Guideline sources The following summarized guidelines for the evaluation and management of vitiligo are prepared by our editorial team based on guidelines from the German Society of Dermatology (DDG 2022) and the British Association of Dermatologists (BAD 2022). 1 2 Guidelines 1. Diagnostic investigations Initial assessment: elicit a full history in patients with vitiligo including the site and type of vitiligo (segmental, nonsegmental), disease extent (affected BSA), disease stability, speed of onset, trigger factors, quality of life, psychological and psychosocial impact, and personal and family history of associated thyroid dysfunction or other autoimmune disease. E Screening for thyroid disease: As per BAD 2022 guidelines, screen for antithyroid antibodies and thyroid function in patients with vitiligo (including pediatric patients) to identify patients at high risk of developing autoimmune thyroid disease. E As per DDG 2022 guidelines: https://web.pathway.md/diseases/reciCMbY14Ckz5DTD 1/7 6/29/23, 3:44 AM Vitiligo Pathway Obtain TSH, anti-TPO antibody and anti-TG antibodies in the initial evaluation and then annually in patients with vitiligo, given the increased prevalence of thyroid diseases. E Consider obtaining additional screening tests for circulating autoantibodies only in case of relevant clinical and laboratory evidence. E Screening for vitamin D deficiency: Consider measuring serum vitamin D levels in patients with vitiligo avoiding all sun exposure. E Consider offering vitamin D3 supplementation (10-25 g/day) if levels are reduced or deficient, and advising increasing their intake of foods high in vitamin D, such as oily fish, eggs, meat, fortified margarines and cereals. E Assessment of psychosocial impact: As per BAD 2022 guidelines: Discuss with patients with vitiligo (including pediatric patients) the psychosocial impact of living with the condition, emphasizing the relationship between the skin and the mind. E Assess and monitor the quality of life and level of psychological distress associated with living with vitiligo. Consider using the PHQ-4, PHQ-9, GAD7, DLQI and more specifically the VIPs or the VitiQoL assessment tools. A As per DDG 2022 guidelines: Recognize that different esthetic norms, stigmatization, unpredictable course of disease, and lack of availability of a universal therapy result in psychological distress and impairment of quality of life. E Consider using special assessment tools, such as Vitiligo Impact Patient scale, DLQI, or Vitiligo-specific Quality-of-Life questionnaire, for identification of patients benefitting from psychotherapeutic intervention. E 2. Medical management Topical therapies, corticosteroids: As per BAD 2022 guidelines, offer a potent or very potent topical corticosteroid once daily, to minimize potential side-effects and avoiding the periocular area, as first-line therapy in primary or secondary care in patients with vitiligo. A Show 3 more As per DDG 2022 guidelines: Offer topical corticosteroids as first-line therapy in patients with limited vitiligo and extrafacial involvement. E Administer potent corticosteroids (class 3) with improved therapeutic index, such as mometasone furoate, for a period of 3-6 months. Recognize that this therapy is also suitable in pediatric patients, but take into consideration systemic resorption in intertriginous areas. E Topical therapies, calcineurin inhibitors: As per BAD 2022 guidelines: Consider offering topical tacrolimus 0.1% ointment BID in patients with facial vitiligo as an alternative to potent or very potent topical corticosteroids. C https://web.pathway.md/diseases/reciCMbY14Ckz5DTD 2/7 6/29/23, 3:44 AM Vitiligo Pathway Consider offering topical tacrolimus 0.1% ointment BID under occlusion on photoexposed areas only in patients with nonfacial vitiligo as an alternative to potent or very potent topical corticosteroids. C As per DDG 2022 guidelines: Consider offering off-label topical calcineurin inhibitors as an alternative to topical corticosteroids in patients with vitiligo. E Apply topical calcineurin inhibitors BID and, depending on the response, for 6-12 months. Offer proactive therapy applied twice weekly in case of successful repigmentation (even after phototherapy and targeted phototherapy), to reduce the recurrence risk. E Topical therapies (vitamin D analogs): insufficient evidence to support the use of topical vitamin D analogs in patients with vitiligo. I Systemic therapies, corticosteroids: As per BAD 2022 guidelines, insufficient evidence to support the use of any currently available systemic treatments as monotherapy in patients with stable vitiligo. Show 2 more As per DDG 2022 guidelines, consider offering oral mini-pulse therapy with corticosteroids (such as dexamethasone, prednisone, or methylprednisolone) on 2 consecutive days per week to achieve disease arrest in patients with acute, rapidly progressing vitiligo. Administer treatment for a duration of 3-6 months. Do not offer this therapy as monotherapy for repigmentation. E Systemic therapies, other agents: As per BAD 2022 guidelines: Do not offer azathioprine in combination with PUVA therapy (or narrowband UV B) in patients with vitiligo, due to the risk of malignancy. D Insufficient evidence to support the use of minocycline, methotrexate or tofacitinib in patients with vitiligo. I As per DDG 2022 guidelines, recognize that anti-TNFs may increase the risk of developing vitiligo. E Depigmentation therapy: As per BAD 2022 guidelines, consider offering depigmentation therapies, after adequate psychological assessment and/or intervention, in patients with extensive vitiligo on visible sites, if the condition is having a negative psychological impact. E As per DDG 2022 guidelines, consider offering depigmentation therapy only in extremely rare cases and after exploitation of all therapeutic options, used very restrictively and only after a thorough discussion with the patient due to the irreversible destruction of melanocytes. E 3. Nonpharmacologic interventions Sun protection: As per BAD 2022 guidelines, advise using sunscreen with 4-star or 5-star UV A rating and sun protection factor 50 in patients with vitiligo, applied to affected patches and surrounding skin before going outdoors into the sun. E https://web.pathway.md/diseases/reciCMbY14Ckz5DTD 3/7 6/29/23, 3:44 AM Vitiligo Pathway As per DDG 2022 guidelines, advise strict application of highly potent broad-spectrum sunscreens in patients with vitiligo, given the increased sensitivity of lesioned skin areas to sunlight. E Cosmetic camouflage: As per BAD 2022 guidelines, consider providing a skin camouflage consultation in patients with vitiligo desiring to explore this option. C As per DDG 2022 guidelines, recognize that dermatocosmetic products, such as creams and sprays, liquid or compact formulations, concealers, and fixing sprays, enable covering of vitiligo lesions. Pay attention to gentle cleansing techniques due to potential koebnerization. Recognize that permanent pigment correction may lead to esthetically unsatisfactory results due to the unpredictable course of vitiligo. E Psychological support: As per BAD 2022 guidelines, refer patients with vitiligo with moderate-to-severe psychological distress to psychological services for group or/and individual CBT. A As per DDG 2022 guidelines, advise patients participation in self-help organizations and self- help groups for questions concerning the day-to-day management of the disease, to provide an opportunity for mutual contact. E Complementary therapies: insufficient evidence to support the use of a specific complementary therapy in patients with vitiligo. I 4. Therapeutic procedures Ultraviolet phototherapy: As per BAD 2022 guidelines, offer narrowband UV B (whole body or localized, such as home-based handheld) as first-line phototherapy in patients with vitiligo exhibiting inadequate response to topical therapy and/or having extensive or progressive disease. A Show 5 more As per DDG 2022 guidelines, offer narrowband UV B (2-3 times weekly) in patients with non- segmental vitiligo. E Show 6 more Targeted phototherapy: As per BAD 2022 guidelines, consider offering excimer laser or light in patients with localized vitiligo in combination with topical calcineurin inhibitors (tacrolimus). Counsel patients before treatment about the theoretical increased risk of skin cancer with this combination of treatment. C As per DDG 2022 guidelines: Consider offering 308-nm excimer laser and 308-nm excimer lamp phototherapy in patients with segmental vitiligo and non-segmental vitiligo of limited extent. E Consider offering combination with topical and systemic medications (such as corticosteroids, topical calcineurin inhibitors) to enhance the effect of targeted phototherapy. E Carbon dioxide laser therapy: https://web.pathway.md/diseases/reciCMbY14Ckz5DTD 4/7 6/29/23, 3:44 AM Vitiligo Pathway Consider offering CO laser in combination with 5-fluorouracil in adult patients with nonsegmental vitiligo on the hands and feet, if other treatments have been ineffective. Apply 5-fluorouracil once daily for 7 days per month for 5 months; CO laser treatments once a month for 5 months. C Insufficient evidence to recommend combination treatment of potent or very potent topical corticosteroid with narrowband UV B plus CO laser in people with vitiligo. I 5. Surgical interventions Tissue grafting: As per BAD 2022 guidelines: Consider offering cellular grafting, for example blister grafting or cell suspension, in patients with stable, segmental or nonsegmental vitiligo unresponsive to other treatments, and in patients remaining distressed by the condition. C Insufficient evidence to recommend mini-punch grafting in patients with vitiligo. I As per DDG 2022 guidelines, offer surgical therapies in selected patients (based on the location of the skin lesions, individual expertise and equipment) with therapy-resistant stable vitiligo, especially for segmental and focal vitiligo. E 6. Patient education Patient education: Provide patients with vitiligo (including pediatric patients) with a patient information leaflet on the condition and prescribed treatments. E Provide information on self-help (such as leaflets, books, websites, apps) in patients with vitiligo with mild psychological distress. A 7. Follow-up and surveillance Indications for referral: refer patients with suspected vitiligo to a healthcare professional experienced in managing the condition (secondary care specialist or general physicians with enhanced role) if there is diagnostic uncertainty, or the condition condition is progressing rapidly, has a significant psychosocial impact or does not respond to topical treatment. E Assessment of treatment response: monitor the skin of patients with vitiligo for treatment response (or rapid progression) via medical photography (digital imaging) taken at the beginning of treatment and at regular intervals of approximately 3-6 months. E Show 2 more Clinical findings Past medical history Ocular exam Alopecia areata Iris depigmentation https://web.pathway.md/diseases/reciCMbY14Ckz5DTD 5/7 6/29/23, 3:44 AM Vitiligo Pathway Hyperthyroidism Lab findings Hypothyroidism serum 25-hydroxyvitamin D Lichen sclerosus Morphea Pernicious anemia Psoriasis Rheumatoid arthritis SLE Scleroderma Integument exam Hypopigmented skin patches Serological findings serum anti-TPO antibodies serum anti-thyroglobulin antibodies Studies 2022 TRuE-V2 In patients 12 years of age who had non-segmental vitiligo with depigmentation covering 10% of total body-surface area, ruxolitinib was superior to placebo with respect to the percentage of patients achieving depigmentation of 75% in the facial vitiligo area scoring index at week 24. David Rosmarin et al. N Engl J Med. 2022 Oct 20. 2022 TRuE-V1 In patients 12 years of age who had non-segmental vitiligo with depigmentation covering 10% of total body-surface area, ruxolitinib was superior to placebo with respect to the percentage of patients achieving depigmentation of 75% in the facial vitiligo area scoring index at week 24. David Rosmarin et al. N Engl J Med. 2022 Oct 20. Show 1 more References 1. Markus B hm, Jo Ana Schunter, Klaus Fritz et al. S1 Guideline: Diagnosis and therapy of vitiligo. J Dtsch Dermatol Ges. 2022 Mar 4. Open 2. V Eleftheriadou, R Atkar, J Batchelor et al. British Association of Dermatologists guidelines for the management of people with vitiligo 2021. Br J Dermatol. 2022 Jan;186 1 18 29. Open 3. K S Thomas, J M Batchelor, P Akram et al. Randomized controlled trial of topical corticosteroid and home-based narrowband ultraviolet B for active and limited vitiligo: results of the HI Light Vitiligo Trial. https://web.pathway.md/diseases/reciCMbY14Ckz5DTD 6/7 6/29/23, 3:44 AM Vitiligo Pathway Br J Dermatol. 2021 May;184 5 828 839. Open 4. David Rosmarin, Thierry Passeron, Amit G Pandya et al. Two Phase 3, Randomized, Controlled Trials of Ruxolitinib Cream for Vitiligo. N Engl J Med. 2022 Oct 20;387 16 1445 1455. Open https://web.pathway.md/diseases/reciCMbY14Ckz5DTD 7/7