Vogt-Koyanagi-Harada Syndrome from Dermatological Perspective: A Case Report
Year: 2025
Type: article
Study design: Case Report
Abstract: Dear Editor, Vogt-Koyanagi-Harada syndrome (VKHS) is a rare, multi-system granulomatous, auto-inflammatory condition that affects melanocyte-rich tissues and is characterised by a broad spectrum of neurological/auditory manifestations preceding the ophthalmic/dermatological symptoms.[1,2] Although VKHS primarily presents to ophthalmologists because of visual impairments, the involvement of multiple systems necessitates comprehensive understanding across various specialties. The current case highlights the importance of a multi-disciplinary approach in diagnosing and managing VKHS. This case also illustrates the critical role dermatologists can play in the diagnosis of VKHS. A 27-year-old female patient presented to dermatology outpatient with irregular patches of hair loss for 10–12 days. The onset was sudden and associated with scalp tenderness [Figure 1]. There was no pruritus in the alopecia patches. The hair pull test was positive, limited to the patches. The examination of alopecia patches did not reveal any pigmentation, scarring, or any other morphological change (depression in affected areas, thinned-out skin, or pigmentation). There were a series of symptoms weeks before the onset of the hair loss including frequent headaches, tinnitus, and a brief episode of neck stiffness. She also reported experiencing a subtle decrease in vision and orbital pain multiple times in the last 5–6 weeks, which, she attributed to the generalised fatigue and extended night-hour studies. There was no history of prolonged illness/ophthalmic surgery/trauma. During the past few months, she had consulted the general practitioner in her vicinity for the ophthalmic symptoms and was advised short courses of oral prednisolone (20 mg/day), non-steroidal medications (ibuprofen and paracetamol), and eye drops (carboxymethyl-cellulose and moxifloxacin) on multiple occasions with partial relief during the each treatment period. At the time of her presentation to us, she revealed orbital pain and blurred vision (right eye-6/6, left eye- 6/12) with the perception of a blind spot in the left eye. The clinical details were shared with the ophthalmology and neurology teams. The routine blood workup (complete hemogram, serum iron profile, thyroid profile, and liver/renal function tests) was normal. Optical coherence tomography revealed serous, and, septate, retinal detachments [Figure 2]. Fundoscopy and fluorescein angiography (FA) showed intense hyper-fluorescence at the disc [Figure 3]. Trichoscopic findings (empty follicles in affected areas with normal follicular morphology/number, preserved homogeneity of diameters among the remaining hair shafts throughout the alopecia patches and surrounding uninvolved areas, absence of white areas of fibrosis) did not reveal anything specific to alopecia areata, lichen plano-pilaris, or other common causes of hair loss. Chest computerised tomography showed normal lung architecture. The Mantoux test did not show clinically significant induration. Cerebrospinal fluid analysis and scalp biopsy were not conducted as the patient did not consent. Based on the presenting clinical features (alopecia), their close relation to the constellation of prior symptoms, and the results of the ophthalmic evaluation, the diagnosis of VKH syndrome was made.Figure 1: Alopecic patches of recent onset hair loss on the scalpFigure 2: Optical coherence tomography shows multiple serous, septate, retinal detachments (left eye)Figure 3: Fundoscopy and fluorescein angiography shows intense hyper-fluorescence at the disc (sunset glow fundus) with few small, focal, lesions with intensity alterations in the surrounding areasOral corticosteroids (methylprednisolone at 1 mg/kg/day) with prednisolone acetate 1% eyedrops two hourly were advised. An initial regimen with a very slow tapering of the oral steroids (2.5 mg/week) was planned. However, because of the development of facial oedema and weight gain (4.5 kg) within a month, azathioprine (3 mg/kg/day) was added as a steroid-sparing agent. Currently, methylprednisolone is being tapered by 5 mg/week. By the end of six weeks, the patient reported improvement, with scalp and orbital tenderness completely resolved, hair shedding decreased, and vision fully restored (right eye-6/6, left eye- 6/6). The patient is on fortnightly follow-up. VKHS primarily affects the melanocyte-rich tissues including eyes, central nervous system, inner ear, and skin.[1,2] VKHS accounts for 1%–7% of all cases of the uveitis, globally.[1] In India, prevalence is 1.4%–3.5% in uveitis cohorts.[3] The prevalence among the patients with cutaneous symptoms presenting to dermatology services is not known. Based on the clinical features, the Standardization of uveitis nomenclature (SUN) working group has classified the VKHS into early and late types.[4] During the early VKHS, vitiligo and poliosis has been observed in 4% and 2% cases, respectively. During the late stages, prevalence increases to 22% (vitiligo), 18% (poliosis), and 14% (alopecia).[4] VKH syndrome progresses through four main stages.[1-4] Prodromal stage: lasts for hours to weeks with flu-like symptoms with or without neurological involvement. Acute uveitic stage: bilateral vision impairment predominates. Convalescent/chronic stage: Vitiligo, alopecia, and poliosis can become identifiable. Resolving retinal detachments and sunset-glow fundus on FA can be present, manifesting weeks to months after the prodrome. Chronic recurrent stage: Repeated episodes of uveitis lead to ocular complications. Sympathetic ophthalmia is a close clinical differential of the ophthalmic symptoms of the VKHS. However, it is associated with the preceding invasive trauma, and the features of uveitis can take months to appear.[5] VKHS is a Th1-mediated auto-inflammatory disease triggered by environmental factors and viral infections (Epstein-Barr and cytomegalovirus) in genetically susceptible (HLA-DRB1-04*05) individuals. The tyrosinase family peptides have been detected as the antigenic targets of the T-cells in the VKHS.[1-4,6] The cornerstone of treatment is prompt recognition and aggressive anti-inflammatory therapy. High-dose systemic corticosteroids (1–2 mg/kg/day) with gradual tapering are standard to control disease activity. In poorly responsive patients/experiencing intolerable side effects, other immunosuppressive agents such as methotrexate, cyclosporine, azathioprine, cyclophosphamide, or mycophenolate mofetil are used as steroid-sparing or replacement therapy.[1,2,6,7] The skin involvement is not only a diagnostic clue but is also suggestive of inadequate disease control. Failure to obtain a thorough clinical history in common dermatological conditions like hair loss may delay diagnosis resulting in inappropriate treatment that could harm patients’ overall well-being, particularly ophthalmic. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.
Authors: Sumit Sehgal, Harshit Bansal
Language: English
Subfield: Ophthalmology
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4406933451