A rare case of complete Vogt-Koyanagi-Harada disease presenting to a tertiary care hospital in late stage: Clinical features, diagnosis, and management

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Vol 7 | Issue 2 | February 2021 Indian J Case Reports 44
Case Report
A rare case of complete Vogt-Koyanagi-Harada disease presenting to a tertiary
care hospital in late stage: Clinical features, diagnosis, and management
Pallavi Priyadarsani Sahu1, Matuli Das2
From 1Senior Resident, 2Assistant Professor, Department of Ophthalmology, Kalinga Institute of Medical Sciences, Kalinga Institute of Industrial
Technology University, Bhubaneswar, Odisha, India
Vogt-Koyanagi-Harada disease (VKHD) is a rare
multisystemic granulomatous autoimmune disease
affecting organs with high melanocyte concentrations
such as the eye, central nervous system (CNS), inner ear, and
skin [1]. VKHD has a predilection for dark-complexioned persons
and more prevalent in certain ethnicities such as Hispanics, people
from the Middle East, and Asian Indians but not the blacks of sub-
Saharan African descent [2]. It is more common in adults and
women are more affected than men [3]. In India, a few cases have
been reported with prevalence in uveitic cohorts being 1.4–3.5% in
the South Indian population [4]. Pathogenesis of VKHD involves
T-cell-mediated autoimmune disorder targeting melanocytic self-
antigens. It has a genetic predisposition associated with HLA-
DRB1*0405 [5].
CASE REPORT
A 35-year-old female presented to our hospital with chief
complaints of pain, redness, and progressive diminution of vision
in both eyes for 2 years. It was associated with headache, vertigo,
and hearing loss. She also complained of a gradual loss of hair,
white patches over the scalp, and forehead with whitening of
eyebrows and eyelashes over a period of 3 years for which she
had taken homeopathic medications. She had a history of multiple
episodes of pain, redness, and diminution of vision in both eyes
for 1 year for which she was seen and treated at many periphery
hospitals in line of conjunctivitis with topical antibiotic drops
but the detailed evaluation was not done anywhere. Later on, she
developed further loss of vision for which she came to our hospital.
On general examination, the patient was conscious,
cooperative, and well oriented. Vitals were stable. Pallor was
present. There was the presence of multiple vitiligo patches over
the scalp and forehead (Fig. 1a). On ocular examination, there was
the presence of hypopigmented patches over the eyebrows and
poliosis (Fig. 1b). Slit-lamp examination showed circumcorneal
congestion, mutton-fat keratic precipitates over corneal
endothelium, Grade 1+ cells, and minimal flare in the anterior
chamber, poorly reacting pupils, segmental posterior synechiae,
iris atrophic patches, and immature cataract in both eyes. Visual
acuity was CF 1 m in both eyes. The intraocular pressure (IOP)
was normal in both eyes.
Posterior segment examination revealed mild vitritis and
retinal detachment involving the macula. Fundus photograph
of both eyes showed similar findings as above (Fig. 2a).
Optical coherence tomography (OCT) showed separation of the
neuroretinal layer from outer hyper-reflective layer, presence of
pockets of subretinal fluid separated by fibrous septa, subretinal
deposits of fibrin, and internal limiting membrane irregularities
in both eyes (Fig. 2b). Dermatological examination revealed
alopecia and vitiligo. ENT examination showed sensorineural
ABSTRACT
Vogt-Koyanagi-Harada disease (VKHD) is a rare T-cell-mediated multisystemic autoimmune disorder affecting organs with high
melanocytic concentrations such as uvea, skin, ear, and meninges. VKHD is difficult to diagnose because its clinical presentation is
variable and multisystemic which often leads to late diagnosis and treatment allowing the appearance and progression of the disease
sequelae. Here, we report the case of a young adult female who was previously undiagnosed and inadequately treated at multiple centers
presenting to our hospital in the late stage of VKHD with panuveitis, retinal detachment, hearing loss, alopecia, and vitiligo, which was
classified as complete VKH disease and successfully treated in our hospital.
Key words: Panuveitis, Rare autoimmune disease, Retinal detachment, Vogt-Koyanagi-Harada disease
Correspondence to: Dr. Pallavi Priyadarsani Sahu, Flat no. 005, Basera
Impression Plus, Beside Reliance Smart , Patia, Bhubaneswar-751024, Odisha.
E-mail: pallavipsahu@gmail.com
© 2021 Creative Commons Attribution-NonCommercial 4.0 International
License (CC BY-NC-ND 4.0).
Access this article online
Received - 13 December 2020
Initial Review - 28 December 2020
Accepted - 28 January 2021
Quick Response code
DOI: 10.32677/IJCR.2021.v07.i02.001

Sahu and Das VKHD – clinical features, diagnosis, and management
Vol 7 | Issue 2 | February 2021 Indian J Case Reports 45
hearing loss of moderate degree (53 db) in both ears. CNS
examination was normal. The rest of the systemic examinations
were within normal limits.
Serological tests showed mild anemia (HB%: 8 g%) and
elevated liver enzymes. Cerebrospinal fluid (CSF) study showed
lymphocytic pleocytosis (lymphocytes: 85%). Chest X-ray,
magnetic resonance imaging scan brain, and orbit were normal.
Based on these findings, a diagnosis of complete VKH disease was
done according to the revised diagnostic criteria for VKHD [6-8].
The patient was started on IV methylprednisolone (500 mg)
for 3 days followed by oral prednisolone (1 mg/kg body weight)
which was subsequently tapered at weekly intervals. She was also
prescribed instillation of topical 1% prednisolone drop and 1%
atropine 3 times/day.
Following treatment, pain and redness resolved over a week
and there was a decrease of subretinal fluid over a period of
2 weeks. Follow-up at the end of 2 months of corticosteroid
therapy showed complete resolution of subretinal fluid and
complete reattachment of the retina on fundus photograph
(Fig. 3a) and OCT (Fig. 3b) in both the eyes. However, there
was a progression of cataract in both the eyes and worsening
of alopecia. Best-corrected visual acuity at the end of 2 months
was 20/200 in both eyes. The patient is now maintained on
low-dose topical steroids. The patient has been educated about
the nature of the disease and its complications and advised for
timely follow-ups.
DISCUSSION
Vogt-Koyanagi-Harada syndrome is an uncommon autoimmune
disorder having multisystemic involvement. The clinical course of
VKHD includes four stages: (a) Prodromal stage characterized by
non-specific symptoms such as malaise, fever, headache, and neck
stiffness. Neurological involvement such as cranial nerve palsies,
hemiparesis, transverse myelitis, optic neuritis, and hearing loss
can also occur. CSF shows lymphocytic pleocytosis [9]. (b) Acute
uveitic stage: It follows the prodromal phase and lasts for several
weeks. Bilateral posterior uveitis is the most common finding;
however, unilateral cases have also been reported. Fundus findings
include multiple serious retinal detachments, thickening of the
posterior choroid, and optic disc edema [10]. (c) Convalescent
stage shows progressive depigmentation of the skin leading to
vitiligo [9] and uvea leading to “Sunset glow” appearance of
the fundus. Perilimbal vitiligo is the earliest depigmentation to
occur, often within 1 month of onset of disease [11]. Alopecia and
poliosis can also occur. (d) Chronic recurrent stage: Recurrent
anterior granulomatous uveitis and ocular complications such as
glaucoma, cataract, and choroidal neovascular membrane [9].
At present, VKHD is classified as per “The Revised diagnostic
criteria” (2001) by the International Committee on Nomenclature
as follows: [6-8] (a) Complete VKH disease – in which ocular,
integumentary, and neurologic/auditory involvement are present.
(b) Incomplete VKH disease – ocular with either neurological/
auditory involvement is present. (c) Probable VKH disease
is uveitis consistent with VKH without any extraocular
manifestations.
Although multiple cases of VKHD have been reported,
yet the prevalence of this disease is low in our region. Due to
dermatological manifestations of the disease such as vitiligo
and alopecia which are very bothersome to the patients, these
patients first seek treatment for such condition. The patient, in
this case, was treated with multiple homeopathic and Ayurvedic
medications for alopecia and vitiligo for 3 years and was not
referred to seek any medical advice. This was the reason for the late
diagnosis of the entity. Further, the beneficial and harmful effects
Figure 1: (a) Multiple vitiligo patches and alopecia on the scalp of the
patient; (b) whitening of eyebrows and eyelashes
ab
Figure 2: Fundus photo (a) and optical coherence tomography image
(b) showing retinal detachment with subretinal fluid in both eyes
a
b
Figure 3: Fundus photo (a) and optical coherence tomography image
(b) showing resolution of subretinal fluid and reattachment of retina
post-treatment in both eyes
a
b

Sahu and Das VKHD – clinical features, diagnosis, and management
Vol 7 | Issue 2 | February 2021 Indian J Case Reports 46
of such medications in the disease progression are not known.
Multifaceted presentation of the disease, lack of awareness, and
improper diagnosis, all these commonly lead to late presentation
of this disease to the ophthalmological department. This case also
was treated at multiple peripheral centers in line of conjunctivitis
but posterior segment evaluation and systemic evaluation were
not done anywhere which is another reason for the late diagnosis
of this case.
The goal of treatment in VKHD is to suppress active
inflammation, prevent disease relapse, and avoid complications.
Treatment mainly includes administration of systemic
immunosuppressive supplemented by local corticosteroids [12].
Early administration of oral prednisone at a dose of 1–2 mg/kg/day
followed by slow tapering to avoid recurrences is the generally
accepted regimen, while pulse intravenous corticosteroid therapy
of 1 g/day of methylprednisolone for 3–5 days followed by oral
prednisolone is usually reserved for cases with severe inflammation.
The cases not responding to steroids, other immunosuppressive
such as cyclosporine, azathioprine, and methotrexate, can also
be added. Our patient showed a good response to steroids alone.
Slow tapering of the corticosteroid dose, along with frequent
follow-up examinations, is necessary to avoid the recurrence of
inflammation [12,13]. IOP should be monitored at each follow-up
visit, as glaucoma is a known secondary complication of this
disease [9]. Recurrence of the disease is also very common so all
the patients of VKHD should be counseled for regular follow-up
to prevent further complications.
CONCLUSION
As VKHD is a progressive, rare, and multisystemic disease
with variable outcomes, an early diagnosis and treatment play
a significant role in deciding the fate of the patient. Primary
health workers, being the ones to have the first contact with such
patients, should be more aware of such syndromic multisystemic
diseases and diligent while dealing with such patients. Early
referral to tertiary centers should be the golden rule, as delay
in initiation of treatment can lead to secondary complications
such as serous retinal detachment, cataract, glaucoma, choroidal
neovascularization, and sensorineural hearing loss, some of
which may be irreversible. Moreover, in all the cases presenting
with pain and redness to the eye department, fundoscopy should
be done invariably, to rule out posterior segment involvement.
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Funding: None; Conflicts of Interest: None Stated.
How to cite this article: Sahu PP, Das M. A rare case of complete
vogt-koyanagi-harada disease presenting to a tertiary care hospital
in late stage: clinical features, diagnosis, and management. Indian J
Case Reports. 2021;7(2):44-46.