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Resident PGY 2
Henry Ford Hospital
HISTORY: A 20-year-old male with history of narcolepsy presented to the dermatology clinic for 5-month history of asymptomatic light spots on his trunk and graying of his hair. He initially noted acute onset of light spots slowly enlarging around moles on his chest and abdomen. Three months later, he developed slowly enlarging light spots disassociated with moles on his back and grey hairs on the back and top of his scalp. He denied new or changing moles and had no associated symptoms. Personal and family history was negative for vitiligo, autoimmune conditions, and skin cancers.
EXAMINATION: On examination, the patient has numerous, isolated hypo- to depigmented macules and patches on the trunk and back, some are surrounding symmetric, evenly pigmented, well-demarcated 3-6-mm brown-black macules. The left upper inner arm had two small de-pigmented patches and the right inner thigh had one hypopigmented patch. The scalp is noted to have diffuse hypopigmentation with associated graying of hair. Wood’s lamp examination revealed enhancement and depigmentation of these macules and patches.
COURSE AND THERAPY: The patient was started on topical betamethasone dipropionate 0.05% ointment twice daily to the hypopigmented and de-pigmented patches, and both Gingko biloba and alpha Lipoic acid capsules daily.
DISCUSSION Halo nevi (HN, also known as Sutton's nevus) are thought to be a benign finding affecting 1% of the population. The diameter of the depigmented halo may range from <1 mm to several centimeters, just as the size of the nevi themselves may range from a few millimeters to centimeters. Males and females are equally affected, and the mean age of onset is 15 years. Patients with Turner’s syndrome have an increased tendency and a familial variant has been reported. While the exact pathophysiology of HN is poorly understood, melanocytes are absent under histopathology, suggesting an etiologic link to vitiligo. The inflammatory composition of halo nevi is predominantly T-lymphocytes (4:1 ratio of CD8:CD4) with scattered macrophages. Overall, an immunologic mechanism seems to be the cause of melanocyte destruction in HN, although the trigger and role of lymphocytes remains uncertain. Although usually benign, HN have two important significant associations: vitiligo and melanoma-associated leukoderma. In fact, approximately 20% of individuals with HN have vitiligo. A retrospective study of 101 patients with HN-associated vitiligo demonstrated that, under multivariate analysis, Koebner phenomenon, multiple HN, and family history of vitiligo are independent factors for HN appearance predicting vitiligo. Of these, our patient had only multiple HN. However, he also had premature hair graying (PHG). Lesional leukotrichia and family history of PHG are sometimes seen in patients with non-segmental HN-associated vitiligo. However, reports of PHG occurring concurrently in a patient with acute presentation of HN-associated vitiligo are lacking. Ezzedine and colleagues remark that PHG is an inherited trait, but an immunological factor may be implicated in a subset of cases. Nevertheless, PHG is an unusual finding in a patient with HN-associated vitiligo and should prompt all practitioners to search for melanoma. Thus, patients should be referred to dermatology for a full body skin and oral examination, ophthalmology to assess for uveal melanoma, and if appropriate, OBGYN to assess for mucosal melanoma. A thorough total body skin examination of our patient did not reveal lesions concerning for melanoma. He is pending examination by Ophthalmology. Thus, his acute clinical presentation of HN, non-segmental vitiligo, and PHG represents a rare triad.
Ramachandran, Vignesh; Kim, Katelyn; Fidai, Chelsea; and Zhang, Lisa B., "Disseminated non-segmental vitiligo with halo nevi and grey hair" (2020). Case Reports. 64.