Langerhans Cell Histiocytosis in a 5-Year-Old Girl: A Case Report and Review of Literature

Abstract

Langerhans cell histiocytosis (LCH) is an uncommon hematological disorder affecting infants and young children. LCH is a rare disorder of the reticuloendothelial system associated with proliferation of Langerhans cells and mature eosinophils. LCH can involve any bone, but the most common are pelvis, ribs, skull, long bones, vertebra, and facial bones. In the skull, frontal and parietal bones are commonly involved followed by the jaws, where mandible is more commonly affected than the maxilla. In this article, we report a case of LCH in a 5-year-old child involving the mandible. Swelling of one side of the face and aggressive periosteal reaction led to the diagnosis of monostotic LCH. The manuscript also summarizes the results of a literature search in PubMed of reported cases of LCH over the past 10 years.

Langerhans Cell Histiocytosis in an Adult Female with Multisystem Involvement

“…72 In the skull, frontal and parietal bones are commonly involved followed by the jaws. 73 Mandible is more commonly…”

“…The Indian Journal of Chest Diseases and Allied Sciences, Volume 64 Issue 4 (October-December 2022) involved when compared to the maxilla. 73,74 CT or PET-CT, MRI, or bone scan can delineate the extent of bone and soft tissue involvement. The typical radiographic appearance is a lytic bony lesion with clear demarcation and remodeling at later stages.…”

Langerhans cell histiocytosis in an infant: a case report from North-East India

“…2 Treatment of LCH depends on the site of the lesion, its extent, and the number of lesions present. 3 In patients with LCH limited to the skin, lesions may spontaneously resolve, otherwise a variety of methods may be used, including topical steroids, oral methotrexate or thalidomide, topical nitrogen mustard or psoralens with UV light. 2 Patients may experience long-term permanent consequences that include endocrine disorders (DI and growth hormone deficiency), orthopaedic problems, decreases or loss in hearing and sight, the loss of teeth, neurological defects and impaired lung and liver function.…”

“…2 Recurrence rates around 1.6 to 25% is reported, therefore a close and regular follow-up for a long period is advised. 3 A wide range of clinical spectrum from an asymptomatic form to those with multi-system A B C presentation, diverse prognosis and the risk of long-term complications cause LCH to remain a major challenge. 2…”

“…1 LCH is a rare clonal disease of the monocyte-macrophage system characterized by uncontrolled proliferation and accumulation of CD1a+/CD207+ dendritic cells (DCs) due to continuous immune stimulation, 2 and accumulation of these pathological Langerhans cells causes infiltration and destruction of the local tissues. 3 Clinical feature is highly variable and may range from isolated, self-healing skin and bone lesions to lifethreatening multi-system disease. 2 The cutaneous lesions may be the sole manifestation of LCH where typical manifestation is a seborrheic dermatitis-like lesion on the scalp and the flexural regions.…”