X-linked Ichthyosis With Acute Lymphoblastic Leukemia

Abstract

To the Editor.— Sex-linked ichthyosis is caused by a deficiency of microsomal steroid sulfatase activity in all tissues. The incidence is approximately one in 2000 to 6000 men, and the disease is inherited in an X-linked recessive manner. The steroid sulfatase locus has been mapped to the distal segment of the X-chromosome short arm. 1 No association has yet been described between leukemia and X-linked ichthyosis. We describe an adolescent male with these two entities. Report of a Case.— A 13-year-old boy presented with a four-week history of lethargy, bone pain, and progressive pallor. His skin at presentation was dry and scaly, which condition had developed in infancy. Dark scales were especially prominent around the neck and upper shoulders (Fig 1), sparing the flexural areas. There was no lymphadenopathy or splenomegaly. However, his liver was 2 cm below the right costal margin. Results of the remainder of the physical examination, including testicular