Volume 03 Issue 01-2023
38
International Journal of Medical Sciences And Clinical Research
(ISSN
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2771-2265)
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OCLC
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Publisher:
Oscar Publishing Services
Servi
ABSTRACT
Capillary malformations (capillary angiodysplasia, CAD) in patients are an urgent problem of modern medicine due to
functional, cosmetic, psychological disorders and social maladjustment of this category of patients in the absence of
timely adequate treatment.
KEYWORDS
Flaming nevus, pathogenesis, neurotrophic factors, diagnosis, laser treatment, prognosis, malformation, mutation,
percutaneous laser coagulation, capillary angiodysplasia.
INTRODUCTION
Transcutaneous laser photocoagulation (selective
photothermolysis) is currently the "gold standard" for
the treatment of port-wine stains. However, it is
extremely rare to achieve complete removal of a
vascular lesion, and resistance to laser exposure is
noted in 20% of cases. The article presents clinical cases
of the treatment of port-wine stains in two patients.
Based on our own experience and literature data, we
analyzed the results of treatment, side effects of the
Research Article
MODERN APPROACH TO THE DIAGNOSIS AND TREATMENT OF
FLAMING NEVUS
Submission Date:
January 20, 2023,
Accepted Date:
January 25, 2023,
Published Date:
January 30, 2023
Crossref doi:
https://doi.org/10.37547/ijmscr/Volume03Issue01-07
U.A. Tashkenbayeva
Doctor Of Medical Sciences, Professor Head Of The Department Of Dermatovenereology, Tashkent Medical
Academy, Tashkent, Uzbekistan
M.R. Mukhamedova
Lecturer Department Of Dermatovenereology, Tashkent Medical Academy Tashkent, 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 01-2023
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use of lasers, and also considered alternative promising
methods of treatment.
A flaming nevus is a capillary angiodysplasia that occurs
in the embryonic period of prenatal development.
Occasionally, an acquired form of nevus is observed,
associated with injuries, past infections, and taking
medications. A flaming nevus has the appearance of a
bright pink, red or purple spot of various sizes and
bizarre shapes. It is predominantly located on the face,
neck, and upper torso.
THE MAIN RESULTS AND FINDINGS
In the medical literature, two synonymous concepts
are used: a flaming nevus (nevus flammeus) and a wine
stain (port wine stains, PWS), which describe the same
congenital anomaly of development. Pathology occurs
in 0.3-0.5% of newborns, no significant gender, ethnic
and racial differences were found.
The intensity of the color can change during the day,
for example lighter in the morning, darker in the
evening. The brightness of the spot increases with a
rise in div or environmental temperature, during
exercise or feeding a child. But these changes are
reversible: when the effect of the irritating factor
stops, the stain acquires its usual color.
As a result of the pathological expansion of capillaries
on the skin of a person, an irregularly shaped spot
appears, the color of which varies from light pink to
purple, burgundy and violet. Most often, a flaming
nevus is located on the skin of the face and neck, but in
some cases it can be localized in other parts of the
div. As a person grows older, the port wine stain
increases in size.
Wine birthmark is not able to degenerate into a
malignant tumor, so it does not pose a direct danger to
the patient's life. However, it must be borne in mind
that a flaming nevus can lead to a number of problems,
so it must be treated.
Introduction. All vascular malformations of the skin are
the result of a violation of the formation of the
embryonic vascular system from the 4-8th to the 19-
20th week of embryo development. They exist from
birth, although they may not appear for a long time.
The most common capillary malformations are
described in the literature as port-wine stains, flaming
nevus,
capillary
malformations,
and
capillary
angiodysplasias (CAD). The prevalence of CAD is 3-5
cases per 1000 newborns; distinctive features - a large
number of abnormally dilated blood vessels without
signs of endothelial proliferation in the papillary and
partially reticular layers of the dermis, do not undergo
spontaneous involution and progress with age. The
size of the spots varies from a few millimeters to half
of the div surface, the area increases in proportion to
the growth of the child, about 80% of the lesions occur
in the head and neck region. Capillary angiodysplasia
can be part of a number of pathological conditions, the
most common are Sturge-Weber and Klippel-
Trenaunay syndromes.
Characteristic external signs of a flaming nevus: Wine
stains on the skin have an irregular shape, reminiscent
of a geographical map. As a person grows older, their
area proportionally increases.
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Color - from pinkish to maroon. They may turn blue
with age. When lightly pressed with a finger, the spots
lighten, then darken again.
In the initial stages of development, a flaming nevus
does not cause physical discomfort; itching and
inflammation are absent.
Over time, the surface of the nevus may become
uneven, with convex nodules
–
angiomas.
Classification and stages of development of capillary
malformation
The most convenient classification of vascular
anomalies was created by the International Society for
the Study of Vascular Anomalies (ISSVA). It is
constantly supplemented with new nosologies and
refined. Now in this classification the following types
of capillary malformation are distinguished:
simple;
combined with other types of vascular anomalies:
capillary-venous, capillary-lymphovenous, capillary-
arteriovenous;
as part of syndromes, such as Sturge-Weber syndrome
and Klippel-Trenaunay syndrome. The first syndrome is
accompanied by damage to the skin, eyes, nervous
system and internal organs. It is characterized by the
presence of capillary malformation on the skin of the
face, angioma of the choroid and pia mater. Klippel-
Trenaunay syndrome is a rare congenital disorder in
which the formation of blood and lymphatic vessels,
skin, muscles, and bones is disrupted. It is accompanied
by the appearance of a port-wine stain, malformations
of the veins, proliferation of soft tissues and bones.
Etiology. There is no exact data regarding the causes of
this pathology in modern medicine.
Genetically, the formation of wine stains is associated
with capillary malformations. Patients with flaming
nevus often have defects in the GNAQ6, RASA16,
EPHB4, and PIK3CA genes. Genetically proven
connection of the formation of malformations with the
following syndromes:
Sturge-Weber syndrome - the location of port-wine
spots on the face along the branches of the trigeminal
nerve. In addition to skin symptoms, vascular
anomalies of the brain and eyes occur, and atrophy of
the nervous tissue is possible.
CLOVES syndrome. Pathology is manifested by
multiple capillary, venous and lymphatic dysplasia.
They are associated with epidermal nevi. Patients have
lipomatosis, scoliosis, lesions of bones and joints.
Proteus Syndrome. Vascular malformations are
combined with abnormally rapid and excessive growth
of certain parts of the div, namely in the area of the
palms and soles.
CLAPO syndrome. Capillary dysplasias are located in
the region of the lower lip, perioral zone. Pathology is
accompanied by macroglossia, deformation of facial
features and limbs.
Klippel-Trenaunay syndrome. A distinctive feature of
the flaming nevus with this defect is its location on the
lower extremities. Pathology is accompanied by
varicose veins in the legs, hypertrophy of bones and
soft tissues.
The acquired flaming nevus, which was first described
by the German dermatologist F. Fegeler in 1949, is
distinguished into a separate category. Wine stains
occur after trauma to the cervical or thoracic spine.
Pathology can manifest itself at any age. Later, other
triggers of acquired PWS were established: frostbite,
hormonal and lipid-lowering drugs, and herpes zoster.
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Pathogenesis. The formation of congenital pathology
falls on the 5-10th week of the prenatal period. In
pathogenesis, two key hypotheses are distinguished:
impaired vascular innervation and genetic mutations of
angiogenesis. It has been established that the number
of S-100 nerve fibers is sharply reduced in pathological
dermal vessels. This causes a decrease in basal vascular
tone and a decrease in the number of neurotrophic
factors - the main causes of abnormal vascular growth.
Abnormal expression of arterial (EfnB2) and venous
(EphB1) markers occurs in abnormal skin vessels. Such
processes cause disturbances in the primary
differentiation of dermal arterioles and venules, and
contribute to excessive capillary growth. In the
pathogenesis of the disease, pathological activation of
the signaling pathways of enzymes is involved:
activated protein kinases, phosphoinositide-3-kinases.
Pathologically, a flaming nevus is characterized by
dilated capillaries and post-capillary venules, which are
located in the papillary layer and the upper part of the
reticular dermis. Signs of endothelial proliferation and
cellular atypia are absent. Gradually, the number of
ectatic vessels increases, and perivascular fibrosis
progresses.
Diagnosis requires a clinical examination, Doppler
ultrasound,
MR
angiography,
and
computed
tomography. The decisive role in the diagnosis is
played by the congenital nature of the spot, its typical
localization and wine color. It is necessary to take into
account the burden of the anamnesis with the genetic
manifestations of the disease.
Doppler ultrasound. Ultrasound diagnosis of the
affected area of the div is necessary to assess blood
flow in pathological vessels, to exclude more
dangerous arteriovenous malformations that can
mimic a flaming nevus.
MR angiography. MRI can determine the presence of a
vascular component in soft tissues. In the case of a
simple capillary malformation, such changes are
insignificant, so the information content of such a
study is minimal. Given that in young children this study
is performed under anesthesia or sedation, routine
MRI is not recommended for children. Indications for
MRI are: combined forms of vascular malformations
and syndromic forms of the disease, such as Sturge-
Weber syndrome.
CT scan. The study is carried out with malformations in
order to assess the structure of the connective tissue,
to identify pathologies of the musculoskeletal system.
With concomitant neuralgic symptoms, a CT scan of
the brain is indicated.
tissue biopsy. Sampling of biomaterial is required for
differential diagnosis between congenital and acquired
forms of port wine stain, for genetic research as part of
the diagnosis of a hereditary syndrome.
Treatment of a flaming nevus. To date, laser exposure
methods are used on the skin much more often than
on any other tissues, due to the exceptional diversity
and prevalence of skin pathology and various cosmetic
defects, as well as the relative ease of performing
procedures, which is associated with the superficial
location of objects requiring treatment.
Due to its prevalence and pronounced cosmetic defect,
flaming nevus is an important problem in practical
dermatology. The efforts of specialists are aimed at
developing minimally invasive and effective ways to
remove stains.
Wine stains are subject to aesthetic correction by laser
therapy. The effectiveness of treatment depends on
the age of the patient, the size, number and depth of
the vessels. The optimal age for the first procedure is
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from 3 months to 6 years. In this period, it is possible
to achieve lightening by 55% in one procedure, while
with age, the effectiveness of laser coagulation is 1.5-2
times lower.
The technique of laser coagulation with a pulsed laser
with a wavelength of 595nM shows the maximum
effectiveness in superficially located neoplasms. Wine
stains on the lateral surfaces of the face are best
corrected, since the vessels are located in the papillary
layer of the dermis. Nevi in the central part of the face
and on the div go away more slowly, require a long
course of laser therapy.
20% of patients with giant and nodular vascular
malformations show resistance to standard laser
treatment. In such a situation, alternative hardware
procedures are used: exposure to intense pulsed light
(phototherapy), alexandrite and neodymium lasers. To
achieve a lasting result, you will need about 10 sessions
with intervals of 2 to 6 weeks between them.
Patients and methods. On the basis of the Department
of Dermatovenereology of the Tashkent Medical
Academy, 230 patients with CAD aged from 2 weeks to
36 years received treatment with a pulsed dye laser
with a wavelength of 595 nm. Light waves emitted by
laser devices, together with the use of a dye, penetrate
the epidermis and are absorbed in the dermis by the
target chromophore, in this case, hemoglobin. The
peak absorption of a light pulse by hemoglobin
depends on the degree of its saturation with oxygen.
So, for oxyhemoglobin, the peak absorption is 577 nm,
for carboxyhemoglobin - about 585 nm.
Wine stains are located on the right half of the woman's face.
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Wine stain on the right side of the neck.
(a) Before starting treatment. (b) After 5 sessions of
laser therapy performed 1 month apart.
The final improvement was 70%.
Results. Analysis of the results of laser therapy showed
that the total frequency of "excellent" and "good"
effect of CAD lightening was 83%. Satisfactory results
were observed in 18.9% of patients, unsatisfactory in
0.6%. Lack of response to treatment due to deep
vascular involvement was noted in one patient with
Klippel-Trenaunay syndrome. Complications are not
registered.
A positive correlation was found between the skin
phototype and the effect of laser treatment (r=0.46;
p<0.05). In 96 and 86.3% of patients with phototypes I
and II, respectively, the degree of clarification of the
CAD zone exceeded 50%. In the vast majority (82.1%) of
patients with skin phototype III, the regression of
malformations was only 25-49%.
In addition, a significant positive correlation was found
between the localization of the CAD and the degree of
its lightening as a result of laser treatment (r=0.56;
p<0.05). Laser exposure is especially effective in the
projection of the 1st branch of the trigeminal nerve, for
example, the forehead, and the 2nd branch, for
example, in the area of the zygomatic bone. It should
be emphasized that in all patients who received laser
treatment of CAD in the facial area, the degree of
clarification of the malformation exceeded 25%, there
were no failures.
Of the side effects of laser treatment of CAD, it is
necessary to note the appearance of temporary gray-
blue spots - purpura or bruises at the site of laser
exposure and edema of varying severity. In this study,
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we observed them in all 230 patients. The purpura
persisted for 7-10 days.
CONCLUSION
Our own research results have shown high efficiency of
CAD laser therapy in patients. An integrated approach
to the diagnosis and treatment of patients with
vascular malformations based on large medical
institutions ensures individual treatment regimens and
the effectiveness of the method.
Forecast and prevention. Laser coagulation can
significantly lighten the flaming nevus, which has a
positive effect on the appearance and psychological
comfort of patients. With an isolated vascular anomaly,
the prognosis is favorable. With a combination of
vascular spots with genetic syndromes, the prognosis
is determined by the severity of somatic symptoms.
Measures to prevent the disease have not yet been
developed.
CONCLUSION
"Port-wine stains" are a set of dilated capillaries of
various diameters, occurring at different depths. The
thickness of the epidermis over the vascular lesion and
the thickness of the papillary dermis are different in
different parts of the div. In addition, in the
pathogenesis of this pathology, there is a violation of
the innervation of blood vessels. Therefore, the use of
"vascular" lasers for the treatment of this pathology
has its own difficulties and nuances.
Despite the fact that laser photocoagulation remains
the only effective method for the treatment of port-
wine stains, new technologies are being sought to
improve treatment results. The use of devices with
different wavelengths (755 nm, 810 nm, 1064 nm), the
use of epidermal cooling can in some cases solve the
problem of PWS resistant to ILK 595 nm.
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