Successful Treatment of Nonsegmental Periorbital Vitiligo

Successful Treatment of Nonsegmental Periorbital Vitiligo

Case author Stephen W. Lewellis, MD, PhD, FAAD, presents a case of a 38-year-old female with loss of pigmentation on her forehead, cheeks, and eyelids who did not respond to prior treatment with a topical corticosteroid or calcineurin inhibitor.

By Stephen W. Lewellis, MD, PhD, FAAD

A 38-year-old White female presented to the dermatology clinic for new patient evaluation and management of a chief complaint of loss of pigmentation on her forehead, cheeks, eyelids, hands, left arm, left foot, and axillae that she first noticed about 2 years prior to presentation. It started on her dorsal hands and gradually progressed to other areas. She noticed new areas of involvement appearing over time. 

She denied itching, burning, or other symptoms in the areas of concern. All the areas were distressing to her, most notably the area around her eyes. This made her feel self-conscious and embarrassed, making it difficult to carry herself confidently in various areas of her life. She was otherwise feeling well but was worried that depigmentation would continue to spread.

Physical Exam, Lab Results and Medical History 

Physical examination revealed depigmented macules and patches on the dorsal hands, left forearm, left dorsal foot, bilateral axillae, forehead, bilateral upper and lower eyelids and periorbital area, and lateral cheeks. There was no erythema within the depigmented areas. Skin temperature in the affected areas felt normal. Total body surface area affected was approximately 2%. 

Prior to presentation to the dermatology clinic, she tried triamcinolone 0.1% ointment at the recommendation of her primary care physician and did not notice any improvement. 

She could not recall any significant changes in medications or personal care products in the months preceding the onset of the skin changes. She denied family history of similar skin changes. She could not identify any exacerbating or alleviating factors. 

The patient denied a history of atopy or autoimmune disease. 

The patient’s thyroid stimulating hormone level had been checked several months prior and was within normal limits. Other lab tests and skin biopsy were not felt to be indicated for this patient.

Diagnostic Review 

There are several key considerations that healthcare providers should keep in mind when diagnosing vitiligo. 

Inquire about any previous skin diseases, autoimmune disease, or family history of vitiligo. Vitiligo is sometimes associated with other autoimmune diseases such as thyroid disorders, type I diabetes mellitus, and alopecia areata. Identifying these associations can provide valuable insight into the underlying cause of the skin changes and can help raise awareness about other diseases for which the patient may be at risk. 

A thorough exam of the patient’s skin, including at least inquiring about the genital skin and examining it if the patient is comfortable. 

In skin affected by vitiligo, illumination with a Wood’s lamp in an otherwise dark room will accentuate the contrast between the pigmented and depigmented areas, which helps confirm the diagnosis. This can also help identify smaller, more subtle areas that may just be starting to develop. 

It is important to educate the patient about the chronic nature of vitiligo and the absence of a cure. Offer support and referral to a mental health professional if appropriate. Emphasize the availability of treatments for vitiligo that can make it much less noticeable as well as coping strategies to help manage the emotional aspects of living with vitiligo. 

Additionally, advise the patient about the importance of sun protection for the depigmented areas as they are more susceptible to sunburns and DNA damage that can lead to skin cancers than the areas of normal skin. 

Vitiligo can be mistaken for other conditions such as pityriasis alba, tinea versicolor, and post-inflammatory hypopigmentation.

What is the maximum body surface area and grams per week that is recommended when using ruxolitinib 1.5% cream for nonsegmental vitiligo?

Before & After Photos
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BEFORE: Depigmented macules on the forehead, bilateral upper and lower eyelids and periorbital area, and lateral cheeks
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BEFORE: Depigmented macules on the forehead, bilateral upper and lower eyelids and periorbital area, and lateral cheeks
Click to enlarge image
BEFORE: Depigmented macules on the forehead, bilateral upper and lower eyelids and periorbital area, and lateral cheeks
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AFTER: Significant repigmentation on the forehead, bilateral upper and lower eyelids and periorbital area, and lateral cheeks after treatment with ruxolitinib 1.5% cream over 6 months
Click to enlarge image
AFTER: Significant repigmentation on the forehead, bilateral upper and lower eyelids and periorbital area, and lateral cheeks after treatment with ruxolitinib 1.5% cream over 6 months
Click to enlarge image
AFTER: Significant repigmentation on the forehead, bilateral upper and lower eyelids and periorbital area, and lateral cheeks after treatment with ruxolitinib 1.5% cream over 6 months

Treatment Discussion and Outcome 

Treatment options for vitiligo aim to promote repigmentation of the affected skin by suppressing the immune response that is destroying the melanocytes. Treatment outcomes vary, and complete repigmentation is not always achievable, especially in cases of segmental vitiligo. Options include topical corticosteroids, topical calcineurin inhibitors (safer for sensitive areas such as the face and genital skin), topical Janus kinase (JAK) inhibitors, light-based therapies (eg, NB-UVB, excimer laser, PUVA), and surgical interventions such as autologous melanocyte transplantation. 

Topical steroids and calcineurin inhibitors are typically the most cost effective and accessible treatments, so they are often considered first-line treatments. Phototherapy is safe and effective but requires travel to and from a clinic 2 to 3 times per week for several months or acquisition of a home phototherapy booth. Recently, a topical JAK inhibitor was approved for treatment of nonsegmental vitiligo. 

In this case, the patient had already tried a topical corticosteroid without relief and had extensive periorbital and eyelid involvement, which makes topical corticosteroid use not ideal. A topical calcineurin inhibitor (tacrolimus 0.1% ointment) was then recommended. Unfortunately, she had no appreciable improvement after 3 months of twice daily use. 

At that point, ruxolitinib 1.5% cream, a topical JAK inhibitor that is indicated for nonsegmental vitiligo, was prescribed to be applied twice daily to affected areas. There are boxed warnings associated with this medication, but they are likely only relevant to certain oral rather than topical JAK inhibitors, especially when the amount of cream used is limited as directed. 

She returned approximately 6 months later feeling excited and optimistic about the amount of repigmentation, especially around her eyes and on her eyelids. She denied any side effects. Physical exam revealed significant repigmentation in the areas she had been treating, although there were still some patchy areas of depigmentation remaining. 

She had not yet started using it on other affected body parts but now planned to do so. She felt relieved and was excited to continue using this medication to try to achieve full repigmentation in the areas that cause her the most distress. The patient was appreciative and grateful for access to this treatment option.

Case Discussion

April Armstrong, MD, MPH Chief, Division of Dermatology David Geffen School of Medicine at UCLA Los Angeles, CA

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