64 - Morphea and Lichen Sclerosus

Question 1

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

Answer 1

Yes

Question 2

Morphea is more common in (males/females)

Answer 2

Females

Question 3

Most common pediatric subtype of morphea

Answer 3

Linear morphea

Question 4

Formerly described as Parry-Romberg syndrome

Answer 4

En coup de sabre or

Progressive hemifacial atrophy

Question 5

Subtypes that predominate in adults

Answer 5

Circumscribed morphea

Generalized morphea

Question 6

Stages of cutaneous lesions in morphea

Answer 6

Inflammatory
Sclerotic
Atrophic

Question 7

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

Answer 7

Inflammatory stage

Question 8

Stage of morphea:

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

Answer 8

Sclerotic stage

Question 9

Stage of morphea:

Sclerotic plaques softens and becomes atrophic with hypopigmentation or hyperpigmentation

Answer 9

Atrophic stage

Question 10

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)

Answer 10

Atrophic

Question 11

Morphea subtypes

Answer 11

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

Question 12

Both linear and generalized morphea may begin with

Answer 12

Circumscribed lesions

Question 13

Though to be a residua of plaque-type morphea

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

Answer 13

Atrophoderma of Pasini and Pierini

Question 14

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

Answer 14

4

2

Question 15

3 variants of generalized morphea

Answer 15

1. Isomorphic
2. Symmetric
3. Pansclerotic

Question 16

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

Answer 16

Acrosclerosis or sclerodactyly

Question 17

Linear morphea usually affects the

Answer 17

Extremities

Face

Question 18

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

Answer 18

En coup de sabre (“cut of the sword”)

Question 19

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

Answer 19

Groove

Question 20

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

Answer 20

Eosinophilic fasciitis

Question 21

Most common extracutaneous finding in morphea patients

Answer 21

Musculoskeletal involvement

Question 22

Complications associated with en coup de sabre

Answer 22

Neurologic

Ocular

Question 23

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

Answer 23

Pansclerotic

Question 24

_____ morphea is associated with an increased rate of autoimmune disease

Answer 24

Generalized

Question 25

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

Answer 25

Th1 | Th2

Question 26

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

Answer 26

Advancing edge

Question 27

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

Answer 27

Linear or | Generalized

Question 28

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

Answer 28

Yes

Question 29

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

Answer 29

Deep

Question 30

Biopsies should be taken form the _____ when present or _____

Answer 30

Inflammatory or indurated border | Sclerotic center and include subcutaneous fat

Question 31

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

Answer 31

Site-matched unaffected skin

Question 32

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

Answer 32

MRI | Ultrasonography

Question 33

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)

Answer 33

Systemic sclerosis

Question 34

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

Answer 34

Carcinoma en cuirasse

Question 35

Morpheaform reactions can also develop secondary to

Answer 35

Vitamin K1 injections Taxanes IFN-beta1a Balicatib

Question 36

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

Answer 36

Linear morphea of the extremities

Question 37

_____ disease is most responsive to therapy

Answer 37

Early, active

Question 38

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

Answer 38

Topical therapy or phototherapy | Systematically

Question 39

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

Answer 39

Linear | Generalized

Question 40

Phototherapy modalities with level 1 evidence

Answer 40

Broadband UVA Narrowband UVB UVA-1

Question 41

Narrowband UVB should be considered for lesions affecting the

Answer 41

Superficial dermis

Question 42

UVA-based therapies are more appropriate for _____ lesions

Answer 42

Deeper dermal

Question 43

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

Answer 43

10 to 20 | 20 to 30

Question 44

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

Answer 44

Methotrexate

Question 45

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

Answer 45

Systemic corticosteroids

Question 46

Can be used as a second-line systemic treatment | Effective for morphea refractory to methotrexate or patient with contraindications or intolerance of methotrexate

Answer 46

Mycophenolate mofetil

Question 47

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

Answer 47

Tacrolimus 0.1% ointment

Question 48

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.

Answer 48

Yes

Question 49

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

Answer 49

Squamous cell carcinoma

Question 50

Lichen sclerosus is more frequent in (males/females)

Answer 50

Females

Question 51

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

Answer 51

Fifth or sixth decade | 10 years of age

Question 52

Genital lichen sclerosus in males is almost exclusively seen in

Answer 52

Uncircumscribed men

Question 53

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

Answer 53

Labia minora and majora

Question 54

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

Answer 54

Figure-8

Question 55

Male genital lichen sclerosus is also known as

Answer 55

Balanitis xerotica obliterans

Question 56

Many male genital lichen sclerosus are simply diagnosed as

Answer 56

Phimosis

Question 57

Extragenital lichen sclerosus typically affects the

Answer 57

Thighs Neck Trunk Lips

Question 58

Noncutaneous findings in lichen sclerosus

Answer 58

``` Association with: Autoimmune thyroid disease Alopecia areata Pernicious anemia Morphea Vitiligo ```

Question 59

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

Answer 59

4 to 6

Question 60

Represent significant risk factors for the development of SCC in LS

Answer 60

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

Question 61

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

Answer 61

IRF6

Question 62

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

Answer 62

CD8 cytotoxic T cells | CD4 T cells

Question 63

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

Answer 63

Th1 and Type 1 IFN-regulated

Question 64

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

Answer 64

Atrophic | Lichenoid

Question 65

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

Answer 65

Vagina

Question 66

Common complication in children with anogenital LS

Answer 66

Constipation

Question 67

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

Answer 67

Perineal pyramidal protrusion

Question 68

First-line treatment for genital LS

Answer 68

Ultrapotent topical corticosteroids, most commonly clobetasol propionate 0.05%

Question 69

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

Answer 69

Ointments | Creams

Question 70

Will generally resolve male genital LS and the associated phimosis

Answer 70

Circumcision