X-LINKED ICHTHYOSIS

Course overview

X-LINKED ICHTHYOSIS (XLI), KNOWN AS STEROID SULFATASE (STS) DEFICIENCY AND X-LINKED RECESSIVE ICHTHYOSIS, IS A GENETIC SKIN DISORDER RECOGNIZED IN 1965 BY DRS. WELLS AND KERR. BECAUSE OF THE ABNORMAL SHEDDING, SKIN TENDS TO BE DRY AND ACCUMULATES POLYGONAL SCALES SKIN FINDINGS USUALLY APPEAR WITHIN THE FIRST YEAR OF LIFE, AND 15% TO 20% HAVE MANIFESTATIONS AT BIRTH. THE FLEXURES (POPLITEAL AND ANTECUBITAL FOSSAE), PALMS, AND SOLES ARE SPARED, AS ARE THE HAIR AND NAILS. THIS ACTIVITY REVIEWS THE PATHOPHYSIOLOGY AND PRESENTATION OF X-LINKED ICHTHYOSIS AND HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM IN ITS MANAGEMENT.