10 - 64 - MORPHEA AND LICHEN SCLEROSUS

Question 1

What subtype predominates in children

Answer 1

Linear Morphea

Question 2

What subtype predominates in aduts?

Answer 2

Circumscribed and generalized

Question 3

how do you differentiate morphea from scleroderma

Answer 3

lack of acrosclerosis / sclerodactyly

Question 4

What are the stages of morphea

Answer 4

1. Inflammatory stage
   - Morphea begins as erythematous plaques or patches, sometimes with a reticulated appearance. Later, hypopigmented sclerotic plaques develop at the center of the lesion, surrounded by an erythematous or violaceous border.
2. Sclerotic stage
   - Sclerosis develops centrally, has a shiny white color with surrounding hyperpigmentation
3. Atrophic stage
   - Over months to years, the sclerotic plaque softens and becomes atrophic with hypopigmentation or hyperpigmentation

Question 5

This 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 5

Atrophic stage

Question 6

presents as oval to round lesions that are of limited cutaneous distribution so do not meet criteria for generalized disease

Answer 6

Circumscribed Morphea

Question 7

Generalized morphea is characterized by more than or equal to how many lesions on how many anatomic sites?

Answer 7

more than or equal to 4 lesions on at least 2 of 7 different anatomic sites

Question 8

3 variants of genaralized morphea

Answer 8

(a) isomorphic,
(b) symmetric, and
(c) pansclerotic

Question 9

where do lesions of generalized morphea begin?

Answer 9

lesions frequently begin on the trunk and spread acrally, sparing the fingers and toes

Question 10

Linear morphea usually affects what areas

Answer 10

extremities and face, but it can occur on the trunk

Question 11

Classification of Morphea Subtypes

Answer 11

Question 12

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

Answer 12

En coup de sabre (“cut of the sword”)

Question 13

involves the deep dermis, subcutaneous tissue, fascia, and muscle

The skin feels thickened and bound down to the underlying fascia and muscle.

Answer 13

Deep morphea, or morphea profunda

Question 14

5 subtypes of morphea

Answer 14

1. Circumscribed Morphea
2. GeneralizedMorphea
3. Linear Morphea
4. Deep Morphea
5. Mixed Morphea

Question 15

most common extracutaneous manifestation of morphea

Answer 15

Musculoskeletal involvement: arthritis, myalgias, neuropathies, and carpal tunnel syndrome

Question 17

En coup de sabre is associated with what complications

Answer 17

neurologic and ocular complications (3.6%) including seizures, headaches, adnexal abnormalities (eyelids, eyelashes), uveitis, and episcleritis

Question 18

this type of morphea is associated with an increased risk of squamous cell carcinoma caused by chronic ulcers

Answer 18

pansclerotic morphea

Question 19

What HLA confer an increased risk for morphea

Answer 19

HLADRB1 ∗ 04:04 and HLA-B37

Question 20

only in the subset of linear morphea, __________ antibodies were associated with functional limitation

Answer 20

antihistone

Question 21

only in the subset of linear morphea, ______ was associated with extensive body surface area involvement

Answer 21

ANA

Question 22

Where should biopsy be taken in morphea?

Answer 22

inflammatory or indurated border when present or sclerotic center and include subcutaneous fat

Question 23

Histopath findings of Inflammatory phase of morphea

Answer 23

• interstitial and perivascular inflammatory cell infiltrate in the dermis and sometimes subcutaneous tissue, composed mostly of lymphocytes and plasma cells,
• eosinophils, mast cells, and macrophages also may be present
• tissue edema, enlarged tortuous vessels, and thickened collagen bundles

Question 25

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

Answer 25

linear morphea of the extremities

Question 26

define Therapeutic success in morphea management

Answer 26

Therapeutic success is defined by resolution of erythema, typically over 2 to 3 months, lesion softening, which can take 12 months or more, cessation of lesion growth, and no new lesion development.

Question 27

T/F.
In general, active lesions are most amenable to treatment.

Answer 27

True

Question 28

morphea-specific outcome measure

Answer 28

localized scleroderma cutaneous assessment tool (LoSCAT)

consists of 2 scores: LoSSI (localized scleroderma severity index) and LoSDI (localized scleroderma damage index)

Question 29

In determining which therapy is appropriate for morphea, what factors must be considered?

Answer 29

■ Disease activity and damage
■ Depth of involvement
■ Disease progression
■ Systemic involvement
■ Disease subtype

Question 30

Indicators of active morphea disease

Answer 30

• development of new lesions or extension of existing lesions (photographs are critical),
• erythema and/or induration of the advancing edge of the lesion, and
• patient-reported symptoms, such as itch or tingling

Question 31

what are examples of disease damage in morphea

Answer 31

• (reversible or irreversible) includes pigmentary change,
• sclerosis of the lesion center,
• atrophy (dermal, subcutaneous, muscle),
• contracture,
• limb-length discrepancy, and
• scarring alopecia

Question 32

Question 33

Question 34

in morphea, define superficial involvement

Answer 34

defined by histologic evidence of papillary dermal involvement

Question 35

in morphea, define deep involvement

Answer 35

defined as sclerosis or inflammation of the deep dermis, subcutis, fascia, or muscle

Question 36

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

Answer 36

Methotrexate

Question 37

Vulvar lichen sclerosus is associated with an increased risk of

Answer 37

SCC

Question 38

Lichen sclerosus is predominant in what age group and gender

Answer 38

5th to 6th decade of life and children younger than 10 years

F>M (5:1)