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INTRODUCTION Vitiligo is an acquired chronic disease characterized by depigmented macular patches due to loss of epidermal melanocytes. It affects 1,6% of the general population in Croatia.Female patients are more affected (53,95%) than male patients, with no difference in the severity of vitiligo. Most of our patients were in generally good health, showing no association of vitiligo with thyroid dysfunction, diabetes mellitus, pernicious anemia nor gonadal failure. Localized form of vitiligo most frequently affects 21 to 28-year-old patients, while generalized form prevails in the age group from 29 to 36 years. Patients older than 77 years of age are very rarely affected. The most common localized type was focal (93,28%), and the most common generalized type was vitiligo vulgaris (53,7%). Most of our patients were admitted in September, probably due to increased contrast between involved and uninvolved skin. Most patients attribute the onset of their disease to specific life events (physical injury, emotional distress, illness or pregnancy).AIMSThe aim of this case report was to present our patient suffering from vitiligo vulgaris, and to evaluate clinical presentation, diagnostic and therapeutic difficulties in this condition. CASE REPORTA 23-year-old Caucasian female patient was admitted to our Hospital in September 2010, due to prominent, generalized depigmented patches. The disease begun acutely, “over night” (in patients` own words), and progressed in the following order: dorsal aspects of hands, upper extremities, trunk, face and lower extremities. Within a few weeks 60% of her body was affected.On admission to our Hospital her height was 175 centimeters and weight was 60 kilograms, body mass index was normal (19,6). The affected area had no associated scaling. There was a lack of cutaneous induration or sclerosis. Skin lesions were asymptomatic and lack clinical sings of inflammation. During dermatological examination leukotrichia of the occipital area was found. There was no mucosal involvement. She was without any subjective difficulties (e.g. pain, fever, weight loss). There was family history of this disorder (her 12-year-old brother has acrofacial vitiligo). The disease appeared almost simultaneously with his sister`s condition. She attributed the onset of her disease to emotional stress, while her parents died in a car accident six months before she has noticed first signs of vitiligo.She had also noticed new depigmented lesions in sites of physical injury (Koebner phenomena). History of chronic sun exposure was negative. She was non-smoker.Illumination with Wood`s lamp showed no fluorescence of the affected depigmented skin.CL has been diagnosed based on the clinical picture and pathohistological appearance. - Disclaimer- This PPT is loaded as student material "as is", from the VRF Vitiligo Master Class Barcelona November 2011; VRF does not endorse or otherwise approve it.
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VITILIGO IN CROATIAVedrana Bulat & Mirna Šitum
Department of Dermatovenereology University Hospital Center ,,Sestre milosrdnice’’
INTRODUCTION
0 10 20 30 40 50 60 70 80 90 100
4.437.460 (2001.)
%
1,6% ~ 71.000 with vitiligoDepartment of Dermatovenereology has given treatment to over 300 patients.
Croatia population
INTRODUCTION
FEMALE53,95%
MALE46,05%
Source: Department of Dermatovenereology University Hospital Center ,,Sestre milosrdnice’’
MALE – FEMALE RATIO - no difference in the severity of vitiligo
0
20
40
60
80
100%
INTRODUCTION
Most of our patients were in generally good health, showing NO association of vitiligo with:
thyroid dysfunction
diabetes mellituspernicious anemia
gonadal failure
Addison’s disease
INTRODUCTION
LOCALIZED FORM OF VITILIGO
(most from 21 to 28)
GENERALIZED FORM OF VITILIGO
(most from 29 to 36)
PATIENTS (>77 years)very rarely affected
INTRODUCTION
JANUARY JUNE DECEMBER
Most of our patients were admitted in September,
probably due to increased contrast between involved and uninvolved skin.
VITILIGO IN CROATIA – A CASE REPORT
AGE: 23
RACE: Caucasian
GENDER: Female
ADMITTED TO HOSPITAL: September 2010.
DUE TO: prominent, generalized depigmented patches
VITILIGO IN CROATIA – A CASE REPORT
Lesion borders were convex as if the depigmenting
process was invading the surrounded pigmented
skin.
VITILIGO IN CROATIA – A CASE REPORT
There was a lack of cutaneous induration or sclerosis.
The affected area had no associated scaling.
Skin lesions showed no sings of inflammation.
VITILIGO IN CROATIA – A CASE REPORT
The disease begun acutely, “over night” (in patients’ own words), and progressed in the following order:
1
dorsal aspects of hands
2
upper extremities
3 trunk
4
face
5 lower extremities
VITILIGO IN CROATIA – A CASE REPORT
+ brother
There is family history of vitiligo (her 12-year-old brother has acrofacial vitiligo).
The disease appeared almost simultaneously with his sister’s condition.
VITILIGO IN CROATIA – A CASE REPORT
ONSET: emotional stress (her parents died in a car accident six months before she noticed first signs of vitiligo).
0%
50%
100%
60%
Koebner phenomena positive
Within a few weeks 60% of her body was affected.
VITILIGO IN CROATIA – A CASE REPORT
Obtained abdominal ultrasound was without significant changes.
Ocular fundus on ophthalmological examination showed the absence of depigmented lesions.
She had no hearing problems.
Serum level of tumor markers:
- CA 19-9,- CEA,- CA 125,- CA 15-3,- CYFRA 21-1 was normal as well
VITILIGO IN CROATIA – A CASE REPORT
Serology for Borrelia burgdorferi in our patient was negative.
Cytomegalovirus serology and Epstein-Barr virus-specific serologies were negative.
HLA typing was positive for HLA A2, B51; B62; DRB1; DRB4; DR5; DR11; DR13; DR52; DQ1 and 3.
VITILIGO IN CROATIA – A CASE REPORT
In addition, our patient had autoimmune endocrinopathies.
Type I, insulin-dependant diabetes mellitus was diagnosed when she was 10 years old.
Serum free T4 levels were low and thyroid-stimulating hormone (TSH) level was elevated.
Antimicrosomal antibodies were detected as well.
Ultrasound imaging of the thyroid gland revealed diffuse enlargement of the thyroid gland, so she was started on levothyroxine for hypothyroidism.
VITILIGO IN CROATIA – A CASE REPORT
Skin-homing, melanocyte-specific T lymphocytes (CTLs) were detected in the periferal blood of our patient, strongly implicating their involvement in the destruction of melanocytes.
These CD8+ cells demonstrated specific cytotoxic responses against Melan A/MART-1, tyrosinase and gp 100.
Presence of HLA-A2-restricted, melanocyte-specific CD8+ T lymphocyte cells directed against Melan A/MART-1 may explain vitiligo in our patient as a T-cell-mediated autoimmune disease.
On admission, excisional biopsy of depigmented and clinically uninvolved skin has been performed.
A CASE REPORT: RESULTS
Oral mini-pulse corticosteroid therapy (dexamethasone 5 mg for 2 consecutive days for 6 months) has shown the ability to stabilize the disease with no exacerbations.
Topical class III corticosteroids were used for depigmented patches on her arms for two months, several follicular repigmentations were seen.
SIDE-EFFECTS: atrophy and telangiectasiae have been observed.
A CASE REPORT: RESULTS
Topical 0,1% tacrolimus ointment was used for face and intertriginous areas, but with no success observed.
A CASE REPORT: RESULTS
Narrowband UVB was applied for six months (48 exposures) with neither perifollicular repigmentation pattern nor repigmentation from the periphery of lesions.
The starting dose was 250 mJ/cm2 with increments of 10% at each subsequent exposure. Treatments were administered two times per week, but never on two consecutive days.
A CASE REPORT: SIDE-EFFECTS
Short-term side effects included pruritus and xerosis.
Two months after phototherapy she developed clinically dysplastic nevus on her back, and histopathologic analysis of the excised skin lesion has shown characteristic histologic features of a dysplastic nevus.
COMMENTS- TREATMENT SUGGESTION
Dendritic cells mediate interactions between innate and specific immunity.
Modulation of innate and adaptive immune responses by TOFACITINIB (CP-690,550)- Janus kinase inhibitor
TOFACITINIB reduces the number of Langerhans cells and cutaneous CD3+, CD4+ and CD8+ T lymphocytes and
alters their antigen-presenting function
Langerhans cell
COMMENTS-TREATMENT SUGGESTION
JAK3JAK1
JAKinhibitors
Downstream signaling pathways
STATS
Lymphocyte
Activation
Proliferation
Function
Cytokinereceptor
cץ
IL2 IL-4 IL-7 IL-9 IL-15 IL-21
Innate immunity mechanisms do not require the presence of the disease-causing antigen, even in T- and B-cell specific diseases.
TAFOCITINIB prevents the activation of STAT1, and inhibits the production of inflammatory mediators and subsequent generation of Th1 cells.
COMMENTS- TREATMENT SUGGESTION
TAFOCITINIBmay improve autoimmune diseases by
suppressing the differentiation of pathogenic
suppressing theinnate immune cell signaling
Th1cell
Th17cell
Th1
Th17
ICS
TAFOCITINIBTh1
Th17
ICS
QUESTIONS
1. What is leukotrichia?
a. depigmentation of hair
b. depigmentation of nails
c. depigmentation of teeth
2. When does vitiligo have its onset?
a. before age 20
b. after age 60
c. after age 70
