SURVEY
OF OPHTHALMOLOGY
VOLUME 28.
AFTERIMAGES
NUMBER 6. MAY-JUNE 1984
JONATHAN
D. WIRTSCHAFTER,
Choroidal Halo Nevus Occurring With Vitiligo GEORGE A. FOURNIER, M.D., DANIEL AND MICHAEL D. WAGONER, M.D.
.\lassachusett~
Abstract. malignant discussed.
M. ALBERT,
&se nnd Ear Irzfirmay:,
Haward
cutaneous
melanoma
l
ocular
V
pertrophy ioretinal
consistent
nexus,
M.D.,
.Uedical
halo nc~us
itiligo, a disorder affecting 0.5% of the general population, is caused by destruction ofpigment cells.“~” It may begin with the dcpigmentation ofthc skin, graving of the hair at an earl?, a,qe (less than 30 vears), poliosis, or the appearance of a halo nexus.” The patient presented here illustrates the fundus counterpart of the halo nevus. . The pattent IS a Caucasian female who was in good health until age 50 years when she developed a malignant melanoma of the right calf which was surgically excised without recurrence. At age 56. vitiligo was diaCgnosed after the development of extensive areas of cutaneous drpigmentation of the chest. back, arms, and neck. At age 57, routine ophthalmic examination was remarkable for lid depigmentation and a piamented fundus lesion about one disc diameter in circumference. The lesion resembled a mottled nevus with a large surrounding area of scarring and depigmentation four disc diameters temporal to the macula OS (Fig. 1). In the course of live years, the diametrr of the surrounding area of depigmentation doubled in size. The findings of the fluorescein angiography were rrviewcd hv a number of‘experienced observers who choroidal
in a Patient
.Sc.hool, Ho.rton, .Iln.c.tarhusett.r
A halo nevus of the ocular fundus is described in a patient with vitiligo and history of melanoma of the calf excised six years previously. The significance of halo nevi is (Surv Ophthalmol Z&671-672. 1984)
Key words.
tklt they were
EDITOR
but not pathognomonic
Ivith retinal
and hyperplasia scarring a possible
pigment
epithclial
l
vitiliqo
\Ve he1iev.e that our patient’s ocular lesion represents a choroidal halo nexus for the following reasons: (1) observation of the patient’s lesion over a period of sis years during which there was a pro-
I;iq. 1. Halo-type ncvus of the choroid with surrounding four disc diameters area of scarring and depigmentation temporal to the macula. Enlargement of the surrounding zone ofdepigmentation had been documented during six ( X 2 1) years of observation.
for hy-
and surrounding choralternative diagnosis. 671
672
Sure Ophthalmol
28(6) May-June
1984
Fi,?. 2. \‘itiliginous involvement of the neck and anterior light chest under normal (A) and IVood’s (ultraviolet) (IS). It was impractical to obtain enough contrast to illustrate the lid depigmentation in this patient.
FOURNIER
ET AL
ses.’ It has been hypothesized that the destruction of nevus cells may represent an immunologic response to the antigens of melanocytic cells.’ In fact, an immunologic association between cutaneous melanoma and the halo nevus has been established: both humoral and cellular factors have been observed in the blood of patients with halo nevi which react to malanoma cells in vitro.‘~H Halo-nevus-type lesions of the ocular fundus have previously been described,” although not, to our knowledge, in vitiligo patients. The halo has been observed in choroidal melanomas and shown histologically to consist of balloon-cell degeneration Lvithin the peripheral tumor.” Histologic confirmation with benign choroidal melanocytic lesions has been rare. Consistent with the cutaneous counterpart, the presence of a halo around both choroidal nevi and melanomas suggests that it probably has no significance with regard to the malignancy or the malignant potential of the ocular lesion. The incidence ofchoroidal nevi is relatively high” and possibly some of the fundus lesions in vitiligo patients pre\iously thought to be chorioretinal scars may, in f’act, represent advanced ocular halo nevi.
References I.
gressive increase in size of the surrounding area of depigmentation, and (2) its occurrence in association with cutaneous vitiligo. Thus the interpretation of the fundus lesion by the ophthalmologist may depend on recognizing vitiligo of the eyelids, face, neck (Fig. 2), or other regions of the skin. There is considerable evidence that vitiligo may have an immunologic origin. Associated autoimmune disorders such as hyperthyroidism,‘” thyroiditis,’ adrenal insuffIciency,” and pernicious anemia“ are present in 20-30% of vitiligo patients.” If vitiligo indeed has an immunologic origin, any pigment cell in the body, including those in the eye should be affected: normal uveal melanocyte, retinal pigment epithelium, and uveal nevus cell. Albert and associates’ have shown that the uveal melanocyte and retinal pigment epithelium may be affected by vitiiigo. Previously documented ocular changes in vitiligo patients include uveitis, either active or healed,’ as well as multiple changes in the ocular fundus including chorioretinal scars, RPE hypopigmentation and atrophy, and vitiliginous chorioretinitis.’ The patient presented here illustrates a fundus lesion representing the ocular counterpart of the halo nevus. Halo nevus refers to the progressive centripetal extension of a zone of depigmentation about a cutaneous nevus.’ This depigmentation may encircle not only a benign nevus, but also malignant melanomas, as well as cutaneous metasta-
2.
3 4. 5.
6. 7.
8.
9. 10. I I. 12.
13.
If.
.~Ihcrt l).\l. Nordlund ,J,J.
Lrrncr .-\B: Ocular ahnormalitirs nccurrinq \vith vitiligo. O,Mlhn~~no~o,~~HG:I 145-l 1.58. 1979 Coprman I’lV.\l. Ix& Xl<;. I’hillips’l‘M. Elliott PG: ImmunoIlqir association uf thr halo nrvus with cutancuus malignant mc13110ma. U,_.] Dermtl~ol RR: 127-137. 1973 I)ukr-Eldrr S: I)irme.r oft/w 1 ‘maI Tract. S~wtemsofO~hthnlmolo~r. \‘()I 9. St. 1,ouis. C:\’ 1loshy. 1966. pp 373, 558 Epstrin \VI.. Sagrlx-il R. Spitlrr I,. ct al: Halo ncvi and mclanoma. j‘4M.4 L?7:373-377. 1973 Fitzpatrick ‘1‘B, \VolTT EK. Frccdbcrg IM. Austin KF: I$dote, I~ennntolql in Gmernl .\ledicine. NCM. York. McGraw-Hill. 1983. p 237 Hovitz ,J. Schwartz 11: Vitiligo. achorhydria, and prrnicious anrmia. LnncetI: 1331-1334, 1971 Kopf A\V. Llorrill SI). Silxvhcrg I: Broad spectrum of Icukodrrma acquistumitum crntri@yrn. ,4rrh Dermntol W:l4-35, I965 Krrhhs’1‘.4. Rownigk HH,Jr. Drodhar SD: Haloncvus: Competent survrillancc or potential melanoma? Clecelnnd Clinic QU 43: I l-15, 1976 Lcrncr XB. Nordlund ,Jj: Vitiligo: \C’hat is it? Is it important? ,/.4.\1.4 X9:1183-1187, 1978 \IcGrrgor BC:, Katz HI, Doe RI’: Vitiligo and multiple glandular insuflicicncirs. ,/.4.1{.4 219: 1X-125, 1972 Xordlund,J,J. Lrrnrr AB: Vitiligo: It is important. .4rch L)rrmatol lIR:5-7, 1982 Perry HII. Font RI,. Clinical and histopathologicohscrvation in scvcrc \‘ogt-Kovanani-Harada syndromr. Am .J 0,bhthalmoi %:242-254; 197; Rodrieucs MM. Shields 1.4: Maliwant melanoma of the choroid with balloon cells: A clinicopathologic study of three cases. C?zn J Ojhthaimol 11:208-216, 1976 Shields JA: Diagnosis and .2lanagetnent of Intraocular Tumorr. St Louis. (h Moshy. 1983 ,I
.,
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