Publish date: Posted on
Last updated: October 25, 2023
Keywords #
autoimmune
depigmentation
hypopigmentation
vitiligo
Diagnosis #
Vitiligo is an acquired skin condition characterized by sharply-demarcated depigmented macules and patches which is typically chronic in nature. The distribution can be either localized or widespread and often favors sites of frequent friction or trauma. The precise etiology remains controversial, but proposed hypotheses include a host attack on normal melanocytes and intrinsic melanocyte defects in genetically-predisposed individuals.[1] Psychosocial support is a cornerstone of management, as vitiligo can have devastating psychological effects for the patient and family. Spontaneous repigmentation can occur, but response to treatment is slow.[2]
Key Concepts #
- Vitiligo is a disorder of pigmentation manifesting as white macules and patches.
- Vitiligo is not an infectious condition.
- It can occur at any age, affects both sexes equally, and can be psychologically devastating.
- Repigmentation is often slow and incomplete.
- The cornerstone of management is halting progression and preventing further spread.
Epidemiology #
The prevalence of vitiligo ranges from 0.4 to 2.0% with regions of greater or lesser prevalence.[1] Most studies demonstrate slightly greater prevalence and earlier onset in females. Childhood vitiligo has been associated with an atopic diathesis, halo nevi, and family history of vitiligo and autoimmunity. Post-pubescent vitiligo has been associated with greater acrofacial disease and thyroid disease. Early data also support reduced non-melanoma and melanoma skin cancer risk in patients with vitiligo. Natural progression can be unpredictable – initial onset can be insidious or rapid and years of stable disease can be followed by a suddenly progressive trajectory.[3]
Clinical Features #
- Vitiligo appears as sharply-demarcated depigmented macules and patches.
- Leukotrichia (white hair) can be observed in hair-bearing areas.
- Lesions typically develop in areas of friction, reflecting koebnerization.
- Vitiligo is classified into localized, generalized, and universal forms.[4]
- Localized forms may be segmental or focal, neither of which crosses the midline.
- Mucosal involvement may be observed in generalized forms, which include vitiligo vulgaris (widely distributed macules and patches) and acrofacial (distal extremities and face).
- In vitiligo universalis >80% of the body surface area is depigmented.[1]
Clinical variants include:
- Inflammatory vitiligo describes erythema and raised borders at lesion margins.
- Trichrome vitiligo has a hypopigmented zone between normal and depigmented skin.
- Quadrichrome vitiligo has additional marginal or perifollicular hyperpigmentation.
- Vitiligo ponctué has punctate amelanotic macules on normal or hyperpigmented skin
- Blue vitiligo has a blue-grey hue because of the absence of epidermal melanocytes and presence of numerous dermal melanophages; it is often within an area of post-inflammatory hypopigmentation.
Differential Diagnoses #
- Nevus depigmentosus (aka nevus achromicus) is a hypopigmented non-scaly patch detectable in the first year of life that increases in size in proportion to the child’s growth.
- Nevus anemicus can look similar, but rubbing through a nevus anemicus yields erythema around it, not within it, reflecting increased vascular tone rather than an abnormality of pigmentation.
- Medication-induced or chemical leukoderma will often have a corresponding history of exposure to substances containing phenols and catechols.
- Discoid lupus erythematosus typically has other findings such as atrophy and telangiectasias
- Piebaldism is an autosomal dominant disease presenting at birth with anterior midline depigmentation and a white forelock.
- Lichen sclerosus presents as hypopigmented sclerotic and pruritic plaques in the genital area or non-genital skin.
- Onchocerciasis should be suspected if the lower extremities are involved and the patient is from an endemic region.
- Hypopigmentation rather than depigmentation may suggest alternative diagnoses such as pityriasis alba, tinea versicolor, post-inflammatory hypopigmentation, or idiopathic guttate hypomelanosis.[5]
Diagnostic Workup #
The diagnosis is typically made clinically and with the use of a Wood’s lamp to determine extent and activity of disease. To date, there is no standardized staging system for vitiligo. Association with autoimmune polyendocrinopathies or other autoimmune disorders such as thyroid disease and pernicious anemia should be evaluated in the appropriate clinical context.[1]
Biopsy is typically not needed, but histopathology can help confirm the diagnosis. Melanocytes are absent, and there is a scant inflammatory infiltrate. Active lesions may have a lichenoid interface dermatitis. Immunohistochemical staining would verify the complete absence of melanocytes in skin that may still have melanin granules within keratinocytes.[5]
Treatment #
First-line treatments include topical corticosteroids and topical calcineurin inhibitors, such as tacrolimus or pimecrolimus.[2] Topical calcineurin inhibitors in combination with phototherapy are thought to have the highest efficacy among current treatment options. Alternatives include other forms of phototherapy, such as psoralen plus UVA (PUVA), excimer laser, skin grafts, and topical vitamin D analogs. Emerging systemic therapies include janus kinase inhibitors and alpha-melanocyte-stimulating hormone analogs.[6]
Quality of life should be the foremost priority of treatment, and expectations should be set that improvement is often slow, and that certain areas, especially acral skin, can be recalcitrant to therapy.[2] Cover-up makeups, such as Dermablend products, can be useful for cosmetically sensitive areas.
Slide Viewer #
https://utahderm.med.utah.edu/image-viewer/References #
- 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;65:473-91.
- Felsten LM, Alikhan A , Petronic-Rosic V. Vitiligo: a comprehensive overview Part II: treatment options and approach to treatment. J Am Acad Dermatol 2011;65:493-514.
- Silverberg N. The Epidemiology of Vitiligo. Current Dermatology Reports 2015;4:36-43.
- Rodrigues M, Ezzedine K, Hamzavi I, Pandya AG , Harris JE. New discoveries in the pathogenesis and classification of vitiligo. J Am Acad Dermatol 2017;77:1-13.
- Ortonne J, Passeron T. Vitiligo and other disorders of hypopigmentation. In: J. J. Bolognia JL, Schaffer JV, editors. Dermatology. London: Elsevier Saunders; 2012. p. 1023-30.
- Rodrigues M, Ezzedine K, Hamzavi I, Pandya AG , Harris JE. Current and emerging treatments for vitiligo. J Am Acad Dermatol 2017;77:17-29.
