Case of the Month

Edited by Robert N. Johnson, MD

Figure 1: Color photo montage of the right eye. Note the pigmented lesion temporal to the macula with depigmentation around margin.

Case History

A 51-year-old woman was referred for evaluation of an asymptomatic retinal lesion in her right eye.

On examination visual acuity was 20/25 in the both eyes. Her pupils were equal round and reactive with no afferent pupillary defect.  The intraocular pressures and anterior segment were unremarkable. Fundus examination demonstrated a 1.5mm by 1.5mm flat brown choroidal lesion with surrounding yellow border in the right eye (Figure 1).

Differential Diagnosis

Choroidal nevus, Congenital hypertrophy of the retinal pigment epithelium, Choroidal melanoma, Metastatic lesion, choroidal neovascularization

Additional History and Diagnosis

The patient has a history of a skin melanoma that was removed on her chest. She had no other past ocular history. Based on her clinical history and exam findings the patient was diagnosed with a halo nevus in her right eye.

Discussion

Halo nevus is a form of choroidal nevus where the lesion has a surrounding depigmented ring or “halo”.¹ The first depictions of such findings in the skin were thought to have occurred in the “Temptation of Saint Anthony” painting by Mattias Grunewald in the 16th century.² In 1916, dermatologist Richard Sutton was the first to recognize the halo nevus of the skin as a distinct entity, naming it leukoderma acquisitum centrifugum.³

The first reports of choroidal halo nevi came in the 1980’s.⁴ Their prevalence is relatively uncommon: one large series identified 162/3,422 (4.7%) of eyes with choroidal nevi with a halo appearance.⁵

Patients may be followed with serial fundus photography. On fundus autofluorescence the pigmented portion is typically hypofluorescent while the surrounding ring is hyperautofluorescent. Ultrasound may display a plateau or dome shaped lesion. Ocular coherence tomography may show presence of cystoid edema, subretinal fluid, retinal edema, or pigment epithelial detachment.¹

There is no observed increased risk of uveal melanoma in patients with halo nevi but there was a greater incidence of cutaneous melanoma.⁵ The association between cutaneous halo nevi and malignant melanoma is postulated to be due to lymphocytes attacking cancerous melanocytes also affecting nevus cells.^6,7 Patients should first be monitored biannually and then annually with regular full body dermatologic exams for signs of cutaneous melanoma.

References

1. Shields CL, Maktabi AM, Jahnle E, et al. Halo Nevus of the Choroid in 150 Patients: The 2010 Henry van Dyke Lecture. Arch Ophthalmol 2010;128(7):859–864.
2. Happle R: [Grünewald nevus]. Hautarzt 1994;45:882-3.
3. Steffen C, Thomas D. The man behind the eponyms: Richard L Sutton: periadenitis mucosa necrotica recurrens (Sutton’s ulcer) and leukoderma acquisitum centrifugum-Sutton’s (halo) nevus. Am J Dermatopathol 2003;25:349-54.
4. Shields JA. Diagnosis and Management of Intraocular Tumors. St Louis, CV Mosby, 1983.
5. Shields CL, Furuta M, Mashayekhi A, et al. Clinical spectrum of choroidal nevi based on age at presentation in 3422 consecutive eyes. Ophthalmology 2008;115:546-52.
6. Epstein WL, Sagebeil R, Spitler L, et al. MS Halo nevi and melanoma.  JAMA 1973;225(4):373- 377.
7. Langer K and Konrad K. Congenital melanocytic nevi with halo phenomenon: report of two cases and a review of the literature.  J Dermatol Surg Oncol 1990;16(4):377-380.