Cutaneous Keratocyst With D2-40 Immunoreactivity in Basal Cell Nevus Syndrome

Although not a diagnostic criterion for basal cell nevus syndrome (BCNS, OMIM#109400), cutaneous cysts, particularly epidermoid cysts, are common in this condition. Cutaneous keratocysts, on the other hand, are extremely rare in general and have been identified in only 5 patients with BCNS. Here, we describe a BCNS patient with a cutaneous keratocyst that demonstrated D2-40 (podoplanin) immunoreactivity, which has been detected in odontogenic keratocysts but not cutaneous keratocysts. This finding suggests that cutaneous keratocysts may be developmentally homologous to odontogenic keratocysts and may behave similarly in terms of invasion and growth pattern.