A rare case of aplasia cutis – absence of skin, in which there is congenital absence of a portion of skin in a localized or widespread area at birth, with or without bone involvement. Most commonly seen on the scalp, it can affect any part of the body, including the trunk and limbs. While most people with aplasia cutis congenita have no other abnormalities, some patients have congenital malformations involving the cardiovascular, gastrointestinal, genitourinary, and central nervous systems. The exact cause of this condition is unclear and appears to be multifactorial and the contributing factors may include teratogens, genes, infections, trauma, and vascular compromise of blood flow to the skin.
Prognosis is excellent. If the defect is small, recovery is uneventful, with gradual epithelialization and formation of a hairless, atrophic scar over several weeks. Small underlying bony defects usually close spontaneously during the first year of life. Surgical repair of large or multiple scalp defects with excision and primary closure, if feasible, or with the use of tissue expanders and rotation of a flap, may be considered. Truncal and limb defects, despite their large size, usually epithelialize and form atrophic scars, which can later be revised if necessary.