Aplasia cutis is a rare congenital absence of skin that can occur on the scalp or other parts of the body. It is often associated with scarring alopecia and can be caused by teratogens or genetic disorders. Treatment ranges from wound dressing to surgical repair, but complications are rare and prognosis is typically excellent.
Aplasia cutis is an extremely rare congenital absence of skin or type of skin loss that occurs during the developing fetus. It is the most common form of congenital scarring alopecia, a type of permanent hair loss that involves the simultaneous destruction of hair follicles and the creation of scar tissue. In addition to the congenital absence of skin and scarring alopecia, aplasia cutis is also known as aplasia cutis congenita, cutis aplasia or congenital scars.
The defect that constitutes aplasia cutis usually occurs, about 70% of the time, on the scalp as a single circular lesion. There are cases, however, where multiple lesions are present and they can be oval or linear in shape. Aplasia cutis can occur in other parts of the body, such as the face, trunk, and limbs. At birth, the lesions have either completely healed or have a scaly, reddish appearance.
In 1986, the condition was divided into classifications based on the possible cause. The source for these categorizations was “Aplasia Cutis Congenita: A Clinical Review and Proposal for Classification” by IJ Frieden, which was published in the Journal of the American Academy of Dermatology. One of the most popular theories for the cause of such congenital scars is the presence of teratogens, which are defined as agents that hinder normal fetal development. Such culprits are usually pharmaceuticals such as methimazole, which is used to treat thyroid problems; and misoprostol, which is used to stimulate labor artificially or prematurely.
Aplasia cutis has also been linked to a rare congenital disorder involving scalp defects called Adams-Oliver syndrome (AOS). Despite an incredibly diverse collection of possibilities, there is no unifying reason for the occurrence of aplasia cutis congenita. Compounding this problem of developing a unifying theory is the rarity of such a disorder, as only 500 cases have been reported since it was first described in the mid-18th century.
Treatment of aplasia cutis ranges from simple wound dressing to surgical repair. The latter option does not apply to small or closed congenital scars. Otherwise, surgeons use skin grafts to cover them. Surgery in some cases, however, comes with a complication due to operating on such a sensitive part of the human anatomy, specifically where the brain resides.
Complications from aplasia cutis include infection, bleeding, meningitis, and a rare form of stroke known as sagittal sinus thrombosis. However, these signs are very rare. Additionally, after treatment of aplasia cutis, the prognosis for the patient is typically excellent.
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