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Spotting the Diagnosis of Vitiligo

by Jacob Mathew, Jr. DO FACOI FACP CHSE FAWM

Your patient

A 21-year-old male with a past medical history significant for diabetes mellitus type 2 and hypothyroidism presents to you for white spots on both his arms. He first noted them approximately 1 year ago and they have been progressively enlarging since then. They do not itch, and he has not noticed any other symptoms. They are embarrassing to him and as a result, he often wears long sleeve shirts when going out in public. He hopes that you can tell him what it is and what can be done to help him regain confidence in going out in public now that beach season has arrived.

vitiligo_dark_skin_hands_nonseg.jpgSo, what is vitiligo?

Vitiligo is an acquired pigmentary disorder with a genetic predisposition in which melanocytes progressively lose their function, resulting in abnormal skin hypopigmentation (white macules and patches) throughout the body1,2. The exact pathophysiology of vitiligo remains unknown but multiple hypotheses exist3. While it is not associated with life-threatening outcomes, patients may suffer from significant psychosocial stress and altered quality of life due to their appearance, especially in those with dark skin2.

Vitiligo is divided into either segmental or nonsegmental. Segmental vitiligo presents as asymmetric/unilateral white patches that may partially or fully match a dermatome or a Blaschko line (see Figures 1 and 2)4,3. Nonsegmental, the most common manifestation and seen in up to 90% of patients with vitiligo, is described as white symmetric/bilateral patches that first occur during childhood in areas of previous trauma and increase in size over time3


Figure 1 Blaschko lines on right vs dermatomes on left3

 blaschko_lines.png

 

Figure 2 The appearance of segmental vs nonsegmental vitiligo

Segmental

Nonsegmental

vitiligo_segmental.jpgvitiligo_torso_nonseg.jpg

 

If it's not vitiligo, what else could it be?

Pigmentation disorders are best divided into hyperpigmented and hypopigmented conditions. Hypopigmented disorders include vitiligo, pityriasis rosea, and tinea versicolor. Hyperpigmented disorders include postinflammatory hyperpigmentation (such as acne, atopic dermatitis), melasma, and solar lentigos, among others.


Table 1 Pigmentation disorders

 
Pathophysioloy
Description
Treatment
Hyperpigmentation
   
Postinflammatory pigmentation
Commonly due to acne, dermatitis (atopic vs contact), lichen planus, trauma, mediccations. Most often seen in those with dark skin. Lesions may last for months to years.Often irregularly shaped, darkly pigmented macules or patches.Can be difficult, prolonged; Hydroquinone 3-4% and azelaic acid are effective.
Melasma
Associated with chronic UV exposure (commonly seen on the face) and hormonal fluctuations (hyperandrogenism)Irregularly bordered, evenly pigmented tan macules on the face.Hyrdoquinone 4% is the first-line treatment; effective when used with tretinoin 0.05% and fluocinolone acetonide 0.01%.
Solar lentigos
Uncertain. Thought to be due to UVR-induced proliferation of the epidermal keratinocytes resulting in excess production of melanocytes, leading to abnormal pigment retention.Benign pigmented, smooth or irregular, brown to black macules on sun-exposed skin.Sun protection, cryotherapy, phenols, and retinoids
Hypopigmentation
   
Pityriasis rosea
Unclear pathophysiology. May be related to reactivation of human herpesvirus 6 and 7. Has also been associated with many commonly prescribed medications.Asymptomatic macule (herald patch) that precedes the development of circular patches and plaques along skin folds.Self-limiting' antihistamines and mid-potency corticosteroids may hasten pruritis.
Tinea versicolor
Benign non-contagious, non-pruritic, superficial fungal infection due to the Maleziassa genus often associated with heat and high humidity exposure.Macules, papules, patches of varying pigmentation in the upper trunk and arms, neck, and face.Typically responds to short-course topical corticosteroids or oral antifungals.

 

vitiligo_legs_nonseg.jpgHow am I going to diagnose it?

Luckily, biopsy is not required for the diagnosis, only an astute physical exam is.

Is vitiligo associated with any other concurrent conditions?

While the pathophysiology is uncertain, the most widely accepted cause of vitiligo remains autoimmune in nature, given studies that have shown the presence of auto-antibodies against melanocytes and autoimmune conditions seen in up to 23% of vitiligo patients1. Furthermore, many of the therapeutic options for vitiligo focus on immunosuppression. Of the associated autoimmune disorders, the most common remain hypothyroidism, rheumatoid arthritis, alopecia areata, diabetes mellitus, and systemic lupus erythematosus, which do appear to have a racial variation (see Table 2)1. Therefore, if evaluating a patient with vitiligo, be cognizant of other autoimmune conditions that may be present and the requirement diagnostic evaluation required for each. Screening laboratory assessment for asymptomatic patients is not recommended.


Table 2 Common autoimmune conditions seen with vitiligo

Hashimoto thyroiditis
Rheumatoid arthritis
Systemic lupus erythematosus      
Addison disease
Scleroderma
Dermatomyositis
Alopecia areata
Inflammatory bowel disease
Diabetes mellitus
Psoriasis
Pernicious anemia
 


How can I treat vitiligo?

Unfortunately, as with most pigmentary disorders, vitiligo can be challenging to treat, and many options may not provide completely satisfactory results. Options vary depending on the amount of skin involvement, varying from systemic to topical (see Table 3). One of the safest and effective treatments is phototherapy, comprised of narrow-band ultraviolet-B therapy (NB-UVB) and psoralen plus ultraviolet A (PUVA)5. Both UVA and UVB are the first-line treatments for vitiligo affecting more than 10-20% of skin3. They work via both immunosuppression and melanocyte proliferation3. As a provider, you may be more familiar with PUVA, as it has been around longer than other phototherapies. However, its side effect profile including nausea, vomiting, and carcinogenic potential has caused it to fall out of favor among dermatologists. An advantage to phototherapy is that home-treatment options are available, which will lead to increased patient compliance6. The pharmacologic treatments include topical steroids (best for localized vitiligo) and topical calcineurin inhibitors (causes less skin atrophy but a higher cancer risk due to immunosuppression). Synthetic topical vitamin D3 analogs directly inhibit T-cell function and can help with melanocyte maturation and halt further loss7. More extreme treatments involve surgical intervention and are often reserved for those who have had stable lesions for over 2 year and have failed to respond to medical therapy8.


Table 3 Treatments for vitiligo8

Treatment
Description
Side Effects
Topical
  
Tacrolimus
Calineurin inhibitor, 53% repigmentation rate in patients with facial vitiligo9.Minimal
Clobetasol
Approximately 50% repigmentation rate in patients with facial vitiligo9Minimal 
Mometasone
65% repigmentation rate. Atrophy, telangiectasias, erythema 
Pimecrolimus 
Calcineurin inhibitor, 42% repigmentation rate.Burning sensation, pruritis 
Systemic 
  
Methotrexate
Equally as effective as systemic corticosteroidsNausea 
Dexamethasone 
Systemic corticosteroids are generally employed in rapidly progressive cases to help with disease stabilization. Small doses (2.5 mg/day) for 2 days/weeks can be effective. Weight gain, acneiform eruption, headache, weakness 
Minocycline
An antibiotic that has been found to be equally effective as steroids when given for 6 months. Often prescribed in 100 mg/day doses for patients with progressive, slowly spreading vitiligo. Facial hyperpigmentation, mucosal hyperpigmentation, nausea, vomiting 
Physical: UV Light Therapy
  
PUVA
Was originally an option for phototherapy but has largely been replaced with NB-UVB.Nausea, vomiting, phototoxicity, carcinogenic
NB-UVB 
Greater repigmentation rates; may also help in patient with low vitamin D levels. Results seen after 18-36 sessions. New home-therapy administration options available.Cutaneous carcinogenic effects have been hypothesized but no studies have proven a link 
Monochromatic excimer light therapy (MEL)10
Monotherapy has shown up to 75% improvement in 50% of patients in 2 weeks; can be combined with other treatments.Low side effect profile; most common adverse reaction was erythema 
Treatments on the Horizon
  
Afamelanotide
Analog to alpha-melanocyte-stimulating hormone given as a subcutaneous implant. Best studied in nonsegmental vitiligo, which showed superior results when combined with UVB therapy than UVB therapy alone. Nausea, increased libido, spontaneous erections, erythema 
Melanocyte-keratinocyte transplant procedure
First described in 1992, involves the transplantation of epidermal cells (often from the lateral thigh) from a donor and conversion into a suspension that can then be used in a recipient. Results often start within 2-8 weeks postoperatively and take up to 18 months for full repigmentation to occur. Surgical site infection, pain, bleeding. Graft-vs-host phenomena not reported.

Successful responses were those with greater than 50% repigmentation.

 

References

1. Dahir AM, Thomsen SF. Comorbidities in vitiligo: comprehensive review. Int J Dermatol. 2018 Oct;57(10):1157-64.

2. Zubair R, Lyons AB, Vellaichamy G, Peacock A, Hamzavi I. What's New in Pigmentary Disorders. Dermatologic Clinics. 2019 Apr;37(2):175-81.

3. Guerra L, Dellambra E, Brescia S, Raskovic D. Vitiligo: Pathogenetic Hypotheses and Targets for Current Therapies. CDM. 2010 Jun1;11(5):451-67.

4. Bolognia JL, Orlow SJ, Glick SA. Lines of Blaschko. Journal of the American Academy of Dermatology. 1994 Aug;31(2):157-90.

5. Scherschun L, Kim JJ, Lim HW. Narrow-band ultraviolet B is a useful and well-tolerated treatment for vitiligo. J Am Acad Dermatol. 2001 JUN:44(6):999-1003.

6. Khanna U, Khandpur S. What is New in Narrow-Band Ultraviolet-B Therapy for Vitiligo? Indian Dermatol Online J. 2019;10(3):234-43.

7. AlGhamdi K, Kumar A, Moussa N. The role of vitamin D in melanogenesis with an emphasis on vitiligo. Indian J Dermatol Venereol Leprol. 2013 Dec:79(6):750-8.

8. Dillon AB, Sideris A, Hadi A, Elbuluk N. Advances in Vitiligo: An Update on Medical and Surgical Treatments. J Clin Aesthet Dermatol. 2017 Jan;10(1):15-28.

9. A double-blind, randomized, placebo-controlled trial of topical tacrolimus 0.1% vs. clobetasol propionate 0.05% in childhood vitiligo. - PubMed - NCBI [Internet]. [cited 2019 Jun 19]. Available from: https://www.ncbi.nlm.nih.gov/pubmed/21457214.

10. Park KK, Liao W, Murase JE. A review of monochromatic excimer light in vitiligo. Br J Dermatol. 2012 Sep;167(3):468-78.

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