Progression and flaring of focal dermal hypoplasia during an acute illness

Focal dermal hypoplasia (FDH), or Goltz syndrome, is a rare genodermatosis affecting tissues of mesodermal and ectodermal origin. The characteristic skin changes have been reported to symptomatically flare in response to certain triggers as well as to progress over time in some cases. We present the case of a 5-year-old girl with cutaneous flaring and progression of FDH in the setting of septic shock. This case adds to the growing body of literature on both flaring and progression of the cutaneous manifestations of FDH.