Goltz syndrome with multiple eccrine hidrocystomas – Rare case with a novel association

Focal dermal hypoplasia (FDH), also known as Goltz syndrome, is a rare mesoectodermal disorder with very few reports from India. We report a rare case of Goltz syndrome with multiple eccrine hidrocystomas. A 50-year-old female with multiple asymptomatic translucent papules over the face and atrophic macules on the body, with other features suggestive of FDH, was evaluated. Biopsy from the papule revealed features compatible with eccrine hidrocystoma, and from the atrophic macule showed features consistent with FDH. Treatment of FDH is largely supportive and often requires a multidisciplinary approach for effective management.