Familial cutaneous collagenoma syndrome (FCC) is characterized by the development of collagenomas, which are a type of connective tissue nevi. In FCC, collagenomas present as skin-colored asymptomatic nodules on the trunk and upper extremities.

FCC is an extremely rare, autosomal dominant condition with approximately 10 affected families reported. Patients typically present at puberty between the ages of 15 and 19 but can develop nodules as early as age 4. One family with FCC demonstrated a splice site mutation in the LEMD3 gene. Different loss of function mutations in LEMD3 are found in Buschke-Ollendorff syndrome, which results in connective tissue nevi but is also associated with bone malformations. Unlike Buschke-Ollendorff syndrome, FCC is not associated with bone defects. However, FCC is infrequently associated with cardiac anomalies and cardiomyopathy.