What is the diagnosis and treatment for a patient with vitiligo, hearing loss, pulsatile tinnitus, and weakness?

Vogt-Koyanagi-Harada Syndrome: Diagnosis and Urgent Treatment

This patient has Vogt-Koyanagi-Harada (VKH) syndrome, a rare autoimmune disorder targeting melanocytes, and requires immediate high-dose corticosteroid therapy to prevent permanent vision loss and hearing damage.

Clinical Diagnosis

The constellation of vitiligo, hearing loss, pulsatile tinnitus, and weakness strongly suggests VKH syndrome, an autoimmune condition that attacks melanocyte-containing tissues throughout the body 1.

Key Diagnostic Features

• Dermatologic manifestations: Vitiligo (depigmented patches) occurs in VKH syndrome due to autoimmune destruction of melanocytes in the skin 1
• Auditory symptoms: Hearing loss, tinnitus (including pulsatile tinnitus), and hyperacusis are characteristic vestibular-auditory manifestations 1
• Neurologic symptoms: Weakness and other peripheral neurologic symptoms are common presentations 1
• Ocular involvement: Bilateral uveitis is a hallmark feature that must be actively sought, as it can lead to blindness if untreated 1

Essential Workup

• Ophthalmologic examination: Urgent evaluation for bilateral uveitis is mandatory, as ocular involvement is present in virtually all VKH cases and vision loss can be permanent 1
• Audiometric testing: Formal hearing evaluation should be performed, though findings may be within normal limits early in disease despite patient-reported symptoms 1, 2
• Thyroid function tests: Check thyroid function and thyroid autoantibodies, as autoimmune thyroid disease occurs in approximately 34% of vitiligo patients 3, 4
• Neuroimaging: Consider MRI if focal neurologic abnormalities are present or if asymmetric hearing loss is documented 2

Immediate Treatment

Start high-dose systemic corticosteroids immediately upon diagnosis to prevent irreversible damage to vision and hearing 1.

Corticosteroid Therapy

• VKH syndrome requires aggressive systemic corticosteroid therapy, unlike isolated vitiligo where oral dexamethasone is contraindicated due to unacceptable side-effects 5, 6
• The patient described in the literature achieved complete resolution of neurologic, vestibular, ocular, and auditory symptoms with corticosteroid therapy 1

Critical Distinction from Isolated Vitiligo Management

• Oral corticosteroids are NOT recommended for vitiligo alone: The British Association of Dermatologists explicitly states oral dexamethasone cannot be recommended for arresting vitiligo progression due to unacceptable risk of side-effects 5, 6
• VKH syndrome is the exception: Systemic corticosteroids are the standard of care for VKH syndrome due to the multi-organ involvement and risk of permanent vision and hearing loss 1

Long-term Vitiligo Management (After Acute VKH Treatment)

Once the acute VKH syndrome is controlled, the vitiligo component can be managed with standard approaches:

Topical Treatments

• Potent topical corticosteroids: Clobetasol propionate 0.05% or betamethasone valerate 0.1% applied twice daily for maximum 2 months achieves 15-25% repigmentation in approximately 43% of patients 4
• Calcineurin inhibitors: Tacrolimus 0.1% or pimecrolimus 1% provide comparable efficacy with superior safety profile, particularly for facial involvement 5, 4
• Never extend potent topical steroids beyond 2 months to prevent skin atrophy 4

Phototherapy for Widespread Disease

• Narrowband UVB is preferred over PUVA for widespread vitiligo due to greater efficacy and safety 5
• Reserve phototherapy for patients with darker skin types and monitor with serial photographs every 2-3 months 5
• Safety limits: maximum 200 treatments with NB-UVB for skin types I-III 5

Monitoring and Psychological Support

• Serial photographs every 2-3 months to objectively document disease progression or response 4
• Psychological interventions should be offered as vitiligo can be cosmetically disfiguring and stigmatizing 5, 4
• Screen for other autoimmune conditions given the high association with autoimmune thyroid disease and the autoimmune nature of VKH syndrome 3, 4

Critical Pitfalls to Avoid

• Missing the diagnosis of VKH syndrome: Failing to recognize the multi-system involvement (vitiligo + hearing loss + tinnitus + weakness) can lead to delayed treatment and permanent vision or hearing loss 1
• Delaying ophthalmologic evaluation: Bilateral uveitis requires urgent treatment to prevent blindness 1
• Treating as isolated vitiligo: Using only topical treatments when systemic corticosteroids are needed for VKH syndrome 1
• Assuming normal audiometry excludes auditory involvement: Patients may report significant auditory symptoms despite normal audiometric findings 1