28 Localized Scleroderma Flashcards
1
Q
T/F Compared to SSc, LS is usually unilateral and less extensive
A
T
2
Q
T/F SSc has a different pattern of extracutaneous involvement compared to LS
A
T
3
Q
T/F SSc has a GENERALLY much better prognosis than LS
A
F
4
Q
PReS classification of LS
A
1) Circumscribed (superficial or deep) 2) Linear (trunk/limb or head) 3) Generalized 4) Pansclerotic 5) Mixed
5
Q
T/F CM lesions occur most commonly in the trunk and less often in the extremities
A
T
6
Q
T/F CM lesions often spare the face
A
T
7
Q
4 or more individual lesions, typically >3cm diameter, involving at least 2 of 7 anatomic sites
A
GM
8
Q
7 anatomical sites of affectation in morphea
A
Head-neck, RUE, LUE, RLE, LLE, anterior trunk, posterior trunk
9
Q
MC type of LS in pediatrics
A
Linear scleroderma
10
Q
T/F Linear scleroderma typically affect the trunk
A
F, upper or lower ext and face
11
Q
Distribution of linear scleroderma typically follow this embryonic pattern
A
Blaschko’s lines
12
Q
T/F Linear scleroderma are confined to the superficial layers of the skin
A
F, may involve underlying tissues up to bone
13
Q
What are en coup de sabre
A
Linear lesions of the face or scalp
14
Q
T/F Scalp involvement of linear scleroderma results in non-scarring alopecia
A
F, SCARRING and often irreversible
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
16
Q
LS described as having a lilac ring
A
Circumscribed morphea
17
Q
2 forms of linear Scl of the head
A
ECDS (en coup de sabre) and PRS (parry romberg syndrome)
18
Q
Circumferential involvement of limbs affecting skin up to bone
A
Pansclerotic morphea
19
Q
Extremely rare in children; most severe subtype of LS with potential of auto-amputation of the affected limb
A
Disabling pansclerotic morphea
20
Q
Disabling pansclerotic morphea is associated with what CA
A
Sq cell CA
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
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
23
Q
Differentiate EF from DPM (disabling pansclerotic morphea)
A
EF: Spares face and trunk, high peripheral blood eosinophil counts, and thickened, inflamed fascia
24
Q
T/F Most patients with LS present at the 1st decade of life
A
T
25
T/F Skin biopsies of LS and SSc are often indistinguishable
T
26
Proposed initiating event in SSc and LS
Endothelial cell/vascular injury
27
Potential environmental triggers for LS
Trauma, IM injections, meds, radiation, pregnancy, infection
28
Bacteria evaluated as potential etiology for LS-like lesions in many studies
Borrelia
29
Reported triggers for EF
Muscle trauma (more in adults), infections ( more in children, medications, radiotherapy, burns
30
Predominant cells found in skin lesions of patients with LS
T cells, predominantly T helper cells, with reduction in functional regulatory T cells
31
T/F Patients with LS can present with generalized LAD
T, especially pansclerotic morphea
32
T/F Major organ system manifestations such as arthritis or neurologic manifestations may precede development of skin lesions in LS
T
33
T/F LS can present at birth
T
34
Color of early skin lesions of LS, reflecting the initial inflammatory phase
Erythematous to violaceous, with normal skin texture and thickness
35
Characteristics of active LS lesions
Red/violaceous rim, increased local warmth, raised borders, dermal thickening
36
T/F Skin thickening in LS is SPECIFIC for disease activity
F, associated with both active and inactive lesions
37
T/F LS does not present with extracutaneous manifestations
F, 20-70% have extracutaneous
38
MC extracutaneous manifestations of LS
MSK, neurologic, ocular, oral
39
LS MC associated with growth defects
Linear Sc of an extremity
40
LS MC associated with neurological, oral, and ocular problems
Linear Sc of the head
41
LS Mc associated with joint involvement
Linear Sc of extremities, pansclerotic, generalized, and deep morphea
42
Extracutaneous manifestations of LS that have been reported to precede the appearance of skin lesions, sometimes by years
Seizures, headaches, arthropathy
43
Involvement of these organ systems are associated with a higher risk of multiple extracutaneous manifestations of Ls
Neurologic, ocular extracutaneous manifestations
44
MC type of extracutaneous manifestations of LS
MSK
45
Painless limitation of joint ROM, thickened synovium associated with fibrosis, and sparse inflammatory infiltrate
Dry synovitis
46
Morphea associated with a higher risk for osteoporosis, caclifications, and MSK problems
Pansclerotic morphea
47
MC GI problem in LS
GER
48
T/F Histological documentation is ESSENTIAL in confirming clinical diagnosis of LS
F
49
Predominant cells seen in active lesions of LS
Lymphocytes
50
Can differentiate ECDS from other causes of alopecia (histopath)
Perineural lymphoplasmacytic infiltration
51
Predominant collagen in later stages of LS
Type I
52
Predominant collagen in early stages of LS
Type III
53
Fascial thickening, dermis/epidermmis may be unaffected, is characteristic of
EF
54
Compared to SSc, pathology of LS shows
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
SSc vs LS: Raynaud phenomenon
SSc
56
SSc vs LS: Internal organ involvement
SSc
57
SSc vs LS: Generally spares the central back
SSc
58
SSc vs LS: More severely affects fingers and hands
SSc
59
T/F EF requires documentation of fascial involvement
T, either through biopsy or MRI (hyperintensity and enhancement of fascia)
60
MC antibody found in LS
ANA, homogenous, speckled, and nucleolar
61
LS antibody associated with EC manifestations
ANA
62
LS antibody associated with activity, extensive disease, functional limitation, and muscle disease
Anti-ssDNA
63
LS antibody found MC in GM
APLA
64
T/F Eye involvement in LS can be asymptomatic
T
65
T/F In LS, damage features if atrophy and dyspigmentation indicate inactive disease
F, these features commonly coexist with active disease
66
LS scoring system that has been widely adopted and easy to use
mLoSSI
67
mLoSSI divides the body into 18 anatomical sites and scores the ff lesion features (3)
Erythema, skin thickening, presence of a new or larger lesion
68
Erythema and skin thickening are scored in mLoSSI on a scale of
0 to 3
69
Imaging recommended for patients with LS and neurological symptoms
MRI with GAD
70
Invaluable imaging modality for diagnosis of EF
MRI
71
Topical treatments are considered appropriate for what classification of LS
Superficial CM
72
Topical treatments considered appropriate for superficial CM
Tacrolimus 0.1%, calcipotriol 0.05%, topical CS, imiquimod or combination therapies
73
DOC for systemic treatment of moderate to severe LS
MTX
74
T/F CS treatment alone is a good choice for patients with LS
F, this has not been found to provide sustained benefit
75
Potential risk factors for relapses of LS
1) Relapsing course in the 1st 2 years of treatment 2) Longer follow-up time 3) Linear subtype
76
Drug that can be used for LS patients intolerant of or have inadequate response to MTX
MMF
77
Biologics that can be used in difficult to treat LS patientts
Infliximab and Tocilizumab
78
First line drug for EF
Corticosteroids
79
Optimal conditions for surgical management in LS
1) Inactive disease 2) Growth is complete
80
Deaths in LS is usually associated with
1) Pansclerotic subtype 2) Chronic skin ulcer complications (sepsis, sq cell CA)
81
T/F Patients with LS have a higher frequency of other autoimmune diseases
T
82
Major cause of disability in LS
MSK problems
83
T/F Most adults with EF achieve remission
T
84
May represent burned out phase of deep morphea
Atrophoderma of Pasini and Pierini
85
Hyperpigmented, atrophic patches with well-demarcated "cliff-drop" borders seen primarily on the trunk
Atrophoderma of Pasini and Pierini
86
Late-stage morphea lesions
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
Proposed initiating event in SSc
Endothelial cell injury resulting in releasing of cell mediators, upregulation of cell adhesion molecules, and activation and recruitment of immune cells
88
Key event in the pathogenesis of LS
Vascular injury
89
T/F Longer disease duration of LS is associated with higher frequency of concomitant autoimmune disease
T
90
Unlike LS, there is a predominance of ___ cells in the muscle and fascia infiltrates of EF
CD8+ rather than CD4+ T cells
91
T/F Skin thickening is specific for activity
F, associated with both active and inactive lesions
92
Damage features of LS
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
MC extracutaneous manif of LS
MSK, neuro, ocular, oral
94
Higher frequency of neuro involvement is seen in LS patients with what subtype
LS of the head (ECDS, PRS)
95
Organ systems that have been found to be uncommonly affected in LS
GI, pulmo, cardiac, renal
96
Subtype of LS associated with higher frequency of lung involvement
Pansclerotic morphea
97
EF vs LS: Acute onset of painful, rapidly progressive symmetrical involvement of extremities
EF
98
T/F Histological documentation is helpful but NOT essential in confirming the clinical dx of LS
T
99
EF shows focal absence of ___ staining
CD34
100
LS vs SSc: Inflammtory infiltrate is more intense and often found at the dermal-subcu junction
LS
101
LS vs SSc: Collagen bundles distirbuted throughout the dermis
LS
102
LS vs SSc: Collagen bundles concentrated in the lower reticular dermis
SSc
103
LS vs SSc: Papillary dermis shows sclerosis
LS
104
Early lesions of LS are most commonly confused with
portwine stain or patch stage of mycosis fungoides
105
T/F LS patients rarely develop RP
T
106
SSc generally spares this area
Central back
107
EF is a common ddx for what phase of LS
All 3 phases
108
Phases of LS
Inflamm
Infiltrative (indurated)
Inactive ("damage")
109
Most frequently found antibody in LS
ANA, homogenous, speckled, and nucleolar
110
Instrument that is highly recommended to assess activity and severity in JLS
LoSSI
111
Instrument that is highly recommended to assess damage in JLS
LoSDI
112
T/F US is NOT recommended to assess disease act, extent of JLS, and response to treatment
F
113
T/F All patients with JLS should be evaluated with a COMPLETE joint exam at dx and every visit
T
114
MRI can be a useful tool to assess MSK involvement in LS especially when
Skin lesions cross the joint
115
T/F It is recommended that all patients with JLS involving the face and head undergo an MRI even without symptoms
T
116
Tx: Superficial CM
1) Topical treatments (tacrolimus, calcipotriol, CS, imiquimod)
2) Phototherapy
117
Moderate to severe LS
Systemic: MTX, CS
118
T/F CS treatment alone has been found to have sustained benefit for LS patients
F
119
Treatment of EF
1) Oral and IV CS
2) MTX for those who dont respond to CS or are steroid dependent
120
MCC of mortality in LS
Deaths are extremely rare! MC pansclerotic type and due to chronic skin ulcer complications (sepsis, Squamous cell CA)
121
Major cause of morbidity in LS
MSK problems
