Genital Vitiligo in Children: Factors Associated with Generalized, Non-Segmental Vitiligo

BACKGROUND/OBJECTIVES: It remains uncertain as to whether genital vitiligo of childhood is segmental or a forme fruste of non-segmental disease. This type of vitiligo is sometimes termed figure-of-eight disease due to female predominance and perineal and perianal hypopigmentation or depigmentation around two orifices, the anal and vaginal introitus. The objective of this series was to categorize the features of genital-limited vitiligo of childhood including comorbidities, family history, and long-term course. METHODS: IRB-approved retrospective chart review of vitiligo cases. RESULTS: Eight cases of vitiligo that began in childhood in the genital area were identified, including clinical course and comorbidities. Involvement of the contiguous skin, including inner thighs, buttocks, and scrotum, was noted in all males. In females, only 1 patient had other sites of vitiligo, which was a solitary lesion on the chest. Therapeutic response to topical corticosteroids for perianal skin was seen in five of six children and topical calcineurin inhibitors with or without calcipotriene in four out of four patients. CONCLUSIONS: Segmental disease limited to the genitalia was more common in females than males, suggesting that perhaps intervention and work-up for comorbid autoimmune diseases may be required for males with genital-limited vitiligo at onset, but may be deferred in females and added if observation of spread is noted. Differentiation from lichen sclerosus et atrophicus may require biopsy