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VITILIGO

Definisi
The word vitiligo may be derived from the Greek vitelius, signifying a calf's white patches.
Vitiligo is an acquired loss of pigmentation characterized histologically by absence of
epidermal melanocytes. It may be an autoimmune disease associated with antibodies (vitiligo
antibodies) to melanocytes, [88] but the pathogenesis is still not understood. Studies suggest
there is some genetic mechanism involved in the etiology of vitiligo and that it is polygenic in
nature. [89] There is a positive family history in at least 30% of cases. Both sexes are affected
equally. Approximately 1% of the population is affected; 50% of cases begin before age 20.
The pigment loss may be localized or generalized.(habif 571)
Etiologi
Vitiligo is a multifactorial polygenic disorder with a complex pathogenesis. Although several
theories have been proposedto explain the loss of epidermal melanocytes in vitiligo, the
precise cause remains unknown.Theories include autoimmune, cytotoxic, biochemical,
oxidant-antioxidant, neural, and viral mechanisms for destruction of epidermal melanocytes.
Several studies also point to a significant role of genetic susceptibility to vitiligo. (fitz 654)
Gambaran Klinnis
Patients with vitiligo present with one to several amelanotic macules that appear chalk- or
milk-white in color. The lesions are usually well-demarcated, but the margins may be
scalloped. They are accentuated on Wood's lamp examination. Lesions enlarge centrifugally
at an unpredictable rate and can appear on any body site, including mucous membranes.
However, initial lesions occur most frequently on the hand I forearms, feet, and face. When
vitiligo occurs on the face, it often favors a perioral and periocular distribution.(fitz 655)
Klasifikasi vitiligo
Vitiligo is classified as segmental, acrofacial, generalized, and universal, or by pattern of
involvement as focal, mixed, and mucosal types.
Focal vitiligo : Usually a solitary macule or a few scattered macules in one area, most
commonly in the distribution of the trigeminal nerve, although the neck and trunk are also
commonly involved.

Gambar 1(a) dan 1 (b). Kedua-dua gambar ini menunjukkan lesi vitiligo tipe fokal. (fitz 655)
Segmental vitiligo (Fig. 72-2): Unilateral macules in a dermatomal or quasi-dermatomal
distribution. This tends to have an early age of onset and, unlike the other types, is not
associated with thyroid disease or other autoimmune diseases. This type occurs more
commonly in children. Alteration of neural peptides has been implicated in the pathogenesis
of this type. More than one-half of the patients with segmental vitiligo have patches of white
hair, known as poliosis.

Gambar 2(a) dan 2 (b). Segmental vitiligo. (a) segmental vitiligo yang terjadi pada daerah
wajah dan leher dengan distribusi quasi-dermatomal. (b) Segmental vitiligo yang
menyebabkan poliosis pada alis dan bulu mata.(fitz 656)
Acrofacial vitiligo (Fig. 72-3): Depigmentation of the distal fingers and periorificial areas.

Gambar 3. Lesi pada penderita vitiligo tipe akrofasial.
Generalized vitiligo (Figs. 72-4): Also termed vitiligo vulgaris, the most common pattern.
Depigmented patches are widely and usually symmetrically distributed.

Gambar 4. Lesi pada penderita vitiligo tipe generalisata. (andrew 871)
Universal vitiligo (Fig. 72-5): Depigmented macules and patches over most of the body,
often associated with multiple endocrinopathy syndrome.

Gambar 5. Lesi pada penderita vitiligo tipe universal(fitz 656)
Mucosal vitiligo: Involvement of the mucus membrane sites only.
Kelainan yang menyertai
Vitiligo is frequently associated with disorders of autoimmune origin. The most prevalent
associated endocrinopathy is tl1yroid dysfunction, either hyperthyroidism (Graves disease) or
hypothyroidism (Hashimoto thyroiditis). Vitiligo usually precedes the onset of thyroid
dysfunction.(fitz 657)
Addison disease, pernicious anemia, alopecia areata, and diabetes mellitus also occur with
increased frequency in patients with vitiligo. Patients with autoimmune polyendocrinopathy
candidiasisectodermal dystrophy have an increased prevalence of vitiligo.(fitz 657)
Vitiligo may affect active melanocytes throughout the body, including pigment cells present
in hair, the inner ear, and the retina. Poliosis (leukotrichia) occurs in many patients.
Premature graying has been reported in vitiligo patients and in their close relatives. Auditol)'
and visual disturbances are present in some patients. Aseptic meningitis may rarely result
from destruction of leptomeningeal melanocytes.(fitz 657)
OCULAR DISEASE
Although patients with vitiligo do not usually have ophthalmologic complaints, they can have
several ocular findings. Pigmentary abnormalities of the iris and retina may occur. Choroidal
abnormalities have been reported in up to 30 percent of patients and iritis in approximately 5
percent. Uveitis can be a frequent ocular manifestation. Exophthalmos may occur in the
setting of concomitant Graves disease. Visual acuity is generally not affected.(fitz 657)
VOGT-KOYANAGI-HARADA SYNDROME
The Vogt-Koyanagi-Harada syndrome (VKH) consists of vitiligo in association with uveitis,
aseptic meningitis, dysacusis, tinnitus, poliosis, and alopecia. It is a rare, systemic, T-cell-
mediated autoimmune disorder. VKH syndrome is associated with other autoimmune
disorders such as autoimmune polyglandular syndrome, hypothyroidism, Hashimoto
thyroiditis, and diabetes mellitus. VKH syndrome classically occurs in three phases. During
the first phase, the meningoencephalic phase, patients may have headache, meningismus,
seizures, muscle weakness, or paralysis after a prodrome of fever, malaise, nausea and
vomiting. Subsequently, the acute ophthalmic phase occurs when patients may develop
photophobia, eye pain, and altered visual acuity. Patients may develop uveitis, iridocyclitis,
choroiditis, and retinal detachment during this phase and can later develop complications
such as cataracts and glaucoma. Vitiligo, alopecia, and poliosis22 usually follow, but can
occur before the other manifestations.(fitz 657)
AlEZZANDRINI SYNDROME The constellation of clinical findings in Alezzandrini
syndrome includes facial vitiligo, poliosis, deafness, and unilateral tapetoretinal degeneration.
The etiology remains poorly understood, but as in vitiligo and VKH syndrome, autoimmune
processes are thought to be involved.(fitz 657)
Diagnosa
The diagnosis of vitiligo is primarily based on clinical examination. However, given the
association between vitiligo and other autoimmune diseases, several screening laboratol)'
tests are helpful, including thyroid stimulating hormone level, antinuclear antibody, and
complete blood count. Clinicians should also consider investigating for serum
antithyroglobulin and antithyroid peroxidase antibodies, particularly when patients have signs
and symptoms of thyroid disease.(fitz 657)
Examination with the Wood's light in a dark room accentuates the hypopigmented areas and
is useful for examining patients with light complexions. The axillae, anus, and genitalia
should be carefully examined. These areas are frequently involved but often clinically
nonapparent without the Wood's light. Vitiligo may be a predictor of metastases in melanoma
patients, and a Wood's light examination may show early subtle changes in these
patients.(habif 572)

Diagnosa banding
Tinea Versikolor
A mild, chronic infection of the skin caused by Malassezia yeasts, and characterized
by discrete or confl uent, scaly, discoloured or depigmented areas, mainly on the
upper trunk.(rook 36.10)

Gambar 6. Lesi pada penderita tinea versikolor(bologna)

Pitytiasis alba
This is a pattern of dermatitis in which hypopigmentation is the most conspicuous
feature. Some erythema and scaling usually precede the development of
hypopigmentation but these are often relatively mild.(rook 23.27)


Gambar 7. Lesi pada penderita pitiriasis alba. (rook 23.28)

Hipopigmentasi pasca-inflamasi

Gambar 8. Tampak plak inflamatori yang kemerahan pada kulit kepala dengan
hipopigmentasi pasca-inflamasi.(fitz 1562)
Lichen Sclerosus et atrophicus

Gambar 9. Lesi pada penderita Lichen Sclerosus et atrophicus



Penatalaksanaan
There arc many different treatment options available for patients with vitiligo (Box 72-2).
Most therapies are intended to restore pigment to the skin. All approaches have advantages
and disadvantages; and none is appropriate for every patient with vitiligo.(fitz 658)
Sunscreen
Sunscreens help prevent sunburn and thus may lessen photodamage as well as the
chance that a Koebner phenomenon will occur. Sunscreens also decrease tanning of
the uninvolved skin and therefore lessen the contrast with vitiliginous lesions.

Penggunaan kosmetik
Many patients, especially patients with focal vitiligo, find cosmetic cover ups to be a
valuable treatment option. Areas of leukoderma, especially on the face, neck, or hands
can be covered with conventional make-up, self-tanrung prodUCts, or other topical
dyes. Cosmetics offer limited cost, minimal side effects, and the ease of
application.(fiyz 658)

Kortikosteroid topikal
Topical corticosteroids are indicated for the treatment of limited areas of vitiligo and
are often the first line of therapy for children,~29 although most experience is
anecdotal. Lesions on the face appear to have the best response to topical
corticosteroids; lesions on the neck and extremities (with the exception of the fingers
and toes) also have a favorable response. Localized lesions can be treated with
a high-potency fluorinated corticosteroid for 1 to 2 months, after which prudence
dictates that therapy is gradually tapered to a lower-potency corticosteroid.
In children and patients with larger lesions, a medium potency non-fluorinated
corticosteroid is often used. Caution must be used when using topical steroids on and
around the eyelids, as their use can increase intraocular pressure and exacerbate
glaucoma. (fitz 658)

Wood's lamp examination can be used to monitor response to treatment. if no
response is seen by 3 months, therapy should be discontinued, Maximum
repigmentation may take 4 months or longer (there is a 30 percenr to 40 percent
response rate with 6 months of corticosteroid use).(fitz 658)

Imunomodulator topikal
Topical tacrolimus ointment 0.03 percent to 0.1 percent is effective in repigmentation
of vitiligo when applied twice daily in patients with localized disease, particularly on
the face and neck.3<hU.3M7 It is reported to be more effective when combined with
ultraviolet B (WB) or excimer (308 nm) laser therapy.34,fIS Tacrolimus ointment is
generally considered safer for children than topical steroids.(fitz 658)

Calcipotriol topikal
Topical calcipotriol 0.005 percent produces cosmetica.Uy acceptable repigmentation
in some patients with vitiligo. It can be combined with topical corticosteroids in adults
and children to give possibly faster onset of repigmentation with better stability of
achieved pigmentation. (fitz 658)

Pseudokatalase
Catalase, an enzyme normally found in skin that decreases damage hom free radicals,
has been reported to be low in the skin of vitiligo patients. A replacement therapy
using an analog of normal human catalase (pseudocatalase) in combination with
narrowband WB (NB-UVB) phototherapy has been reported in uncontrolled trials to
re-pigment some vitiligo patients and prevent progression of disease.(fitz 658)

Terapi sistemik
Systemic immunosuppressive drugs have many potential side effects that are difficult
to justify for a disease such as vitiligo. However, systemic corticosteroids have been
used as pulse therapy with variable results and may prevent rapid depigmentation in
active disease.(fitz 658)

Terapi PUVA
Topical or oral 8-methoxypsoralen combined with UVA (320 to 400 nm) irradiation
(PUVA) is effective for treating vitiligo,4ll-SO although frequent treatments over
many months are required. Topical PUVA is sometimes used in patients whose
vitiligo involves less than 20 percent of the body surface area. However, unwanted
side effects are common and include cosmetically displeasing hyperpigmentation of
skin surrounding vitiligo areas due to inadvertent psoralen application, severe
phototoxicity reactions, and intense pruritus. Oral psoralens are used for patients with
more extensive involvement or in patients who do not respond to topical PUVA (fitz
658)

Narrowband Ultraviolet B radiation
NB (311 nm)-UVB irradiation is another option for patients with vitiligo and is
considered by many to be the first choice for most patients. In patients with extensive
generalized vitiligo, NB-UVB therapy was more effective than topical PUVA. If no
improvement is seen within 6 months of treatment, NB-UVB therapy should be
abandoned.(fitz 659)

Laser eksimer
Excirner (308 nm) laser has been recently studied in several trials for its cr Bcacy in
treating vitiligo. It has been found to be most effective when treatments are given
three times weekly, with treatment periods of more than 12 weeks necessary to obtain
satisfactory repigmentation. The initial dose is 50 to 100 mj/cm2.(fitz 659)


Depigmentasi
The most commonly used agent is 20% monobenzyl ether of hydroquinone (MBEH),
applied once to twice daily to the affected areas for 9-12 months or longer
[40]
. MBEH
is a potent irritant and/or allergen. It normally takes 1-3 months to initiate a response,
and a loss of pigment can occur at distant sites. Although depigmentation from
MBEH is considered permanent, repigmentation (especially perifollicular and in areas
with pigmented hairs) can be seen following a sunburn or even intense sun exposure.
Monomethyl ether of hydroquinone in a 20% cream can be used as an alternative to
MBEH
[42]
. Side effects include contact dermatitis, exogenous ochronosis and
leukomelanoderma en confetti.(bologna)

Autologous thin thiersh grafting
Thin split-thickness grafts in the treatmentof vitiligo are obtained using scalpel or
dermatome and are placed onto recipient sites prepared in a similar manner or by
dermabrasion. Achromic areas ranging in size from 6 to 100 cm2 can be treated_
Recently, the technique of thin split-thickness grafts has been modified for the
treatment of vitiligo with the harvesting of grafts by mechanical dermatome, which
has shown excellent results. This technique has also been used to liuccessfully treat
Vitiligo of the lip. The advantage of this technique is that it allows the grafting of
large areas in a J elatively short time.(fitz 659)

Prognosis
The prognosis and course of vitiligo are unpredictable. Initial clinical sub-type of vitiligo
does not predict future anatomical sites of involvement or activity of disease. (fitz 657)