Michael D. TharpThe Clark W. Finnerud, M.D.Professor and ChairDepartment of DermatologyRush University Medical CenterChicago, Illinois, USA
Most common pigmentary disorder Patchy loss of pigment from the skin, hair and oral mucosa Reported frequency 0.1-2.0% in various populations Familial tendency with approximately 20% of probands having at least one affected first degree relative Autoimmune mediated?
Segmental Clinical FeaturesZosteriformis macules distributed along a dermatomeNon Segmental Clinical FeaturesLocalized or partial few scattered maculesAcrofacialis macules localized to face, distal hands and feetMucosal macules on mucous membranesGeneralized more widespread macules (face, hands, feet, axillae, limbs)
A questionnaire was given to 2546 pts with vitiligo from the United Kingdom and U.S. Vitiligo pt groups: Vitiligo Society: UK National Vitiligo Society: USA
A questionnaire was given to 2546 pts with vitiligo from the United Kingdom and U.S. Approx 70% female 18% of first degree relatives had vitiligo 19.4% of probands had autoimmune thyroid disease (88% hypothyroidism and 12% hyperthyroidism) 5.7% of first degree relatives with autoimmune thyroid disease
1.9% had pernicious anemia 13 fold increase over the general population 0.38% with Addison’s disease 76 fold increase over the general population 8 fold increase in SLE 2 fold increase in Crohn’s disease No increase in: alopecia areata diabetes mellitus multiple sclerosis myasthenia gravis psoriasis RA Scleroderma Sjogren’s synd.
CONCLUSIONS Vitiligo is associated with other autoimmune disorders Thyroid disease and pernicious anemia were frequent Addison’s dis, SLE and inflammatory bowel dis uncommon The above disorders also increased in probands’ first degree relatives: suggesting shared common susceptibility genes
204 with vitiligo (0.61%) were obtained from 33,252 medical records: 66% pts: localized vitiligo 15% with generalized vitiligo 13% with acromucosal vitiligo 6% with segmental vitiligo Autoimmune disorders were found in 6 (2.9%) of patients
113 vitiligo pts were tested for diseases associated with polyglanduar autoimmune syndrome (APS) 58% generalized vitiligo 38% acrofacial vitiligo 3% segmental vitiligo
22/113 pts with vitiligo had 3 or more autoimmune disorders (APS) Thyroid disease was common (39%) Addison’s disease rare (1/113) but higher frequency than the reported general population ANA positivity was seen in 3% of pts which is typical for the general population Recommend periodic screening of vitiligo pts for other autoimmune diseases
156 pts with vitiligo underwent an eye exam 40% had some fundal abnormality Transillumination of the iris Whites 23% Blacks 5% Fundiscopic findings Focal pigment hypertrophy (18%) Hypopigmented spots (9%) Retinal scars (6%) Chorodial nevi (4%) Uveitis (1%): inflammation rarely seen
Uveitis has been reported to be another ocular abnormality associated with vitiligo Vogt-Koyanagi_Harada syndrome (3 phases) Meningocephalic phase ( headache, meningismus, seizures and/or muscle weakness or paralysis) preceded by fever, nausea/vomiting : aspectic meningitis Acute ophthalmic phase (eye pain, photophobia, altered visual acuity): uveitis, iriditis, retinal detachment Otic involvement (dysacousia) Poliosis Vitiligo Alezzandrini syndrome Whitening of the hair, eyebrows and eyelashes Unilateral depigmentation of the skin on the forehead, nose, cheek, upper lip and chin along with decreased visual acuity and atropic iris Non-inflammatory depigmented lesions in the fundus seen in vitiligo
Thyroid diseaseDiabetes mellitusAdrenal insufficiencyLupus erythematosusAlopecia areataMyasthenia gravisPernicious anemiaRheumatoid arthritisSarcoidosisChronic active hepatitisVogt–Koyanagi–HaradasyndromePsoriasisLichen planusC2 and C4 deficiencyAPS, autoimmunepolyglandular syndromes.