English

Abstract

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder in which pathological Langerhans cells (LCs) accumulate in a variety of organs. This report describes a boy with Langerhans' cell histiocytosis who presented with primarily soft tissue swellings, without pain. A high index of suspicion is required to reach to a diagnosis of langerhans cell histiocytosis to prevent delay in proper management of this disease. INTRODUCTION: The childhood histiocytoses, although individually rare, varies widely in its clinical expression. These disorders are grouped together because they have in common a prominent proliferation or accumulation of cells of the monocyte-macrophage system of bone marrow origin. (1) A systematic classification of the childhood histiocytoses is based on histopathologic findings. The name Langerhans cell histiocytosis (LCH) has been applied to the class I histiocytoses. The normal Langerhans cell is an antigen-presenting cell of the skin. The hallmark of LCH in all forms is the presence of a clonal proliferation of cells of the monocyte lineage containing the characteristic electron microscopic findings of a Langerhans cell. This is the Birbeck granule, a tennis racket-shaped bilamellar granule that, when seen in the cytoplasm of lesional cells in LCH, is diagnostic of the disease. (1) CASE REPORT: A 4 year old male child presented to our pediatric outpatient department of mamata medical college with the chief complaint of multiple swellings over the forehead for the last one year. The child was apparently normal one year back when his parents noticed multiple small swellings which were gradually progressing in size. The child is a product of non-consanguineous marriage, full term, normal vaginal delivery and has a younger sister who is doing well. On examination there were three swellings over the forehead, which were subcutaneous, nontender margins ill-defined and with underlying bony defects in center of the swellings. Bilateral exophthalmos was present There was no organomegaly, generalized lymphadenopathy, skin manifestations or ear discharge.