Morphea Flashcards
What are the clinical subtypes of Morphea?
There are multiple classification systems.
**Plaque type **is the most common
Clinical variants:
- Guttate
- Deep
- Atrophoderma of Pasini and Pierini
- Nodular or Keloidal
- Bullous
**Linear Morphea **
- en coup de sabre
- Parry Romberg syndrome
**Generalised Morphea **
What is Parry Romberg Syndrome?
Classified as a variant of linear morphea
Hemifacial atrophy due to loss of subcutaneous fat
- can affect teh entire distribution of the trigmeninal nerve (including the tongue and the eye)
What is the name of this condition? What is the significance?
En Coup De Sabre (Linear morphea)
Can involve the underlying muscles, osseous structures Rarely the menignes and brain are invovled - causing seizures
Ocular involvement occurs in 15 of children and 25% of adults.
What is linear Morphea?
A subtype of morphea:
- different from plaque-type morphea, with respect to age of onset, distribution, clinical outcome, and serologies.
- may present initially as a linear, erythematous
(inflammatory) streak, but more frequently begins as a harmless-appearing lesion of plaque-type morphea that extends longitudinally as a series of plaques that then join to form a scar-like band
tends to involve the underlying fascia, muscle, and tendons
Causes:
- muscle weakness
- shortening of the muscles and fascia
- impairs joint mobility
- functional impairment
What is generalised Morphea?
A subtype of morphea.
Begins insidiously on the trunk as plaque-type morphea
While individual lesions are indistinguishable in
these two forms, in generalized morphea they do not stop expanding.
plaques rapidly coalesce and can affect nearly the entire trunk, often only sparing the areolae and nipples
Can result in disabling constrictions
What condition is this?
Parry Romberg syndrome
(linear morphea)
What condition is this?
Linear morphea of the right leg leading to hypoplasia, likely significant impact on joint mobility and function
Describe and DDx?
Large white, shiny plaque on the chest
Peripheral “lilac” border
Appears bound down
Sparing of the areolar
Classic distrubition pattern of Generalised Morphea
What investigations do you need to do for this child?
MRI head / brain
Opathmology referral
Does morphea affect children?
Yes, 20% of patients with morphea are children / teenagers
What is the mean age of onset of morphea in childhood?
7 years old
Children who develop linear morphea are at risk of growth retardation, limited range of motion and permanent limb assymetry and hypoplasia
True
What is pansclerotic morphea?
Similar to generalise morphea
Affects children, usually starts before 14 years of age
Can cause lifelong severe disability due to persistent atrophy of the underlying muscles and contractures of the involved joints.
Starts on the trunk and extends to the hands and feets
What are the extracutaneous findings occur children with morphea? (provide the incidence of these)
11% articular
4% neurologic
2% ocular
Of the latter two - 85% had en coup de sabre of parry romberg syndrome
What lab findings might you see in a patient with generalised or linear morphea?
High titers of ANA or antibodies to ssDNA and histones can be detected in 40%–80% of patients with linear and generalized morphea.
Note this is UNCOMMON in patients with plaque type morphea
True or false
80% of children with morphea have elevated ANA
False - ~ 40%
How do you take a biopsy for morphea?
Ideally at the active edge of a plaque
The sample must extend to include subcutanoues
3 or 4 mm punch biopsy to subcut fat.
What are the expected histofindings for a patient with plaque morphea?
Biopsy from the inflammatory border:
- vascular changes are relatively discrete by light microscopy
- Vessel walls show endothelial swelling and edema.
- Capillaries and small arterioles are surrounded by an infiltrate that contains primarily CD4+ T cells and sometimes eosinophils, plasma cells, and mast cells
- The epidermis is basically normal in appearance, but the rete ridges may be diminished,
Biopsy from the sclerotic centre:
- inflammatory infiltrate wanes and ultimately disappears completely
- Edema is no longer visible in the dermis and upper subcutis.
- Capillaries and small vessels are significantly reduced in number
- homogeneous collagen bundles with decreased space between the bundles replace most structures.
What are the expected histo findings of deep morphea?
Primarily affects the deep subcut tissue
Following the inflammatory phase, extensive sclerosis and hyalinization extend into the underlying fascia
Involvement of the underlying fascia is obligatory in deep morphea and is also frequently observed in linear and generalized types of morphea.
Describe?
A. Overview with thickening of the dermis and perivascular and perieccrine infiltrates of lymphocytes and plasma cells (inset). These changes can be seen in the inflammatory border.
B Advanced sclerosis of the entire dermis extending into the fat with thickened collagen bundles and “trapped” eccrine glands (arrow). This corresponds to the areas of induration within the plaque.
List the morphaeform inflammatory syndromes
- Lipodermatosclerosis
- Injections of vitamin K1 (causes a strictly localized eosinophilic fasciitis that is indistinguishable from deep morphea)
- Vaccination-associated morphea
- Paraffin and silicone injections or implants causing morphea
- Porphyrias (porphyria cutanea tarda can lead to morphea-like sclerosis in UV-exposed sites)
- Radiation-induced morphea.
- Nephrogenic systemic fibrosis.
- Chronic GVHD
Provide a DDX for morphea
Systemic Sclerosus
- key differentiating factors: Asymmetric skin involvement as well as the absence of Raynaud phenomenon and lung or esophageal involvement
Chronic GVHD
Lichen Sclerosus
Lipodermatosclerosus
Eosinophillic fascilitis
Keloids
Dupuytren contracture and camptodactyly
Scleradema
How do you manage Morphea?
Patient education
Topical therapy:
- topical cortisocsteroids BD
- intralesional corticosteroids (every 4 - 6 weeks to the acitve rim)
- tacrolimus ointment 0.1 % BD
- crisaborole
Phototherapy:
- UVA 1 (phototherapy of choice)
- UVA
- PUVA
- nbUVB (has a shallow penetration, so prefecence is for UVA)
(NB: phototherapy + oral retinoid combination may be beneficical)
Systemic medications:
- systmic corticosteroids
- Methotrexate
- MMF
- JAK inhibitors (tofacitinib, baricitinib)
- hydroxychloroquine
- systemic retionoids
- tocalizumab
- ?dupilimab (under trials at the moment)
Adjuncts:
- referral to physiotherapy
- referral to occupational therapy
- reconstructive surgery
True or false
nbUVB is the phototherapy of choice for morphea
False - UVA1, followed by BB-UVA and PUVA
UVB does not penetrate as deep and not as good for morphea
True of false
Morphea is recalcitrant to treatment and most people do not respond to treatment.
False - the majority (>60%) respond to phototherapy
List the triggering events for morphea
Local injury
- mechanical trauma
- vaccination
- radiation
What are the key pathogenic changes that occur in morphea?
1/ Vascular damage
- microvessels = capillaries and small arterioles
- endothelial cell damage, activation, swelling
- release of vascular endothelial growth factor, platelet derived growth factor and transforming growth factor
2/ Activation of TH2 cells
- mostly CD4+
- cytokine release IL4, 13, 17A, TGFb
3/ Stimulation of fibroblasts
- leading to fibrosis
Who does morphea affect?
F > M
Adults and children, prevalence increases with age
True or false
~20% of morphea occurs in children and teenagers
True
25% of patients have singificant functional impairment from morphea
False - 11%
