To identify additional genetic factors that may contribute to the pathogenesis of fibromatosis in the setting of Lamin A/C haploinsufficiency, we used exome sequencing and family studies to identify ASTE1 c.230T>C (p.Val77Ala) as a candidate susceptibility variant for DD and LD and a possible role for the ASTE1 gene in the etiology of fibromatosis. The gene discussed is ASTE1; the disease is fibromatosis.