regulatory T cells

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31
Q

T/F Patients with LS can present with generalized LAD

A

T, especially pansclerotic morphea

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32
Q

T/F Major organ system manifestations such as arthritis or neurologic manifestations may precede development of skin lesions in LS

A

T

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33
Q

T/F LS can present at birth

A

T

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34
Q

Color of early skin lesions of LS, reflecting the initial inflammatory phase

A

Erythematous to violaceous, with normal skin texture and thickness

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35
Q

Characteristics of active LS lesions

A

Red/violaceous rim, increased local warmth, raised borders, dermal thickening

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36
Q

T/F Skin thickening in LS is SPECIFIC for disease activity

A

F, associated with both active and inactive lesions

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37
Q

T/F LS does not present with extracutaneous manifestations

A

F, 20-70% have extracutaneous

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38
Q

MC extracutaneous manifestations of LS

A

MSK, neurologic, ocular, oral

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39
Q

LS MC associated with growth defects

A

Linear Sc of an extremity

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40
Q

LS MC associated with neurological, oral, and ocular problems

A

Linear Sc of the head

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41
Q

LS Mc associated with joint involvement

A

Linear Sc of extremities, pansclerotic, generalized, and deep morphea

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42
Q

Extracutaneous manifestations of LS that have been reported to precede the appearance of skin lesions, sometimes by years

A

Seizures, headaches, arthropathy

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43
Q

Involvement of these organ systems are associated with a higher risk of multiple extracutaneous manifestations of Ls

A

Neurologic, ocular extracutaneous manifestations

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44
Q

MC type of extracutaneous manifestations of LS

A

MSK

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45
Q

Painless limitation of joint ROM, thickened synovium associated with fibrosis, and sparse inflammatory infiltrate

A

Dry synovitis

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46
Q

Morphea associated with a higher risk for osteoporosis, caclifications, and MSK problems

A

Pansclerotic morphea

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47
Q

MC GI problem in LS

A

GER

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48
Q

T/F Histological documentation is ESSENTIAL in confirming clinical diagnosis of LS

A

F

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49
Q

Predominant cells seen in active lesions of LS

A

Lymphocytes

50
Q

Can differentiate ECDS from other causes of alopecia (histopath)

A

Perineural lymphoplasmacytic infiltration

51
Q

Predominant collagen in later stages of LS

A

Type I

52
Q

Predominant collagen in early stages of LS

A

Type III

53
Q

Fascial thickening, dermis/epidermmis may be unaffected, is characteristic of

A

EF

54
Q

Compared to SSc, pathology of LS shows

A

1) More intense inflammatory infiltrate often found at the dermal-subcutaneous junction (a site not involved in SSc) 2) Collagen bundles distributed throughout the dermis in LS (concentrated in the lower reticular dermis in SSc, sparing the papillary dermis)

55
Q

SSc vs LS: Raynaud phenomenon

A

SSc

56
Q

SSc vs LS: Internal organ involvement

A

SSc

57
Q

SSc vs LS: Generally spares the central back

A

SSc

58
Q

SSc vs LS: More severely affects fingers and hands

A

SSc

59
Q

T/F EF requires documentation of fascial involvement

A

T, either through biopsy or MRI (hyperintensity and enhancement of fascia)

60
Q

MC antibody found in LS

A

ANA, homogenous, speckled, and nucleolar

61
Q

LS antibody associated with EC manifestations

A

ANA

62
Q

LS antibody associated with activity, extensive disease, functional limitation, and muscle disease

A

Anti-ssDNA

63
Q

LS antibody found MC in GM

A

APLA

64
Q

T/F Eye involvement in LS can be asymptomatic

A

T

65
Q

T/F In LS, damage features if atrophy and dyspigmentation indicate inactive disease

A

F, these features commonly coexist with active disease

66
Q

LS scoring system that has been widely adopted and easy to use

A

mLoSSI

67
Q

mLoSSI divides the body into 18 anatomical sites and scores the ff lesion features (3)

A

Erythema, skin thickening, presence of a new or larger lesion

68
Q

Erythema and skin thickening are scored in mLoSSI on a scale of

A

0 to 3

69
Q

Imaging recommended for patients with LS and neurological symptoms

A

MRI with GAD

70
Q

Invaluable imaging modality for diagnosis of EF

A

MRI

71
Q

Topical treatments are considered appropriate for what classification of LS

A

Superficial CM

72
Q

Topical treatments considered appropriate for superficial CM

A

Tacrolimus 0.1%, calcipotriol 0.05%, topical CS, imiquimod or combination therapies

73
Q

DOC for systemic treatment of moderate to severe LS

A

MTX

74
Q

T/F CS treatment alone is a good choice for patients with LS

A

F, this has not been found to provide sustained benefit

75
Q

Potential risk factors for relapses of LS

A

1) Relapsing course in the 1st 2 years of treatment 2) Longer follow-up time 3) Linear subtype

76
Q

Drug that can be used for LS patients intolerant of or have inadequate response to MTX

A

MMF

77
Q

Biologics that can be used in difficult to treat LS patientts

A

Infliximab and Tocilizumab

78
Q

First line drug for EF

A

Corticosteroids

79
Q

Optimal conditions for surgical management in LS

A

1) Inactive disease 2) Growth is complete

80
Q

Deaths in LS is usually associated with

A

1) Pansclerotic subtype 2) Chronic skin ulcer complications (sepsis, sq cell CA)

81
Q

T/F Patients with LS have a higher frequency of other autoimmune diseases

A

T

82
Q

Major cause of disability in LS

A

MSK problems

83
Q

T/F Most adults with EF achieve remission

A

T

84
Q

May represent burned out phase of deep morphea

A

Atrophoderma of Pasini and Pierini

85
Q

Hyperpigmented, atrophic patches with well-demarcated “cliff-drop” borders seen primarily on the trunk

A

Atrophoderma of Pasini and Pierini

86
Q

Late-stage morphea lesions

A

Visible venous pattern resulting from epidermal and dermal atrophy
Hyperpigmentation and dermal loss with “cliff-border” appearance
Marked areas of dermal and subcutaneous atrophy

87
Q

Proposed initiating event in SSc

A

Endothelial cell injury resulting in releasing of cell mediators, upregulation of cell adhesion molecules, and activation and recruitment of immune cells

88
Q

Key event in the pathogenesis of LS

A

Vascular injury

89
Q

T/F Longer disease duration of LS is associated with higher frequency of concomitant autoimmune disease

A

T

90
Q

Unlike LS, there is a predominance of ___ cells in the muscle and fascia infiltrates of EF

A

CD8+ rather than CD4+ T cells

91
Q

T/F Skin thickening is specific for activity

A

F, associated with both active and inactive lesions

92
Q

Damage features of LS

A

Postinflammatory hyperpig
Epidermal atrophy (shiny skin with visible venous pattern)
Dermal atrophy (loss of hair follicles and adnexal structures and cliff-drop atrophy)
Subcu atrophy (loss of fat)
Progressive skin thickening

93
Q

MC extracutaneous manif of LS

A

MSK, neuro, ocular, oral

94
Q

Higher frequency of neuro involvement is seen in LS patients with what subtype

A

LS of the head (ECDS, PRS)

95
Q

Organ systems that have been found to be uncommonly affected in LS

A

GI, pulmo, cardiac, renal

96
Q

Subtype of LS associated with higher frequency of lung involvement

A

Pansclerotic morphea

97
Q

EF vs LS: Acute onset of painful, rapidly progressive symmetrical involvement of extremities

A

EF

98
Q

T/F Histological documentation is helpful but NOT essential in confirming the clinical dx of LS

A

T

99
Q

EF shows focal absence of ___ staining

A

CD34

100
Q

LS vs SSc: Inflammtory infiltrate is more intense and often found at the dermal-subcu junction

A

LS

101
Q

LS vs SSc: Collagen bundles distirbuted throughout the dermis

A

LS

102
Q

LS vs SSc: Collagen bundles concentrated in the lower reticular dermis

A

SSc

103
Q

LS vs SSc: Papillary dermis shows sclerosis

A

LS

104
Q

Early lesions of LS are most commonly confused with

A

portwine stain or patch stage of mycosis fungoides

105
Q

T/F LS patients rarely develop RP

A

T

106
Q

SSc generally spares this area

A

Central back

107
Q

EF is a common ddx for what phase of LS

A

All 3 phases

108
Q

Phases of LS

A

Inflamm
Infiltrative (indurated)
Inactive (“damage”)

109
Q

Most frequently found antibody in LS

A

ANA, homogenous, speckled, and nucleolar

110
Q

Instrument that is highly recommended to assess activity and severity in JLS

A

LoSSI

111
Q

Instrument that is highly recommended to assess damage in JLS

A

LoSDI

112
Q

T/F US is NOT recommended to assess disease act, extent of JLS, and response to treatment

A

F

113
Q

T/F All patients with JLS should be evaluated with a COMPLETE joint exam at dx and every visit

A

T

114
Q

MRI can be a useful tool to assess MSK involvement in LS especially when

A

Skin lesions cross the joint

115
Q

T/F It is recommended that all patients with JLS involving the face and head undergo an MRI even without symptoms

A

T

116
Q

Tx: Superficial CM

A

1) Topical treatments (tacrolimus, calcipotriol, CS, imiquimod)
2) Phototherapy

117
Q

Moderate to severe LS

A

Systemic: MTX, CS

118
Q

T/F CS treatment alone has been found to have sustained benefit for LS patients

A

F

119
Q

Treatment of EF

A

1) Oral and IV CS
2) MTX for those who dont respond to CS or are steroid dependent

120
Q

MCC of mortality in LS

A

Deaths are extremely rare! MC pansclerotic type and due to chronic skin ulcer complications (sepsis, Squamous cell CA)

121
Q

Major cause of morbidity in LS

A

MSK problems

Inflammatory Connective Tissue Disease (8 decks)

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