Depigmented Macules and Patches on Lateral Back

Vitiligo is a disease of skin depigmentation that was first described in Vedic Sanskrit and ancient Egyptian texts before 1500 BC.1 Many believe the term derives from the Latin vitium, meaning blemish or defect. Descriptions of vitiligo also can be...

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Vitiligo is a disease of skin depigmentation that was first described in Vedic Sanskrit and ancient Egyptian texts before 1500 BC.¹ Many believe the term derives from the Latin vitium, meaning blemish or defect. Descriptions of vitiligo also can be found in the Koran, Buddhist scripts, and Shinto prayers. Throughout history, vitiligo has been confused with other disorders, such as leprosy, resulting in stigmatization of those with the condition.¹

Major advancements in our understanding of vitiligo came in the middle to late 19th century. Moritz Kaposi described the histopathology of vitiligo, noting a lack of pigment granules in the deep epidermis, and Pierre Louis Alphée Cazenave discovered the association between alopecia areata and vitiligo.¹ Since then, much research has been conducted on vitiligo, but many questions about the condition remain unanswered.

The estimated prevalence of vitiligo is 0.5% to 2% worldwide, making it the most common depigmenting skin disorder.² The condition affects all ethnic groups and skin types with no predilection, and it occurs in men and women equally. It usually presents in individuals aged 10 to 30 years, but can occur at any age; nearly 50% of vitiligo patients present by 20 years of age, and 70% to 80% of patients present by 30 years of age.²

Vitiligo is caused by a loss or destruction of melanocytes, the cells responsible for making melanin, but the etiology is not completely understood. Multiple mechanisms have been proposed, including genetic, autoimmune, oxidative stress, innate and adaptive immune response, and melanocyte detachment mechanisms. Evidence suggests that there is an autoimmune component to vitiligo, but no single theory explains all observed phenotypes.² Multiple mechanisms likely contribute to melanocyte destruction in patients with vitiligo.²

Family history is a major risk factor for vitiligo and approximately 1 in 5 patients have a first-degree relative with the condition. Having a first-degree relative with vitiligo increases an individual’s relative risk for having vitiligo 7- to 10-fold. Large genome-wide association studies have found almost 50 genetic loci associated with vitiligo. Patients with autoimmune diseases such as alopecia areata, rheumatoid arthritis, and Addison disease also are at an increased risk for vitiligo.²

The development of vitiligo also has been associated with immunotherapy for the management of melanoma and radiation therapy for other cancers. One study from Korea found that recipients of hematopoietic cell transplants had a 3-fold increased risk for vitiligo compared with control group participants.³

Vitiligo presents as asymptomatic, chalky-white macules and patches with distinct margins.^2,4 Vitiligo can affect any part of the body, but typically affects the face (periorificial and lips), dorsal hands, tips of distal extremities, and genitalia.² Lesions may be more than one color, as seen in trichrome lesions, and may present in areas of friction or recent trauma (Koebner phenomenon).⁴ Depigmented hair also may be found within the lesions.^2,5 Severe sunburns, pregnancy, trauma, and/or emotional stress may precede the onset of new lesions.⁵ The clinical course is unpredictable and varies with each person.⁴

Under Wood’s lamp, vitiligo lesions emit a bright blue-white fluorescence that is sharply demarcated. No characteristic laboratory findings are associated with vitiligo. Skin biopsy of lesions shows complete loss of melanin pigment in the epidermis and an absence of melanocytes. Lymphocytes also may be seen at the advancing border.²

The differential diagnosis for vitiligo is broad because many conditions can present with depigmentation. Disorders commonly mistaken for vitiligo include nevus depigmentosus, tinea versicolor, postinflammatory hypomelanosis, idiopathic guttate hypomelanosis, and chemical leukoderma. History and physical examination can help differentiate most cases. For example, nevus depigmentosus typically presents at birth or in the first year of life and usually displays a normal number of melanocytes with reduced melanin production. Under Wood’s lamp, the difference between lesional and normal skin is less marked in nevus depigmentosus. Lesions of tinea versicolor usually have overlying scale, which is not present in vitiligo. In postinflammatory hypomelanosis, pigment changes are preceded by an active condition, such as psoriasis or atopic dermatitis.^2,4

The diagnosis of vitiligo usually is straightforward and based on characteristic history and clinical findings of vitiligo. Inspection under Wood’s lamp can differentiate between disorders of hypopigmentation and depigmentation. Dermoscopy also can aid in the diagnosis, usually showing residual perifollicular pigmentation and telangiectasia, which is absent in other hypopigmentation disorders.² Patients who are newly diagnosed with vitiligo may need to have blood work performed if it is indicated by their clinical history and review of systems. Biopsy is necessary only if other disorders must be excluded. ^2,4

Management of vitiligo depends on multiple factors, such as patient motivation for treatment, disease activity and extent, age, and phototype.² The face, neck, trunk, and proximal extremities are most responsive to treatment, whereas the lips and distal extremities are more resistant.² First-line treatments for vitiligo are topical corticosteroids and topical calcineurin inhibitors. Calcineurin inhibitors are useful for areas at high risk for skin atrophy.⁶

Phototherapy is a second-line treatment commonly used in vitiligo. Narrowband UV-B has shown to be superior over psoralen and UV-A.⁶ Targeted phototherapy using excimer lasers also has shown efficacy in the treatment of vitiligo.² Use topical medications combined with phototherapy has shown improved outcomes compared with those seen with monotherapy.⁶ Systemic steroids are used urgently in patients with rapidly progressive disease.² Additional treatment options include cosmetic camouflage with foundation and self-tanning products.²

Multiple surgical options are available for treatment in stable vitiligo cases unresponsive to topical treatments and light therapy. Surgical options include tissue grafts, cellular grafts, and hair follicle transplantation. In severe, widespread, and recalcitrant cases, depigmentation of normal skin with monobenzyl ether of hydroquinone (monobenzone) may be considered. Emerging therapies undergoing further investigation include afamelanotide (alpha-melanocyte stimulating hormone agonistic analog), prostaglandin E2, bimatoprost, and Janus kinase inhibitors.² Psychological support and counseling should be offered to all patients because vitiligo can significantly affect quality of life.^2,4

The patient in this case was diagnosed with vitiligo based on clinical history and examination. He has been using daily topical corticosteroids for several months and is showing signs of repigmentation.

Christopher Nguyen, BA, is a medical student at Baylor College of Medicine in Houston, Texas; Tara L. Braun, MD, is a resident in the Department of Dermatology at Baylor College of Medicine; and Christopher Rizk, MD, is a dermatologist at Elite Dermatology in Houston.

References

1. Millington GWM, Levell NJ. Vitiligo: the historical curse of depigmentation. Int J Dermatol. 2007;46(9):990-995. doi:10.1111/j.1365-4632.2007.03195.x
2. Bergqvist C, Ezzedine K. Vitiligo: a review. Dermatology. 2020;236(6):571-592. doi:10.1159/000506103
3. Bae JM, Choi KH, Jung HM, et al. Subsequent vitiligo after hematopoietic stem cell transplantation: a nationwide population-based cohort study from Korea. J Am Acad Dermatol. 2017;76(3):459-463. doi:10.1016/j.jaad.2016.08.064
4. Ezzedine K, Lim HW, Suzuki T, et al. Revised classification/nomenclature of vitiligo and related issues: the Vitiligo Global Issues Consensus Conference. Pigment Cell Melanoma Res. 2012;25(3):E1-E13. doi:10.1111/j.1755-148X.2012.00997.x
5. 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;65(3):473-491. doi:10.1016/j.jaad.2010.11.061
6. Ezzedine K, Whitton M, Pinart M. Interventions for vitiligo. JAMA. 2016;316(16):1708-1709. doi:10.1001/jama.2016.12399