Morphea (localized scleroderma)

Question 1

What proportion of patients with morphea presents in childhood?

Answer 1

2/3rds

Question 2

M:F ratio of Morphea

Answer 2

1:2.6

Question 3

What are the 4 types of morphea?

Answer 3

• plaque type
• linear
• generalized
• morphea profunda

Question 4

What are the frequencies of the 4 types of morphea?

Answer 4

plaque type >50% (most common subtype in adults)

linear 20% (most common subtype in children)

• generalized 13%
• morphea profunda 11%

Question 5

What is the pathogenesis of Morphea?

Answer 5

• thought to be autoinflammatory (genetic predisposition + environemental trigger)

—-> vascular injuury—>inflammation—> profibrotic cytokines (TGF-B, IL-4, IL-6—> fibroblast and collagen deposition

Question 6

What are the associated triggers with Morphea?

Answer 6

• trauma
• radiation
• medications
• Borrelia spp. (europe and japan mainly a/w Borrelia afzelii and B. garinii)

Question 7

Which organism is an associated trigger of Morphea?

Answer 7

Borrelia (B. afzelii and B. garinii mainly in europe and Japan)

Question 8

How does plaque Morphea present?

Answer 8

• begins as erythematous to violacoues patches on trunk and proximal extremities
• evolves into indurated hyperpigmented or ivory plaques
• plaques are often hairless and anhidrotic with prominent follicular orifices
• may have surrounding lilac-violaceous inflammatory rim which indicates active disease

Question 9

How does guttate morphea present?

Answer 9

multiple small chalk white, flat or slightly depressed macules

• appears similar to guttate LS, but lacks follicular plugging and epidermal atrophy

Question 10

How does linear morphea present?

Answer 10

• a/w significant morbidity
• morphology similar to plaque type, but linear distribution often following blaschko’s lines

Question 11

most common site of linear morphea?

Answer 11

lower extremities

Question 12

What antibodies are commonly present in linear morphea?

Answer 12

Anti-ssDNA autoantibodies

Question 13

What are complications of linear morhpea?

Answer 13

undergrowth of limbs (permanent!)

- deformities

• joint restriction/contractures

Question 14

What are the two head/neck subtypes of linear Morphea?

Answer 14

- en coup de sabre

- Parry-Romberg syndrome (aka progressive hemifacial atrophy)

Question 15

Coup de sabre form of Morphea:

Answer 15

Question 16

Parry-Romberg syndrome presents as:

Answer 16

• aka progressive hemifacial atrophy
• unilateral atrophy of face involving dermis, subQ, muscle and bone
• may have associated epilepsy, exophtalmos, trigeminal neuralgia, myopathy of eye muscles, cerebral atrophy, or alopecia

Question 17

What are the possible associated findings of Parry-Romberg syndrome besides the hemifacial atrophy?

Answer 17

epilepsy,

• exophtalmos,
• trigeminal neuralgia,
• myopathy of eye muscles,
• cerebral atrophy
• alopecia

Question 18

Children with head and neck morphea should have regular _____ appointments

Answer 18

• Ophthalmologic, to monitor for asymptomatic ocular inovlvement.

Question 19

Atrophoderma of Pasinin and Pierini is a form of _____ atrophy

Answer 19

dermal atrophy

Question 20

Atrophoderma of Pasinin and Pierini presents as:

Answer 20

large brownish-gray hyperpigmented oval, atrophic, well-demarcated plaques w/ sharp sloping border (biopsy should contain affected skin and adjacent normal skin to show “cliff drop”)

Question 21

Linear atrophoderma of Moulin is a variant of _______ and presents as:

Answer 21

• variant of Atrophoderma of Pasinin and Pierini
• presents very similarly, but in linear distribution following blaschko’s lines

Question 22

Generalized morphea presents as:

Answer 22

• Widespread indurated plaques that expand to involve the entire trunk and extremities–> muscle atrophy and difficulty breathing (due to constrictive taut skin on chest)

Question 23

Which form of morphea is most likely to have systemic symptoms (joints pains, fatigue, malaise)?

Answer 23

generalized morphea

Question 24

Bullous morphea occurs due to:

Answer 24

• diffuse sclerosis of skin—> impaired lymphatic flow–> blisters

Question 25

Morphea Profunda presents as:

Answer 25

- morphea primarily involving the subQ tissues (**fascia, muscle and bone**) - presents with **atrophic muscles, joint contractures, and non-healing ulcers** **-** overlying skin can appear normal, puckered (**pseudo-cellulite)** or hyperpigmented

Question 26

Histology of morphea, Early? Late?

Answer 26

Early: **lymphocytic infiltrate w/ plasma cells at dermal SQ junction** **- loss of CD34+ dendritic cells** Later: - decreased inflammation - **"square biopsy sign"** **- trapped eccrine glands** (surrounded by sclerotic collagen) - pale and edematous papillary dermis - sharp demarcation between dermis and SQ

Question 27

In contast to Systemic scleroderma, all forms of morphea lack _____ antibodies

Answer 27

**lack anti-Scl70 and anti-centromere**

Question 28

All forms of morphea have + _____ autoantibodies.

Answer 28

**Anti-topoisomerase II**

Question 29

Which forms of morphea often have + anti-ssDNA and + anti-histone autoantibodies, and are more likely to be ANA +?

Answer 29

**linear and generalized**

Question 30

Treatments for localized or superficial morphea? More severe/deep morphea?

Answer 30

- for localized and superficial: **topical corticosteroids,** TCIs, calcipotriene, ILK injections - for more severe disease **MTX** often in combination with systemic steroids is **first line**

Question 31

First line tx for mod-severe morphea:

Answer 31

**MTX** often along with systemic steroids

Question 32

Prognosis of superficial plaque type morphea? Generalized morphea?

Answer 32

supericial: **self-limited and** softens over 3-5 years - generalized has worse prognosis

Question 33

Why do you need to treat childhood linear morphea early and aggressively?

Answer 33

**prevents limb shortening and joint contractures**