DR.MOHAMMED IRFAN SHEIKHabout 1 month ago

Langerhans'-Cell Histiocytosis

A 2-year-old boy presented with a 1-year history of refractory eczema and a 4-week history of enlarging neck masses. On physical examination, his weight and height were below the fifth percentile for his age. He had bilateral proptosis, serous discharge from both ears, and extensive rash on the scalp, chest, and upper back, along with palpable defects in the skull and bilateral posterior cervical, submandibular, and supraclavicular lymphadenopathy. The patient did not have hepatosplenomegaly or diabetes insipidus at diagnosis. Laboratory testing revealed anemia of chronic disease, with a hemoglobin level of 6.6 g per deciliter, normal white-cell and platelet counts, and normal results for liver function. A skeletal survey revealed multiple lytic lesions in the skull, with associated soft-tissue masses, and in the right 10th rib. Results on lymph-node biopsy were consistent with Langerhans'-cell histiocytosis. The patient was treated with prednisone, vinblastine, and mercaptopurine and had a complete recovery with a resolution of abnormalities. At 24 months after diagnosis, the patient remained well. Langerhans'-cell histiocytosis is a rare histiocytic disorder, with the highest incidence in toddlers. It should be suspected in a young child with refractory eczematous rash and chronic ear discharge.