Localized Langerhans cell histiocytosis masquerading as Brodie's abscess in a 2-year-old child: a case report

EXCLI J. 2016 Jan 18:15:33-7. doi: 10.17179/excli2015-713. eCollection 2016.

Abstract

Langerhans cell histiocytosis (LCH), formerly known as histiocytosis X, refers to a spectrum of diseases characterized by idiopathic proliferation of histiocytes that produce either focal (localized LCH) or systemic manifestations (Hand-Schüller-Christian disease and Letterer-Siwe disease). Localized LCH accounts for approximately 60-70 % of all LCH cases. Osseous involvement is the most common manifestation and typically involves the flat bones, along with lesions of the skull, pelvis, and ribs. Localized LCH in bone shows a wide spectrum of clinical manifestations and radiologic features that may mimic those of infections as well as benign and malignant tumors. The diagnostic imaging findings of localized LCH are also diverse and challenging. The penumbra sign is a common and characteristic magnetic resonance imaging (MRI) feature of Brodie's abscess, but is rarely seen in localized LCH. In this report, we describe a case of localized LCH misdiagnosed as Brodie's abscess in a 2-year-old child based on clinical symptoms, laboratory findings, and pre-diagnostic MRI findings (penumbra sign). Therefore, the penumbra sign is not sufficient to clearly establish the diagnosis of Brodie's abscess, and the differential diagnosis of localized LCH should be considered when a child with an osteolytic lesion presents with a penumbra sign.

Keywords: Brodie's abscess; Langerhans cell histiocytosis; differential diagnosis; knee pain; penumbra sign.

Publication types

  • Case Reports