Volume 03 Issue 10-2023
18
International Journal of Medical Sciences And Clinical Research
(ISSN
–
2771-2265)
VOLUME
03
ISSUE
10
P
AGES
:
18-23
SJIF
I
MPACT
FACTOR
(2021:
5.
694
)
(2022:
5.
893
)
(2023:
6.
184
)
OCLC
–
1121105677
Publisher:
Oscar Publishing Services
Servi
ABSTRACT
Localized scleroderma (LS) is an inflammatory sclerosing disease of the skin and subcutaneous tissues associated with
their atrophy. Depending on the subtype, severity of the disease and localization of the lesion, involvement in the
pathological process of adipose tissue, muscles, joints and bones, but not internal organs, is noted. The annual primary
incidence of drugs in childhood is 3.4 cases per 1,000,000 child population; in females, the disease occurs 2.6
–
6 times
more often. The wide range of clinical manifestations of the disease has led to the emergence of a large number of
different classifications, which take into account the severity, prevalence and depth of the fibrosis process, as a result
of which five main clinical forms of LS are distinguished: limited, generalized, linear, deep and mixed. The forms of
drugs are not mutually exclusive, since the same patient may experience different manifestations of the disease. One
of the most common forms of LS in childhood is the linear form, which is observed in approximately 40
–
70% of
children.
KEYWORDS
localized scleroderma, lipofilling, maxillofacial area.
Research Article
MODERN TREATMENT AND CORRECTION OF THE CONSEQUENCES OF
LOCALIZED SCLERODERMA
Submission Date:
October 01, 2023,
Accepted Date:
October 06, 2023,
Published Date:
October 11, 2023
Crossref doi:
https://doi.org/10.37547/ijmscr/Volume03Issue10-04
I.O. Nigmatov
Researcher, Tashkent Medical Academy, Uzbekistan
Journal
Website:
https://theusajournals.
com/index.php/ijmscr
Copyright:
Original
content from this work
may be used under the
terms of the creative
commons
attributes
4.0 licence.
Volume 03 Issue 10-2023
19
International Journal of Medical Sciences And Clinical Research
(ISSN
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(2021:
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(2023:
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OCLC
–
1121105677
Publisher:
Oscar Publishing Services
Servi
INTRODUCTION
The linear form of LS is characterized by the presence
of one or more linear bands of compaction, which can
affect the skin, subcutaneous tissue, muscles and
underlying bone tissue [5]. It is usually a single,
unilateral lesion with a linear distribution, affecting the
extremities, face, or scalp. Lesions are often located
along Blaschko's lines (lines of normal development of
skin cells that are invisible under normal conditions,
but may appear as pathological rashes with a linear or
segmental distribution across the skin). When localized
on the scalp, a linear lesion appears, often atrophic and
slightly depressed, the skin is smooth, shiny,
sometimes pigmented. The linear form tends to
deform bone structures, causing depressed lesions,
and when localized on the face, it can spread to the
zygomatic and nasal areas, and the upper lip [6].
In the case of complete damage to half of the face, the
process is classified as Parry-Romberg syndrome
(progressive facial hemiatrophy) [7]. The disease has a
slow, progressive course and usually develops
between the ages of 2 and 20 years. It is characterized
by unilateral facial atrophy with damage to the skin,
subcutaneous tissue, muscles and underlying bone
structures, most often the dermatomes of one or more
branches of the trigeminal nerve are affected. Atrophy
may be preceded by cutaneous induration and
discoloration of the affected skin, such as
depigmentation or hyperpigmentation, and scarring
alopecia is sometimes observed in affected areas of
the scalp [8].
In 40% of cases, progressive facial hemiatrophy is
combined with linear scleroderma of the “saber strike”
type (en coup de sabre). Currently, many authors
combine these forms of drugs into one [9, 10]. The
course of LS of the “saber blow” type is usually slowly
progressive, and the pathological process is usually
limited to damage to one half of the face. This form
often begins with swelling of the affected area,
followed by the formation of a depressed groove in the
frontoparietal region, which can then linearly spread to
the scalp with the development of cicatricial alopecia.
The groove can reach the nose, upper lip and
sometimes the gum, which leads to pronounced
deformation of these structures; the distance between
the teeth and their direction can be changed. The
pathological process may involve the bones of the
skull, including the facial region. Jaw deformation can
lead to malocclusion, poor implantation and atrophy of
tooth roots, as well as a delay in their appearance and
development [11].
The hypothesis of the genetic origin of LS as a systemic
autoimmune disease is supported by the association of
cases with a family history of autoimmune diseases and
the presence of common HLA types with rheumatoid
arthritis. The systemic nature of the disease is also
Volume 03 Issue 10-2023
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Publisher:
Oscar Publishing Services
Servi
indicated by the presence of autoantibodies and
increased concentrations of chemokines and cytokines
associated with T-helper cells circulating in the blood [
10 ].
Along with the signs of a systemic disease, drugs are
characterized by signs of a disease caused by
inflammatory fibrosis, namely the formation of a
lymphocytic and macrophage infiltrate with the
deposition of collagen and fibroblasts [ 11 ]. Fibrosis is
associated with high concentrations of transforming
growth factor beta and interleukin 4 [13]. The tendency
to replace normal tissues during fibrosis and
destruction of adipose tissue leads to phenotypic
changes, including facial atrophy and depigmentation
[ 8 ].
Assessing the activity of a lesion in LS is crucial in
choosing therapeutic tactics. For this purpose, various
instrumental methods are used, such as infrared
thermography, magnetic resonance imaging, Doppler
flowmetry, ultrasound examination (ultrasound), as
well as multifactorial assessment systems. Among the
latter is the modified Localized Scleroderma Skin
Severity Index ( mLoSSI), which is equivalent to the
modified Rodnan Skin Score (mRSS), used for systemic
sclerosis. On a scale of 0 to 3, the Rodnan Index
evaluates erythema, skin thickening, and new lesions in
18 different anatomical sites and can be used in both
adults and children. To assess active inflammatory
lesions in LS, as well as the therapeutic effect, the
LoSDI skin damage index (Localized Scleroderma Skin
Damage Index) was developed, which evaluates
cutaneous and subcutaneous atrophy, as well as the
degree of dyspigmentation. The combination of LoSDI
and the Physician's Global Assessment (PGA) is
designated the Localized Scleroderma Cutaneous
Assessment Tool (LoSCAT), which helps the
practitioner evaluate both active and inactive lesions
[1] .
Therapeutic tactics for managing patients with LS
depend on many factors: the activity of the
pathological process, localization of rashes (foci of
local inflammation (erythema, edema) followed by the
formation of sclerosis and/or atrophy of the skin and
underlying tissues), form of the disease, age patient.
Local therapy usually includes glucocorticosteroids
(GCS), calcineurin inhibitors, vitamin D analogues, and
phototherapy. Systemic therapy, in turn, is effective
for common and severe forms of the disease. The most
commonly used systemic approach is a combination of
corticosteroids and methotrexate [ 5 ]. When planning
treatment, it should be borne in mind that clinical
effects sometimes appear no earlier than 3 months
after the start of therapy [ 2 ]. Mycophenolate mofetil
(MPM) has been proposed as an alternative
immunomodulatory agent in cases of methotrexate
resistance [ 1-4 ]. In vitro studies have shown that MPM
suppresses the proliferation of lymphocytes, as well as
other types of mesenchymal cells, including smooth
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Publisher:
Oscar Publishing Services
Servi
muscle cells and fibroblasts [ 4 ]. Using a series of cases
of drugs resistant to methotrexate therapy, it was
shown that the use of MPM leads to a decrease in the
degree of skin sclerosis and inflammation (according to
infrared thermography and clinical assessment) [ 6 ]. In
recent studies, the combination of GCS and
methotrexate/MFM
showed
inconsistent
effectiveness; several cases of drugs were presented in
adolescents who did not respond to such treatment [ 7
]. The effectiveness of an alternative treatment
method for drugs in adults and children has been
reported using the drug abatacept, a recombinant
fusion protein that blocks T-cell activation, approved in
the USA and the Russian Federation for the treatment
of rheumatoid arthritis [10 ] .
RESULTS
Particular attention is paid to the search for treatment
methods for drugs that can eliminate the
consequences of the disease, namely gross cosmetic
defects of the face that negatively affect the harmony
of the physical and psycho-emotional development of
the child. Recently, fat grafting has become the focus
of attention (due to the effectiveness of the method in
recreating volume and improving skin quality). Fat
transfer, including adipocytes, adipose stem cells,
endothelial cells and vascular smooth muscle cells, has
been shown to reduce inflammation as well as fibrosis
by limiting the synthesis of extracellular matrix
proteins and promotes increased collagenase activity,
as well as providing structural support. support due to
proliferation and differentiation of stem cells [11 ] .
Below is a description of a clinical case of LS in order to
demonstrate the possibilities of correcting skin defects
in children.
CLINICAL CASE STUDY
Patient A., 1 7 years old, complained of skin atrophy in
the forehead area. In 2019 , the diagnosis was made:
“Localized scleroderma, linear form.” The skin
pathological process at the time of diagnosis was linear
in nature and was represented by a focus of atrophy of
the skin and underlying flesh-colored tissues. The
lesion was localized on the skin of the forehead with
transition to the skin of the scalp and supraorbital
region. The size of the skin atrophy is 6 cm in height
and 3 cm in width. When performing an ultrasound of
the skin and soft tissues of the supraorbital region,
forehead, and scalp, thinning of the skin and
subcutaneous fat in the supraorbital region, as well as
in the forehead on the right, was noted.
The patient received methotrexate therapy for two
years with positive dynamics
—
a decrease in the clinical
activity of the disease and stabilization of the skin
pathological process (reduction in the severity of
inflammation) were noted. After 6 months she was
hospitalized for further examination. A general
assessment of the state of health and monitoring of
the titer of antibodies to single-stranded DNA
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(negative test) and to the Scl-70 antigen (negative test)
were carried out. The patient was recommended for
contouring with autologous fat (lipofilling), which was
performed in the plastic surgery department.
After treating the surgical field under intravenous
anesthesia using a cannula with a diameter of 2.7 mm
and a length of 23 cm, syringe lipoaspiration was
performed in the area of the inner thighs. Fat was
obtained in a volume of 30 ml. A cannula was used to
perform a blunt detachment of scar tissue from the
underlying structures. The introduction of adipose
tissue into the recessed areas was carried out using a
cannula with a diameter of 1.6 mm in a volume of 23 ml:
in the scalp area - 3 ml, in the frontal area - 15 ml, supra-
orbital area - 5 ml. The remaining 7 ml of fat graft were
treated with nanotransfer (Tulip nano system, USA), a
cell fraction was obtained, which was injected
intradermally into the affected area. The postoperative
period was uneventful. Methotrexate therapy was
discontinued.
CONCLUSION
Pathogenetic therapy of drugs does not have a
significant effect on the elimination of cosmetic
defects accompanying the disease. In this regard, the
most promising method for correcting a skin defect is
contouring with autologous fat (lipofilling), which
allows you to recreate the natural contour and fullness
in the affected area. Autologous fat grafting may be an
effective therapeutic alternative in patients with LS.
The presented clinical observation demonstrates the
effectiveness of using the method in a teenager -
leveling the cosmetic defect of the soft tissue
structures of the facial skull.
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