When Should Cutis Aplasia be Worked Up?

Discussion Aplasia cutis congenita (aka cutis aplasia, CA) is an uncommon dermal defect that is usually noted at birth or soon after birth. A variable amount of dermal tissue is absent. It is usually an isolated defect. The actual causes are unknown but genetics, developmental and destructive forces are logically the cause. Developmental forces do not allow the appropriate formation and closure of the skin (example would be a syndrome) and destructive forces (such as amniotic bands) harm the tissue so that it is disrupted. On physical examination CA may look like a small hypertrophic or atrophic scar. It can also be covered or uncovered (if covered there may be associated fluid or blood), have ulcerations, erosions or blisters of the skin which can involve the deeper tissues including having bony abnormalities in up to 15-30% of patients. Scalp lesions are very common. Some other associated syndromes include Adams-Oliver syndrome (e.g. limb defects, cardiovascular and central nervous system abnormalities), Bart Syndrome (e.g. gastrointestinal problems including oral ulceration and pylorus atresia, head abnormalities), fetus papyracesus (e.g. associated with demise of fetal twin, skin defects, limb defects), amniotic band sequence, and congenital Volkmann ischemic contractures. Learning Point CA has an estimated incidence of 3:10,000 live births. As CA is relatively rare and each person unique, there is not a standard evaluation and treatment recommendations. However there are...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news