Oculodermal Melanocytosis: A Clinical Case Report

Introduction Oculodermal melanocytosis (ODM) is a rare congenital condition characterized by brown or blue-gray asymptomatic hyperpigmentation of the eye and its surrounding structures, typically unilateral [1,2]. It occurs in approximately 0,04% of the population and is more common in females than males, with a 5:1 ratio. The main ocular complications include an increased risk of glaucoma (10%) and uveal melanoma (3%) [3]. Case Presentation A 7-year-old boy, who had heterochromia (different-colored eyes) from birth, was referred to the Department of Ophthalmology at the Hospital of LUHS KC due to decreased visual acuity (VA) and a complaint of more expressed discoloration in his right eye (OD). On examination, first-degree myopia was diagnosed, and the best-corrected visual acuity was 1,0. Intraocular pressure was normal. Slit-lamp biomicroscopy revealed pigmentation of the sclera and iris in the OD. A cycloplegic fundus examination of the OD showed hyperpigmentation of the choroidal vascular layer. Optical coherence tomography findings indicated that the retinal nerve fiber layer thickness was within the standard range for his age. Glasses were prescribed, the guardian was informed of potential complications, and a follow-up visit was scheduled for six months later. Discussion The clinical features of ODM are closely linked to the theory that the migration of dendritic dermal melanocytes from the neural crest to the epidermis fails, resulting in their entrapment in the dermis during embryonic development [2,4]. As a result, most lesions are apparent at birth, and early recognition of ODM can enable prompt management of complications. Occasionally, distinguishing ODM from primary acquired melanosis of the conjunctiva can be challenging. Conclusions ODM is a benign condition but carries the risk of developing glaucoma or uveal melanoma. Therefore, regular follow-up with ophthalmologists is recommended to ensure that ocular complications are promptly identified and managed.

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Lithuanian University of Health Sciences
Published
2025-03-13
Primary Topic
Ocular Oncology and Treatments
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Oculodermal Melanocytosis: A Clinical Case Report

Eglė Ruseckytė, Monika Vaškaitė, Paulina Mikulėnaitė
Lithuanian University of Health Sciences
Ocular Oncology and Treatments
article

Oculodermal Melanocytosis: A Clinical Case Report

Eglė Ruseckytė, Monika Vaškaitė, Paulina Mikulėnaitė
article en

Abstract

Introduction Oculodermal melanocytosis (ODM) is a rare congenital condition characterized by brown or blue-gray asymptomatic hyperpigmentation of the eye and its surrounding structures, typically unilateral [1,2]. It occurs in approximately 0,04% of the population and is more common in females than males, with a 5:1 ratio. The main ocular complications include an increased risk of glaucoma (10%) and uveal melanoma (3%) [3]. Case Presentation A 7-year-old boy, who had heterochromia (different-colored eyes) from birth, was referred to the Department of Ophthalmology at the Hospital of LUHS KC due to decreased visual acuity (VA) and a complaint of more expressed discoloration in his right eye (OD). On examination, first-degree myopia was diagnosed, and the best-corrected visual acuity was 1,0. Intraocular pressure was normal. Slit-lamp biomicroscopy revealed pigmentation of the sclera and iris in the OD. A cycloplegic fundus examination of the OD showed hyperpigmentation of the choroidal vascular layer. Optical coherence tomography findings indicated that the retinal nerve fiber layer thickness was within the standard range for his age. Glasses were prescribed, the guardian was informed of potential complications, and a follow-up visit was scheduled for six months later. Discussion The clinical features of ODM are closely linked to the theory that the migration of dendritic dermal melanocytes from the neural crest to the epidermis fails, resulting in their entrapment in the dermis during embryonic development [2,4]. As a result, most lesions are apparent at birth, and early recognition of ODM can enable prompt management of complications. Occasionally, distinguishing ODM from primary acquired melanosis of the conjunctiva can be challenging. Conclusions ODM is a benign condition but carries the risk of developing glaucoma or uveal melanoma. Therefore, regular follow-up with ophthalmologists is recommended to ensure that ocular complications are promptly identified and managed.

Lithuanian University of Health Sciences
Good health and well-being
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Ocular Oncology and Treatments
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