Different phenotypes of segmental vitiligo based on a clinical observational study

Background Segmental vitiligo and generalized vitiligo are in general considered separate entities. However, clinico-epidemiological data on segmental vitiligo are scarce compared with those of generalized vitiligo. Objective To analyse the clinical profile and distribution pattern of lesions in segmental vitiligo patients. Methods Segmental vitiligo patients were examined and questioned in a prospective and retrospective setting. The distribution and extent of the lesions were evaluated using clinical photographs. Results Different phenotypes of segmental vitiligo were found, including the unilateral segmental type (124 patients; group 1), the bilateral segmental type (three patients; group 2) and the mixed segmental and generalized type (14 patients; group 3). Furthermore, lesions were present with (10%) or without associated halo naevi. The age of onset of segmental vitiligo (median 14 years) was significantly different between the three subgroups (P = 0.028). Extensive involvement of segmental vitiligo lesions on trunk and extremities was significantly (P = 0.031) more observed in patients with a lower age of onset, while the generalized vitiligo lesions in the mixed vitiligo group were mostly very mild. Associated autoimmune diseases were reported in 11%, whereas a positive family history for vitiligo was present in 14.9% of patients. Lesions were not strictly dermatomal nor Blaschkolinear, although a typical recurring pattern could be observed. Conclusion Our data provide clinical evidence that segmental vitiligo and generalized vitiligo are parts of the same disease spectrum and that segmental vitiligo could have a polygenetic background as well. Whether different aetiopathological mechanisms underlie the different clinical phenotypes of segmental vitiligo remain to be elucidated.