Morphea (localized scleroderma) Flashcards
What proportion of patients with morphea presents in childhood?
2/3rds
M:F ratio of Morphea
1:2.6
What are the 4 types of morphea?
- plaque type
- linear
- generalized
- morphea profunda
What are the frequencies of the 4 types of morphea?
plaque type >50% (most common subtype in adults)
linear 20% (most common subtype in children)
- generalized 13%
- morphea profunda 11%
What is the pathogenesis of Morphea?
- thought to be autoinflammatory (genetic predisposition + environemental trigger)
—-> vascular injuury—>inflammation—> profibrotic cytokines (TGF-B, IL-4, IL-6—> fibroblast and collagen deposition
What are the associated triggers with Morphea?
- trauma
- radiation
- medications
- Borrelia spp. (europe and japan mainly a/w Borrelia afzelii and B. garinii)
Which organism is an associated trigger of Morphea?
Borrelia (B. afzelii and B. garinii mainly in europe and Japan)
How does plaque Morphea present?
- 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
How does guttate morphea present?
multiple small chalk white, flat or slightly depressed macules
- appears similar to guttate LS, but lacks follicular plugging and epidermal atrophy
How does linear morphea present?
- a/w significant morbidity
- morphology similar to plaque type, but linear distribution often following blaschko’s lines
most common site of linear morphea?
lower extremities
What antibodies are commonly present in linear morphea?
Anti-ssDNA autoantibodies
What are complications of linear morhpea?
undergrowth of limbs (permanent!)
- deformities
- joint restriction/contractures
What are the two head/neck subtypes of linear Morphea?
- en coup de sabre
- Parry-Romberg syndrome (aka progressive hemifacial atrophy)
Coup de sabre form of Morphea:
Parry-Romberg syndrome presents as:
- 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
What are the possible associated findings of Parry-Romberg syndrome besides the hemifacial atrophy?
epilepsy,
- exophtalmos,
- trigeminal neuralgia,
- myopathy of eye muscles,
- cerebral atrophy
- alopecia
Children with head and neck morphea should have regular _____ appointments
- Ophthalmologic, to monitor for asymptomatic ocular inovlvement.
Atrophoderma of Pasinin and Pierini is a form of _____ atrophy
dermal atrophy
Atrophoderma of Pasinin and Pierini presents as:
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”)
Linear atrophoderma of Moulin is a variant of _______ and presents as:
- variant of Atrophoderma of Pasinin and Pierini
- presents very similarly, but in linear distribution following blaschko’s lines
Generalized morphea presents as:
- Widespread indurated plaques that expand to involve the entire trunk and extremities–> muscle atrophy and difficulty breathing (due to constrictive taut skin on chest)
Which form of morphea is most likely to have systemic symptoms (joints pains, fatigue, malaise)?
generalized morphea
Bullous morphea occurs due to:
- diffuse sclerosis of skin—> impaired lymphatic flow–> blisters
Morphea Profunda presents as:
- 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
Histology of morphea, Early? Late?
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
In contast to Systemic scleroderma, all forms of morphea lack _____ antibodies
lack anti-Scl70 and anti-centromere
All forms of morphea have + _____ autoantibodies.
Anti-topoisomerase II
Which forms of morphea often have + anti-ssDNA and + anti-histone autoantibodies, and are more likely to be ANA +?
linear and generalized
Treatments for localized or superficial morphea? More severe/deep morphea?
- for localized and superficial: topical corticosteroids, TCIs, calcipotriene, ILK injections
- for more severe disease MTX often in combination with systemic steroids is first line
First line tx for mod-severe morphea:
MTX often along with systemic steroids
Prognosis of superficial plaque type morphea? Generalized morphea?
supericial: self-limited and softens over 3-5 years
- generalized has worse prognosis
Why do you need to treat childhood linear morphea early and aggressively?
prevents limb shortening and joint contractures
