Nevus angiolipomatosus vs focal dermal hypoplasia

Abstract

Widespread linear hamartoma of vascular elastic, adipose, and connective tissue was observed in 2 girls with another and opposite defect, that of failure of development of focal areas of dermis. No abnormality in number of chromosones was found. Multiple associated defects of development of mesodermal and ectodermal tissues were present. The fat tissue appeared to be associated with vascular proliferation in the papillary bodies and to crowd out the corium by downward infiltration of newly developed fat. Elastic tissue fibers were demonstratable crossing between the fat cells and were prominent in the superficial dermis. There was a definite stratum of dermis dividing the superficial nevus fat and the normal subcutaneous fat. This evidence justifies the explanation of the superficial fat tumors as nevoid neoplasms rather than herniations of subcutaneous fat.