13 - 76 - VITILIGO Flashcards
Clinical signs of lesional activity in vitiligo
confetti, trichrome, and inflammatory lesions, koebnerization
this variant of vitiligo tends to occur earlier in life
segmental variant
often the initial sites of vitiigo
face, as well as acral and genital locations
- eportedly more common in adults and typically involves the hands, feet, and face, particularly the orifices
- may evolve to typical generalized vitiligo
Acrofacial vitiligo
- rare form of widespread disease
- usually seen in adults, although cases in children have been reported
- affects a large proportion of the body, frequently defined as greater than 80% of the body surface area
Vitiligo universalis
Classically, vitiligo universalis results from longstanding disease that steadily progresses to nearly complete whitening of the skin.
oral and/or genital mucosae are primarily involved
Mucosal vitiligo
variant that consists of small, isolated lesions
Focal vitiligo
unilateral and segmental, or block-shaped, distribution of the lesions
segmental variant of vitiligo
In segmental vitiligo, there is frequently early involvement of the follicular melanocyte reservoir, resulting in poliosis.
rare form of vitiligo that refers to the occurrence of a clear example of segmental vitiligo plus additional macules or patches that do not fit the segment
Mixed vitiligo
These additional patches may be remote from the segmental involvement and are bilateral and symmetrical, affecting the contralateral side.
In this variant, there is frequently early involvement of the follicular melanocyte reservoir, resulting in poliosis.
segmental vitiligo
describe the course of segmental vitiligo
The disease usually spreads over the segment within 6 to 12 months, and then stabilizes
associated with higher body surface area involvement in vitiligo and** poorer response to treatment**
Koebner phenomenon
observation that depigmentation occurs readily at the site of skin trauma in patients with active vitiligo
Koebner phenomenon, also called the isomorphic response
This can be recognized as linear marks of depigmentation where the skin has been scratched, lacerated, or burned, or nonlinear macules and patches at the site of known skin injury, such as erosions and abrasions
characterized by blurring of lesional borders because of the presence of a hypopigmented zone between the depigmented and normally pigmented border
Trichrome vitiligo
This results in the appearance of 3 distinct colors: the depigmented skin, normally pigmented skin, and hypopigmented skin
Trichrome vitiligo
This pattern is associated with active, rapidly spreading vitiligo
Trichrome vitiligo
consists of multiple small macules of depigmentation clustered together, often at the edge of existing vitiligo lesions
Confetti-like depigmentation
this sign as an important marker of disease activity
Confetti-like depigmentation
very rare form of vitiligo characterized by the presence of erythema, scale, and itch at the border of hypopigmented or depigmented lesions
Inflammatory vitiligo
typically transient, lasting just a few weeks to months but rapidly progressing to involve large areas of the body
6-point scale that was developed with the aim of assessing and monitoring vitiligo activity.
vitiligo disease activity score (VIDA) score
defined as the spread of existing lesions or onset of new lesions
Active vitiligo
VIDA score where vitiligo is stable for 1 year or more with spontaneous repigmentation
-1
- uses the Koebner phenomenon and anatomical location of lesions to determine disease activity in vitiligo patients
- It is based on the presence or absence of vitiligo lesions at 6 different areas of the body (forehead + scalp areas, eyelids, wrists, genital + belt areas, knees and tibial crests) as well as disease duration.
- ranges from** 0 to 56,** with 56 corresponding to the highest likelihood of having the Koebner phenomenon, which serves as an indicator of disease activity in the clinic
K-VSCOR
Associated autoimmune diseases with vitiligo
type 1 diabetes, autoimmune thyroiditis, pernicious anemia, Addison disease, lupus, and alopecia areata
Up to 20% of vitiligo patients have at least 1 additional autoimmune disease, and most of these patients (13% to 19%) have what disease?
autoimmune thyroid disease
- This increased risk has prompted some to advocate testing thyroidstimulating hormone (TSH) in all patients with vitiligo, because the pretest probability of finding a positive result is higher in this patient population.
- Others suggest, however, that this is unnecessary, because many patients will develop thyroid disease much earlier or later than the onset of vitiligo, and recommend that the presence of symptoms should drive TSH screening.
T/F
vitiligo patients have close to 3-fold higher risk of developing melanoma, basal cell carcinoma, and squamous cell carcinoma compared to controls
FALSE
Lower risk
results in skin depigmentation with prominent poliosis, as well as **hearing loss, visual changes, meningitis, and flu-like symptoms
Vogt-Koyanagi-Harada syndrome (VKHS)
segmental vitiligo (unilateral depigmentation) on the face with poliosis, plus ipsilateral hearing loss and visual changes
Alezzandrini syndrome
Vitiligo is an autoimmune disease of the skin in which what type of T cells target melanocytes and destroy them?
CD8+ T cells
Additional studies have now revealed that certain chemicals, typically ______, induce the cellular stress response in melanocytes by acting as analogs of tyrosine, also a phenol
phenols
Thus, these chemicals act as exogenous environmental agents that induce and exacerbate vitiligo by initiating the cellular stress response in otherwise well-compensating melanocyte
play a critical role during the progression of vitiligo, serving as the primary immune effectors that destroy melanocytes
CD8+ T cells
key cytokine that drives vitiligo
(IFN)-γ
IFN-γ is secreted by melanocyte-reactive autoimmune CD8+ T cells, and induces the production of CXCL10 and other chemokines from keratinocytes, which promote the further recruitment of additional T cells that progressively destroy more melanocytes as the disease spreads
drugs that cause depigmentation of skin
- monobenzyl ether of hydroquinone, or monobenzone
- 4-tert-butyl phenol
- 4-tertbutylcatecho
findings signifying presence of repigmentation
perifollicular pigmented macules from pigmented hairs at hair-bearing sites or convex patterns of pigment at lesional borders in glabrous skin
Hair-bearing sites without poliosis repigment easily, whereas glabrous skin and lesions containing mostly white hairs respond poorly.
should be considered the first treatment option in patients with more than 5% of the body surface area affected
full-body phototherapy
may be used as monotherapy when there is limited surface involvement (less than 5% body surface area)
Topical therapies
treatment that achieves highest efficacy for segmental vitiligo in its early phase (ie, disease onset of less than 6 months to 1 year)
Targeted phototherapy
it is the only Food and Drug Administration–approved medical therapy for vitiligo
monobenzone
Surgery in vitiligo should be reserved for patients with highly stable disease, which has been defined as..
absence of new or growing lesions for 1 to 2 years
Because of their improved donor-to-recipient-site ratio, excellent outcomes in percent repigmentation and color match, as well as improved healing, these grafts are becoming the first-line in surgical management of stable vitiligo
cellular grafts
α-melanocyte–stimulating hormone analog
afamelanotide
Treatment algorithm for vitiligo
Treatment algorithm for segmental vitiligo
