APLASIA CUTIS CONGENITA

Course overview

APLASIA CUTIS CONGENITA (ACC) IS A RARE CONGENITAL SKIN DEFECT CHARACTERIZED BY A FOCAL OR EXTENSIVE ABSENCE OF THE EPIDERMIS, DERMIS, AND OCCASIONALLY SUBCUTANEOUS TISSUE. THE EXACT ETIOLOGY OF ACC IS NOT WELL UNDERSTOOD; HOWEVER, IT IS LIKELY DUE TO IMPAIRED PRENATAL SKIN DEVELOPMENT. THE DISEASE PRESENTS AS SOLITARY OR MULTIPLE LESIONS AND CAN APPEAR ON ANY PART OF THE BODY, ALTHOUGH 70 TO 90 PERCENT OF LESIONS ARE LOCALIZED TO THE VERTEX OF THE SCALP. ACC HAS BEEN CLASSIFIED INTO SIX SUBTYPES, SOME OF WHICH ARE ASSOCIATED WITH CONGENITAL DERMATOLOGIC SYNDROMES. ALTHOUGH MOST LESIONS ARE SELF-HEALING, CERTAIN LOCATIONS AND CLINICAL CHARACTERISTICS SHOULD PROMPT A MORE THOROUGH WORKUP TO SCREEN FOR UNDERLYING SOFT TISSUE ANOMALIES THAT CAN POTENTIALLY BE LIFE-THREATENING. THIS ACTIVITY EXAMINES WHEN APLASIA CUTIS CONGENITA SHOULD BE CONSIDERED ON DIFFERENTIAL DIAGNOSIS AND HOW TO PROPERLY EVALUATE IT. THIS ACTIVITY HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM IN CARING FOR PATIENTS WITH THIS CONDITION.