Dr.Tarik Enaairi - Morphea and Lichen Sclerosus.ppsx

Dermatology

1. Morpheaand Lichen Sclerosus
By : Dr. TarikS. Enaairi
NCI \MISURATA
DERMATOLOG DEPARTMENT
May\7 \2024

2. In morphea, there is inflammation of the dermisthat
may extend into subcutaneousstructures.
Lichen sclerosusis primarily a disease of the genital
mucosa (and less often extragenitalsites) that affects
the epidermis and the dermis.
Morpheamay lead to joint contractures and atrophy
of underlying muscle, but there is no involvement of
internal organs. The exceptions are the ocular and
neurologic symptoms seen in a small minority of
patients with linear morpheaof the head or
Parry–Romberg syndrome.
Scar-like sclerosis

3. MORPHEA
Synonyms/subtypes:
Localized scleroderma
Circumscribed scleroderma
Plaque-type morphea
Linear morphea–linear scleroderma
Morpheaen coup de sabre –sclerodermaen
coup de sabre
Deep morphea–morpheaprofunda

4. Asymmetric sclerotic plaques, usually 2–15 cm in diameter.
Active lesions can have a lilac border with central hypo-or
dyspigmentation, while inactive lesions often become
hyperpigmented.
The sclerosis may extend deeply into the fat or underlying
structures (e.g. fascia, muscle, bone), causing disability.
Often progresses for several years, then regresses; the linear
subtype is usually persistent.

5. INTRODUCTION
The small vessel changes, inflammatory
infiltrate, structural modifications are identical in
morpheaand systemic sclerosis (SSc), but the
two diseases are distinct clinically.
Distinctbetween deep morpheaand eosinophilic
fasciitis are more difficult.

6. •Morpheahas an asymmetric patchy or linear
distribution.
•Symmetric tightening of the fingers and hands that
extends progressively toward the forearms
(limited cutaneous SSc[lcSSc]).
•Symmetrically extending sclerosis of the trunk and
proximal upper extremities
(diffuse cutaneous SSc[dcSSc]).
•Rarely, generalized morpheamay resemble early
dcSSc.
•In SSc:Raynaud phenomenon, digital sclerosis, and
involvement of the GIT and the lung.

7. EPIDEMIOLOGY
In a survey conducted Minnesota from 1960 to 1993.
During this period, the annual incidencerate was 27 per
million.
The prevalenceincreases with age, occurring in ~500 and
2200 per million at ages 18 and 80 years, respectively.
According to this study, 56% plaque-type, 20% linear, 13%
generalized, and 11% deep morphea.
women : men (2.6 : 1), with the exception of linear
morphea, which has no sex preference.

8. PATHOGENESIS
Autoantibodies are usually as prevalent in morpheaas
in the general population, with two exceptions:
(1)an increased prevalence of (ssDNA), topoisomerase IIα,
phospholipid, fibrillin-1, and (AHA) in patients with
morphea.
(2) high titers of anti-nuclear antibodies (ANA) in juvenile
patients with linear morpheaand individuals with
generalized morphea.

9. In patients with linear morphea, the presence of
ssDNAantibodies or AHAseems to be associated
with increased risk of functional impairment.
Sclerosis of the skin involve three major, closely
connected components:
vascular damage, activated T cells, and altered
connective tissue production by fibroblasts

11. The local triggering events for morpheainclude
mechanical trauma, injections, vaccinations, and
irradiation.
In one investigation was the potential role of infection
with Borreliaburgdorferi.
However, larger studies failed to confirm this initial
observation.

13. Plaque-Type (Circumscribed) Morphea
The most frequent variant.
It is generally asymptomatic.
Insidious onset of a slightly elevated, erythematous or
violaceousplaque, that undergoes centrifugal expansion.
Depending on the depth of the sclerosis, the skin becomes
progressively indurated.
Centrally, shiny white color and peripherally, a violaceous
or “lilac” ring.

14. The plaques of morpheamost commonly develop on the
trunk and in general are between 2 and 15 cm in diameter.
They are usually multiple and asymmetric.
The course of morpheais also variable. In most patients,
morpheaprogresses over 3–5 years, then arrests and
resolves spontaneously.
However, residual atrophy and dyspigmentationare
commonly observed.

15. Plaque-type (circumscribed) morphea.
Sclerotic plaques with the characteristic lilac-colored rim.

16. Large, welldemarcated, hyperpigmentedplaque that
is depressed centrally; the trunk is a common
location for plaque-type morphea.

17. VARIANTS
●Guttatemorpheapresents primarily as multiple.
Even though relatively small, they may become deeply
indurated.
●Atrophodermaof Pasiniand Pieriniis considered by
some to be a very superficial variant of plaque-type
morphea, while others consider it to be a separate entity.
Hyperpigmentedpatches are seen most commonly on the
posterior trunk; occasionally, lesions follow the lines of
Blaschko(linear atrophodermaof Moulin).

18. ●Deep morpheainflammation and sclerosis that affects
primarily the deep dermis and subcutaneous fat and may
involve underlying structures (e.g. the fascia).
Patients normally develop a small number of hard deep
plaques, lesions may calcify, leading to dystrophic
calcinosiscutis.
Because of the location of the sclerosis, individual lesions
may resemble eosinophilicfasciitis.

19. Deep morphea Eosinophilicfasciitis

20. ●In nodular or keloidalmorphea, inflammation within
the dermis leads to thick, keloid-like nodules or bands.
●Bullous morpheaIn rare patients, especially those
where sclerosis of the skin is associated with diffuse,
rapidly progressive edema, lymphocelesmay result from
stasis of lymphatic fluid.
It is more frequently observed in generalized morphea
and sclerodermoidor morpheaformGVHDand is
rarely a feature of plaque type morphea.

21. Nodular or keloidalmorphea.
Multiple firm, hyperpigmentednodules mimicking
keloids in a 10-year-old girl.

22. Linear Morphea& Parry–Romberg Syndrome
Linear morpheais different from plaque-type morphea, inage
of onset, distribution, clinical outcome, and serologies.
Morpheaen coup de sabre :
(meaning blow with a sword) is a term used for linear
morpheaof the forehead and scalp.
Parry–Romberg syndrome or Hemifacialatrophy:
Very severe variant of linear morphea.
There is a progressive loss of subcutaneous fat, but little or
no sclerosis.

23. Morpheaen coup de sabre.
The linear band(s) of sclerosis and atrophy are usually paramedian
(rather than midline).

24. Parry–Romberg syndrome. Unilateral sclerosis and
loss of subcutaneous fat, leading to facial asymmetry.

25. Linear morpheatends to involve the underlying fascia,
muscle, and tendons.
This leads not only to muscle weakness but also to
shortening of the muscles which then impairs joint
mobility.

26. The en coup de sabre type is typically unilateral and
extends from the forehead into the frontal scalp and
may reach the eyebrows, nose and even the cheeks.
It may start either as a linear streak, which may initially
mimic a port-wine birthmark, or as a row of small
plaques that coalesce.

27. En coup de sabre morpheacan also involve the
underlying muscles and bone.
Rarely progress to involve the meninges and even the
brain.
Ocular involvement has been reported in up to 15%of
juvenile and 25%of adult patients.

28. Linear
morphea
of an
extremity.
A Linear sclerotic band of the arm with both hyper & hypopigmentation
B More inflammatory phase with ulceration in addition to induration.

31. Extensive linear morpheaof the
right leg leading to hypoplasia.
In addition to marked hypoplasia,
there is induration and a flexion
contracture of the knee.

32. Generalized Morphea
Generalized morpheanormally begins on the trunk as
plaque-type morphea.
While individual lesions are indistinguishable in these
two forms, the generalized morpheacan affect nearly
the entire trunk, often only sparing the areolae and
nipples.

33. The sclerosis may involve the extremities down to the
hands (presenting initially as edema).
As the cutaneous sclerosis progresses, it may result in
disabling constrictions that even cause difficulty in
breathing due to inflammation of the intercostal
muscles.

34. Generalized morphea:
Multiple hyperpigmented
plaques on the trunk.

35. Sparing of the central portion of the breast is a classic
distribution pattern.

36. Panscleroticmorphea: also referred to as disabling
panscleroticmorpheaof children, is similar to generalized
morpheaof the adult.
It usually starts before 14 years of age and tends to cause
lifelong severe disability due to persistent atrophy of the
underlying muscles and contractures of the involved
joints.
Periodontal atrophy may occur, but there is only slight
involvement of the esophagus and lung.
Distinction from SScmay prove challenging.

37. LABORATORY FINDINGS
Laboratory abnormalities are not a prominent feature of
morphea, except for generalized and linear morphea.
ESR and serum protein levels are usually normal, but
eosinophilia may occur, especially during early active
phases of the disease.
High titers of ANA or antibodies to ssDNAand
histones can be detected in 40%–80% of patients with
linear and generalized morphea.

38. PATHOLOGY
The histopathology of morpheadepends on:
the stage and area of the lesion sampled (inflammatory
margin or central sclerosis) and the depth to which the
disease extends.
In most situations, morphologic changes are best
seen at the border between the dermis and subcutaneous
fat.
Specimens for histopathology must include
subcutaneous fat

39. At 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
In later stages, the inflammatory infiltrate disappears,
except in some areas of the subcutaneous fat.
The epidermis is basically normal in appearance, but the
rete ridges may be diminished, leaving a flattened
dermal–epidermal junction.

40. Homogeneous collagen bundles with decreased space
between the bundles replace most structures.
At this stage, collagen bundles in the reticular dermis
appear densely packed, and parallel to the DE junction.
Eccrineglands appear atrophic and “trapped” within the
thickened dermis.

41. Deep morpheaaffects primarily the deep subcutaneous
tissue.
Following the inflammatory phase, extensive sclerosis
extend into the underlying fascia.
Involvement of the underlying fascia in deep morphea
and is also frequently observed in linear and generalized
types of morphea.

42. Thickening of the dermis
and perivascular and
perieccrineinfiltrates of
lymphocytes and plasma
cells (inset).
These changes can be seen
in the inflammatory border.

43. Advanced sclerosis of the
entire dermis extending
into the fat with thickened
collagen bundles and
“trapped” eccrineglands
This corresponds to the
areas of induration within
the plaque.

44. Morpheaform& Sclerodermoid)
Inflammatory Syndromes
Some disorders that are characterized by acrosclerosisand
Raynaud phenomenon have a clinical presentation similar
to lcSSc(i.e. sclerodermoid).
While others present with circumscribed plaques similar to
morphea(i.e. morpheaform).

45. ●Lipodermatosclerosis. Chronic venous insufficiency,
along with chronic hypoxia, leads to sclerosis, most
commonly on the lower extremities.
●Injections of vitamin K1 (Texierdisease). Rarely,
injection of oil-soluble vitamin K1 causes a strictly
localized eosinophilicfasciitis that is indistinguishable
from deep morphea.

46. ●Vaccination-associated morphea.
●Paraffin and silicone injections or implants.
●Porphyrias.
●Nephrogenicsystemic fibrosis.
●Chronic GVHD.
●Radiation-induced morphea.
.

47. ●Radiation-induced morphea.
This disorder is characterized by marked sclerosis,
erythema, and pigmentarychanges occurring within the
radiation field or even beyond it.
The incidence is about 1 in 500 patients and it is seen
primarily in patients treated for breast carcinoma.
It can present several years after radiation therapy.

48. TREATMENT
The major therapies for morpheainclude topical
medications, phototherapy, systemic immunosuppressants.
Treatment is most effective in active, inflammatory lesions.

52. LICHEN SCLEROSUS
Synonyms/site-specific names:
Lichen sclerosuset atrophicus
Kraurosisvulvae
Balanitisxeroticaobliterans

53. INTRODUCTION
Sclerotic, ivory-white, flat papules and plaques with
epidermal atrophy and, in extramucosalsites, follicular
plugging.
„Most commonly affects female or male genitalia, less
often extragenitalskin.
„Phimosisor scarring of the vaginal are the most frequent
complications.„
Severe pruritus.
„No systemic manifestations.
If left untreated, ~4% may develop vulvar
intraepithelial neoplasia(VIN) or (SCC).

54. EPIDEMIOLOGY
Lichen sclerosusis relatively uncommon.
female-to-male ratio varies widely, depending upon the
study 10 : 1 to 1 : 1.
In both sexes, the anogenitalarea is affected in ~85% of
patients.
In women, the peak incidence is during the fifth and sixth
decades, and a second peak occurs in girls between 8 and
13 yearsof age.
ExtragenitalLS is rare in children.

55. PATHOGENESIS
Association with HLA-DQ7was observed.
IgGautoantibodies against ECM-1are found in 80% of
patients with LS.
Moreover, in female patients with LS, there is a higher
prevalence of autoimmune diseases
(especially autoimmune thyroid disease).

56. PATHOLOGY
superficial dermal edema is associated with a
band-like lymphocytic infiltrate.
The epidermis is thinned, with orthohyperkeratosisand
vacuolar degeneration of the basal layer.
Hyperkeratosis is especially pronounced at follicular
openings and may lead to plugging.
Vacuolar degeneration of the basal layer and flattening of
the rete ridges predispose to the development of blisters,
which may become hemorrhagic.

57. The most important changes are found in the superficial
dermis, where the pale staining reflects edema initially
and then homogenized dermal collagen.
Loss of elastic fibers is typical for LS and is not
observed in morphea.

58. Early lesion with band-like infiltrate of lymphocytes in the upper
dermis and vacuolar change at the dermal–epidermal junction.

59. Late lesion: orthohyperkeratosis, thinning of the epidermissclerosis
of the papillary dermis.

60. DIFFERENTIAL DIAGNOSIS
The most important DD of extragenitalLS is morphea,
and in the case of genital LSis sexual abuse.
However, morpheaand LS may coexist in the same
patient.
Inadults, genital LS may mimic erosive lichen planus,
mucous membrane pemphigoid.

61. TREATMENT
General
Regular use of emollients
Circumcision for phimosis
Regular (at least yearly)
Examination for SCC
First-line
Superpotenttopical CS
Topical calcineurininhibitors
Second-line
Intralesional CS
Oral retinoids
Methotrexate
Hydroxychloroquine
JAK inhibitors
UVA1
Photodynamic therapy
Fractional CO2 laser

62. THANKS