ATYPICAL DENDRITIC CELL-RELATED HISTIOCYTOSIS WITH GOITER AND PRIMARYHYPOTHYROIDISM

Langerhans cell histiocytosis may be seen with goiter and histiocytic infiltration of the thyroid. We report a 21/2-year-old boy who had goi ter and primary hypothyroidism develop, later had pulmonary disease, a nd died of neurologic involvement. Autopsy lesions suggested a transit ional dendritic cell precursor of the epidermal Langerhans cell. Of th e reported cases of Langerhans cell histiocytosis with goiter in child ren and adolescents, 82% were male when the relative incidence of Lang erhans cell histiocytosis is two males to one female.