One Disease, Multiple Manifestations

Abstract

<P>A 5-year-old Hispanic girl was evaluated for recurrent torticollis over a period of 1 year, which was interfering with regular activities. Cervical spine studies were normal. Ibuprofen, rest, and parental reassurance were given. Two months later, she was evaluated for persistence of torticollis. On exam, she was apprehensive, her head was tilted to the left, and she refused to move her neck. The left posterior-lateral neck region was tender on palpation. The remaining physical and neurological exam was unremarkable. Magnetic resonance imaging (MRI) showed a destructive process of the left portion of the body of C2 extending into the left lamina and dens, not involving the spinal cord or vertebral artery (see Figure 1). An osteomyelitic process with an inflammatory phlegmon was considered. Biopsy revealed a histiocytic infiltrate with S100 and CD1a expression.</P><H4>ABOUT THE AUTHORS</H4><P>Vani Selvan, MD, is Resident Physician PGY-II, Department of Family Medicine, Texas Tech University Health Sciences Center, Amarillo, Texas. Tetyana L Vasylyeva, MD, is Resident Physician PGY-III, Department of Pediatrics, Texas Tech University Health Sciences Center. Curtis Turner, MD, is Associate Professor, Department of Pediatric Hematology and Oncology, Texas Tech University Health Sciences Center. Osvaldo Regueira, MD, is Assistant Professor, Department of Pediatric Hematology and Oncology, Texas Tech University Health Sciences Center. </P> <P>Address correspondence to Osvaldo Regueira, MD, Assistant Professor, Department of Pediatric Hematology and Oncology, Texas Tech University Health Sciences Center, 1400 Coulter Drive, Amarillo, TX 79106; or e-mail <a href="mailto:regueira@att.net">regueira@att.net</a>.</P><P>Dr. Selvan, Dr. Vasylyeva, Dr. Regueira, and Dr. Turner have disclosed no relevant financial relationships.</P><P>The authors would like to thank Kenneth McClain, MD, PhD, Baylor College of Medicine, Texas Children’s Cancer Center/Hematology Service, Houston, Texas, for help with the diagnosis of these patients.</P> <H4>EDUCATIONAL OBJECTIVES</H4><OL><LI>Review the histopathology of Langerhan’s cell histiocytosis (LCH).</LI><LI>Discuss the varying clinical presentations of LCH.</LI><LI>Demonstrate currently recommended management strategies for the different presentations of LCH.</LI></OL>