Cutaneous Vitiligo Associated with Chorioidal Vitiligo: Case Report

Vitiligo is a common pigmentary disorder that causes the depigmentation of skin, mucosal or hair. It affects people regardless of race, sex or skin type. There are two main types of vitiligo; a segmental or unilateral form and a non-segmental or generalized vitiligo. If most of the body is affected, it is called universal vitiligo. 

Usually, vitiligo manifests as a hypopigmentation spot. The discolored areas usually spread over time and affect large parts of the body. The etiopathogenetic mechanisms of vitiligo are still poorly understood. Treatment of vitiligo focuses on restoring the colour and stabilizing repigmentation to the affected areas [1]. Pharmacological and non-pharmacological therapies are available, such as phototherapy and surgical options. The therapy depends on the type of vitiligo, phase and general condition of the patient. The first line of pharmacological treatments includes topical corticosteroids, calcineurin inhibitors and photo chemotherapies. Oral steroids and other immunosuppressants have been evaluated in a limited study [2]. 

In literature, the association of ocular abnormalities and vitiligo hasbeen described [3-5]. In this study's case, the patient suffers from full body depigmentation that has not been previously diagnosed. This made the interpretation of the ophthalmology findings difficult.

The 68 year-old European woman was referred to the our department for evaluation of an unclear pigmented choroidal lesion of both eyes (OU), which was found after cataract surgery. 

On examination, the visual acuity was 20/30 in the right eye and 20/25 in the left eye. The intraocular pressure was 18 mmHg in the right eye and 17 mmHg in the left eye. The anterior segment was uninflamed and the iris was unremarkable in both eyes. The fundus examination of the OU showed a diffuse irregular choroidal pigmentation with flat x-shaped areas of choroidal hypopigmentation. 

The additional diagnostic, includingphoto documentation, an ultrasound Examination and Depth Imaging-Optical Coherence Tomography (EDI-OCT), were not able to reveal any elevated areas. Colour fundus imaging with Zeiss Clarus showed the X-shaped choroidal hypopigmentation clearly (Figure 1). The choroid thickness was normal. The autofluorescence was unremarkable. 

Figure 1: Fundus photographs of the right eye (left) and the left eye (right) showing bilateral X-shape hypopigmentation. 

Previously, the patient had no medical history of general or ocular disease except breast cancer for 16 years and phacoemulsification for 6 months. 

Her skin was evenly pale without any unusual spots or hyperpigmentation areas. She never went to see a dermatologist and no skin diseases had been detected until then. When questioned about birthmarks and her episode of skin cancer, she noted the depigmentation of the skin over her whole body starting when she was 30. Her father also had the same condition. Her vitiligo diagnosis was based on her medical history and explained her choroidal features. We excluded ocular malignancy and recommended the following examinations by an ophthalmologist and a consultation with a dermatologist.

Choroidal vitiligo is an acquired condition which might occur as a primary or secondary process. As a primary process, it manifests as part of a cutaneous vitiligo [2], due to the destruction of melanocytes. A secondary process can be the result of uveitis [3] or part of Vogt-Koyanagi-Harada and Alezzandrini's syndrome [4]. 

Choroidal vitiligo usually presents itself with diffuse choroidal hypopigmentation; an X-shaped choroidal pattern like in our case is a rare presentation [6]. Choroidal hypopigmentation is an asymptomatic benign condition, associated with normal visual acuity [7,8]. 

The choroidal thickness may decrease in vitiligo patients [9] and should be checked for possible disorders. Depigmentation can affect other ocular structures. Various studies have reported the association of facial vitiligo and ocular disorders, such as iris and trabecular meshwork hypopigmentation, atrophy of retinal pigment epithelium, uveitis and dry eyes [10,11].

Choroidal vitiligo can mimic a choroidal pigmented tumor and can be misdiagnosed as choroidal nevus or melanoma [7]. The additional diagnostic includes colour fundus imaging, an ultrasound Examination and Depth Imaging-Optical Coherence Tomography (EDI-OCT) which can assist to reduce the number of false diagnoses.

Our case report is based on the clinical case of a female patient who underwent therapy at our clinic. All patients who undergo therapy at the University Hospital sign an informed consent which allows our hospital to use their medical data for purposes of clinical research after complete anonymisation. In our report, we did not use any personal data except for the patient's age, which she agreed to.

The authors have declared no conflicts of interest.

Our study was funded from our own resources, no sponsors were involved.

Ekaterina Sokolenko: work on the conception and design of this paper, analysis and interpretation of data.

Mihael Freistühler, Miltiadis Fiorentzis: revising it critically for important intellectual content.

Nikolaos E Bechrakis: final approval of the version to be published.