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vitiligo

Etiology: 1) presumed autoimmune in origin, anti-melanocyte 2) often seen in association with autoimmune disorders a) thyroid disease 1] Hashimoto's thyroiditis 2] Graves disease b) diabetes mellitus type-1 [3] c) Addison's disease d) pernicious anemia e) alopecia areata f) rheumatoid arthritis g) psoriasis [10] h) Vogt-Koyanagi-Harada syndrome - non-traumatic uveitis - aseptic meningitis 3) acquired condition, congenital cases are rare Epidemiology: 1) incidence is approximately 1% 2) affects both sexes equally 3) more noticeable in dark-skinned individuals 4) peak incidence between 10-30 years Pathology: 1) loss & absence of melanocytes 2) minimal inflammation Genetics: 1) 30% of patients have an affected family member 2) inheritance pattern is unclear 3) mutations in gene for F-box only protein-11 4) susceptibility to vitiligo is associated with variations in PTPN22 Clinical manifestations: 1) hypopigmented macules a) 1 mm to several cm in diameter b) generally white, but may be off-white or tan in color c) circular or oval in shape, may have scalloped edges d) accentuated by viewing with Wood's lamp, especially in light-skinned patients e) focal, segmental or generalized in distribution f) perifollicular skin may be spared [10] g) generalized vitiligo tends to occur in a symmetric pattern h) areas of frequent trauma, i.e. fingertips, bony prominences affected early i) common sites include 1] extensor surfaces a] knees b] elbows c] back of hands 2] body folds a] axilla b] genitalia 3] skin around orifices a] mouth b] eyes c] nostrils d] nipples e] umbilicus 4] previously sunburned areas or sites of skin trauma (Koebner phenomenon) 2) other skin manifestations a) leukotrichia b) halo nevi c) alopecia areata 3) iritis is seen in 10% of patients with vitiligo 4) signs associated with concomitant autoimmune disorder * images [5,6,7,10] Laboratory: 1) diagnosis is made by history & physical examination, laboratory testing is rarely needed 2) skin biopsy reveals normal skin except for an absence of melanocytes 3) screening tests for associated autoimmune disorders a) free T4 or TSI & serum TSH b) complete blood count (CBC) for pernicious anemia c) fasting blood sugar (serum glucose) for diabetes mellitus d) serum Na+, serum K+ & serum cortisol for Addison's disease 3) PTPN22 gene mutation Special laboratory: - lesions more apparent under Wood's lamp (chalk white) Differential diagnosis: 1) tinea versicolor 2) tuberous sclerosis 3) pityriasis alba 4) post inflammatory hypopigmentation 5) leprosy 6) systemic lupus erythematosus 7) chemical leukoderma 8) nevus anemicus Complications: - patients with vitiligo more likely to develop dementia (RR=5.3) [9] - Alzheimer's dementia (RR=12.2) - vascular dementia (RR=4.0) [9] Management: 1) primarily of cosmetic & social concern 2) tends to be stable initially, then progresses over several years 3) untreated vitiligo usually remains for life, but some individuals spontaneously repigment depigmented areas 4) sunscreens (SPF > 30) will diminish pigmentation of adjacent areas which make the vitiligo more noticeable 5) cosmetics may provide good results 6) pharmacologic agents a) topical steroids for isolated lesions 1] hydrocortisone 1% or 2.5% on face & skin folds 2] more potent steroids elsewhere 3] use interrupted schedule if steroids are used for more than 6-8 weeks to reduce risk of steroid atrophy b) topical PUVA photochemotherapy or oral psoralen for more extensive disease c) topical ruxolitinib (Opzelura) FDA-approved 7) depigmentation of normal skin with monobenzone (Benzoquin) a) permanent, uniform bleaching of remaining normal skin b) may be useful for patients with extensive disease

Related

F-box only protein 11; vitiligo-associated protein 1; VIT-1 (FBXO11, FBX11, VIT1, UG063H01) Vogt-Koyanagi-Harada syndrome (VKH)

General

pigmentation disorder

Database Correlations

OMIM 193200

References

  1. Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 952-53
  2. Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 296
  3. Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17. American College of Physicians, Philadelphia 2009, 2012, 2015
  4. Huggins RH, Schwartz RA, Janniger C. Vitiligo Acta Dermatovenerol Alp Pannonica Adriat. 2005 Dec;14(4):137-42, 144-5 PMID: 16435042
  5. Vitiligo (image) American Academy of Dermatology https://www.aad.org/public/diseases/color-problems/vitiligo
  6. Groysman V, Elston DM (images) Medscape: Vitiligo http://emedicine.medscape.com/article/1068962-overview
  7. DermNet NZ. Vitiligo (images) http://dermnetnz.org/colour/vitiligo.html
  8. Alikhan A, Felsten LM, Daly M, Petronic-Rosic V. Vitiligo: a comprehensive overview Part I. Introduction, epidemiology, quality of life, diagnosis, differential diagnosis, associations, histopathology, etiology, and work-up. J Am Acad Dermatol. 2011 Sep;65(3):473-491. Review. PMID: 21839315 - Felsten LM, Alikhan A, Petronic-Rosic V. Vitiligo: a comprehensive overview Part II: treatment options and approach to treatment. J Am Acad Dermatol. 2011 Sep;65(3):493-514. Review. PMID: 21839316
  9. Chang TH, Tai TH, Dai YX et al Association between vitiligo and subsequent risk of dementia: A population-based cohort study. J Dermatol. 2020. Nov 12 PMID: 33180352 https://onlinelibrary.wiley.com/doi/abs/10.1111/1346-8138.15582
  10. Elston CA, Elston MD Identifying Lesions on Skin of Color. Medscape. 2021. May 10 https://reference.medscape.com/slideshow/identifying-lesions-6007985 - Elston CA, Elston DM Identifying Lesions on Skin of Color. Medscape. October 25, 2022 https://reference.medscape.com/slideshow/identifying-lesions-6007985
  11. National Institute of Arthritis and Muscluloskeletal and Skin Diseases (NIAMS) Vitiligo https://www.niams.nih.gov/health-topics/vitiligo