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Fitz 9th > 64 - Morphea and Lichen Sclerosus > Flashcards

64 - Morphea and Lichen Sclerosus Flashcards

1
Q

Y/N: Organ involvement in morphea is distinctly different from systemic sclerosis

A

Yes

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2
Q

Morphea is more common in (males/females)

A

Females

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3
Q

Most common pediatric subtype of morphea

A

Linear morphea

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4
Q

Formerly described as Parry-Romberg syndrome

A

En coup de sabre or

Progressive hemifacial atrophy

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5
Q

Subtypes that predominate in adults

A

Circumscribed morphea

Generalized morphea

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6
Q

Stages of cutaneous lesions in morphea

A

Inflammatory
Sclerotic
Atrophic

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7
Q

Stage of morphea:
Erythematous plaques or patches, sometimes with a reticulated appearance
Develop hypopigmented sclerotic plaques at the center, surrounded by an erythematous or violaceous border

A

Inflammatory stage

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8
Q

Stage of morphea:

Sclerosis develops centrally, has a shiny white color with surrounding hyperpigmentation

A

Sclerotic stage

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9
Q

Stage of morphea:

Sclerotic plaques softens and becomes atrophic with hypopigmentation or hyperpigmentation

A

Atrophic stage

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10
Q

The _____ stage is associated with cigarette paper wrinkling (papillary dermis), cliff drop (dermal), or deep indentions altering the contour of the affected body part (subcutis or deeper atrophy)

A

Atrophic

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11
Q

Morphea subtypes

A
  1. Circumscribed
  2. Linear
  3. Generalized
    a. Coalescent plaque
    b. Pansclerotic
    c. Mixed
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12
Q

Both linear and generalized morphea may begin with

A

Circumscribed lesions

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13
Q

Though to be a residua of plaque-type morphea

Borders have a “cliff-drop” appearance resembling “burnt-out” morphea lesions

A

Atrophoderma of Pasini and Pierini

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14
Q

Generalized morphea is characterized by more than or equal to _____ lesions on at least _____ of 7 different anatomic sites

A

4

2

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15
Q

3 variants of generalized morphea

A
  1. Isomorphic
  2. Symmetric
  3. Pansclerotic
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16
Q

In contrast to systemic sclerosis, generalized morphea does not present with

A

Acrosclerosis or sclerodactyly

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17
Q

Linear morphea usually affects the

A

Extremities

Face

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18
Q

May present as an atrophic linear plaque on the forehead extending to the scalp (where cicatricial alopecia occurs), brow, nose, and lip

A

En coup de sabre (“cut of the sword”)

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19
Q

In deep morphea, a “_____ sign” (depression) may be present at the site of tendons and ligaments

A

Groove

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20
Q

Also known as Shulman syndrome
Presents with rapid onset of symmetric areas of pain and poorly circumscribed indurated, plaques, usually on the extremities

A

Eosinophilic fasciitis

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21
Q

Most common extracutaneous finding in morphea patients

A

Musculoskeletal involvement

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22
Q

Complications associated with en coup de sabre

A

Neurologic

Ocular

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23
Q

_____ morphea is associated with an increased risk of squamous cell carcinoma

A

Pansclerotic

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24
Q

_____ morphea is associated with an increased rate of autoimmune disease

A

Generalized

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25
Q

The pathogenesis of morphea appears to involve a transition form a predominantly _____ profile in the early inflammatory stage of morphea to a _____ profile in the later sclerotic stage

A

Th1

Th2

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26
Q

Histologic examination may aid therapeutic decision making because it is sometimes difficult to determine the degree of activity or depth of involvement by clinical examination alone. Biopsy of the _____ of a lesion may provide insight into both

A

Advancing edge

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27
Q

ANAs occur in 34% to 80% of patients and are more common in patients with _____ disease

A

Linear or

Generalized

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28
Q

Y/N: The clinical and prognostic significance of autoantibodies in morphea remains unclear and testing for them is not indicated

A

Yes

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29
Q

Peripheral eosinophilia, hypergammaglobulinemia, and increased erythrocyte sedimentation rate or C-reactive protein may occur with active disease of any type, but particularly _____ morphea

A

Deep

30
Q

Biopsies should be taken form the _____ when present or _____

A

Inflammatory or indurated border

Sclerotic center and include subcutaneous fat

31
Q

For lesions with minimal clinical change, biopsy of _____ is helpful

A

Site-matched unaffected skin

32
Q

Becoming increasingly useful for determination of lesion activity and depth
Should be considered when deep morphea is present or suspected

A

MRI

Ultrasonography

33
Q

Characterized by acral sclerosis/sclerodactyly, nail-fold capillary changes, Raynaud phenomenon, characteristic internal organ involvement (eg, pulmonary, renal, and GI), and hallmark autoantibodies (these features that are absent in morphea)

A

Systemic sclerosis

34
Q

For a morpheaform plaque on the breast, _____ resulting from metastatic breast cancer is an important diagnosis to rule out

A

Carcinoma en cuirasse

35
Q

Morpheaform reactions can also develop secondary to

A

Vitamin K1 injections
Taxanes
IFN-beta1a
Balicatib

36
Q

A higher risk of recurrence (31% of patients) has been reported for _____ as compared to other subtypes

A

Linear morphea of the extremities

37
Q

_____ disease is most responsive to therapy

A

Early, active

38
Q

Morphea involving the superficial to mid-dermis would logically be amenable to _____; however, involvement of the deep dermis and beyond should be treated _____

A

Topical therapy or phototherapy

Systematically

39
Q

Patients with _____ (particulary those with rapid onset of confluent plaques) are likely at risk for severe, extensive disease

A

Linear

Generalized

40
Q

Phototherapy modalities with level 1 evidence

A

Broadband UVA
Narrowband UVB
UVA-1

41
Q

Narrowband UVB should be considered for lesions affecting the

A

Superficial dermis

42
Q

UVA-based therapies are more appropriate for _____ lesions

A

Deeper dermal

43
Q

Disease is expected to improve (progression halted and erythema improved) after _____ treatments and most trials stopped after _____ treatments

A

10 to 20

20 to 30

44
Q

Considered a first-line systemic treatment for morphea, especially for deep morphea and rapidly progressive or disabling morphea

A

Methotrexate

45
Q

The use of methotrexate (monotherapy) and methotrexate combined with _____ is effective based on level 1 evidence

A

Systemic corticosteroids

46
Q

Can be used as a second-line systemic treatment

Effective for morphea refractory to methotrexate or patient with contraindications or intolerance of methotrexate

A

Mycophenolate mofetil

47
Q

Level 2 evidence suggests the use of occluded topical _____ might be effective for active, inflammatory superficial plaque-type morphea

A

Tacrolimus 0.1% ointment

48
Q

Y/N: The most commonly used treatment for morphea, topical steroids, has not been investigated in a clinical trial. There are also no studies investigating the use of intralesional steroids.

A

Yes

49
Q

Vulvar lichen sclerosus has an increased risk of _____, particularly if left untreated

A

Squamous cell carcinoma

50
Q

Lichen sclerosus is more frequent in (males/females)

A

Females

51
Q

Lichen sclerosus preferentially affects women in the _____ decade of life and children younger than _____

A

Fifth or sixth decade

10 years of age

52
Q

Genital lichen sclerosus in males is almost exclusively seen in

A

Uncircumscribed men

53
Q

Vulvar lichen sclerosus present with porcelain-white atrophic papules coalescing into plaques on the

A

Labia minora and majora

54
Q

Often the classical _____ pattern of the vulva and anus may be observed

A

Figure-8

55
Q

Male genital lichen sclerosus is also known as

A

Balanitis xerotica obliterans

56
Q

Many male genital lichen sclerosus are simply diagnosed as

A

Phimosis

57
Q

Extragenital lichen sclerosus typically affects the

A

Thighs
Neck
Trunk
Lips

58
Q

Noncutaneous findings in lichen sclerosus

A 
Association with: 
Autoimmune thyroid disease
Alopecia areata
Pernicious anemia
Morphea
Vitiligo
59
Q

The lifetime risk of developing squamous cell carcinoma as a complication of longstanding LS has been estimated in the order of _____%

A

4 to 6

60
Q

Represent significant risk factors for the development of SCC in LS

A

Age
Long duration of LS
Human papillomavirus infection
Evidence of hyperplastic changes

61
Q

_____, a tumor-suppressor gene, is downregulated in vulvar squamous cell carcinoma associated with LS

A

IRF6

62
Q

Characterization of the infiltrate of LS has shown a predominance of _____ over _____ cells, and presence of regulatory T cells

A

CD8 cytotoxic T cells

CD4 T cells

63
Q

Gene expression profiling of LS has shown significant upregulation of _____ cytokines and chemokines

A

Th1 and Type 1 IFN-regulated

64
Q

Classical LS shows a/an _____ epidermis and a _____ infiltrate at the dermal-epidermal junction

A

Atrophic

Lichenoid

65
Q

Vulvar lichen planus more commonly involves the _____, whereas LS spares the ______

A

Vagina

66
Q

Common complication in children with anogenital LS

A

Constipation

67
Q

LS in children has occasionally been associated with _____, a common lesion that can be confused with condyloma and is also associated with constipation

A

Perineal pyramidal protrusion

68
Q

First-line treatment for genital LS

A

Ultrapotent topical corticosteroids, most commonly clobetasol propionate 0.05%

69
Q

(Ointments/Creams) tend to be less irritating and better tolerated than (ointments/creams)

A

Ointments

Creams

70
Q

Will generally resolve male genital LS and the associated phimosis

A

Circumcision

Fitz 9th (108 decks)

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