LANGERHANS CELL HISTIOCYTOSIS IN A CHILD WITH ALBINISM AND INVASIVE SKULL LESIONS

Abstract

<h3>Introduction</h3> Langerhans cell histiocytosis (LCH) is a rare disorder that involves excess production of dendritic cells, a form of histiocyte. LCH is heterogeneous in its presentation, varying from single bone lesions to multi-organ failure and considerable mortality. Here, we present a child with LCH who has albinism and persistent skull and orbital lesions. <h3>Case Description</h3> Patient from rural Guatemala was seen at the National Public Hospital of Escuintla during a medical mission trip. He was a 2-year-old boy with congenital albinism and a 6-month history of ulcerative lesions of the right periorbital, frontal, and retroauricular areas with purulent and malodorous discharge (Figure 1A,B). Lesions continued to worsen despite two courses of unknown oral antibiotics and chlorine dioxide rinses. Birth history was unremarkable; he received routine vaccinations including BCG. Skull x-ray showed a large area of decreased lucency in the right frontal and parietal lobe (Figure 1C). MRI of brain and orbits revealed a necrotic mass in the same area (Figure 1D). Wound cultures grew <i>Pseudomonas aeruginosa</i> and intravenous cefepime was initiated. Patient was transferred to the main cancer institute in Guatemala where biopsy of the skull lesion confirmed LCH. <h3>Discussion</h3> The diagnosis of LCH in our case was delayed primarily due to the limited resources and access to medical care. LCH can be invasive, and patients often need a multidisciplinary team approach in a tertiary care center. Additional immunologic studies and genetic evaluation, if available, can provide further insights. To our knowledge, this is the first report of LCH in a patient with albinism.