Langerhans Cell Histiocytosis in Children: Report of six cases with clinicopathological observation / 대한피부과학회지

ABSTRACT

BACKGROUND: Langerhans cell histiocytosis (LCH) is a broad spectrum of clinical diseases having proliferation of Langerhans cells in common. LCH may be manifested in a variety of ways, ranging from a spontaneously regressing skin lesion to a multisystemic life-threatening disorder. Congenital self-healing reticulohistiocytosis is widely regarded as a limited form of LCH. It is present at birth or shortly thereafter and involute spontaneously. OBJECTIVE: Our purpose was to investigate the relationship between clinicopathologic characteristics and prognosis of LCH in children. METHODS: Six children with LCH were investigated clinically and histopathologically. Immunostains were performed with CD1a and S100 antibodies in 5 cases using paraffin-embedded sections and an electron microscopic examination was done in 1 case. RESULTS: Among the 6 cases of LCH, 4 cases were confined to the skin, and 2 cases showed internal organ involvement. All LCH confined to the skin showed resolution of the skin lesion in 1 year, and 2 cases involving internal organ showed bad prognosis. Histopathological findings showed large histiocytes with eosinophilic cytoplasm in common. Immunohistochemical stains (5 cases) showed positive reactions with CD1a and S-100, and electron- microscopical observation in one case demonstrated laminated dense bodies. There was no significant histopathological or immunohistochemical difference between LCH confined to the skin which showed resolution of lesions and LCH involving internal organs with grave prognosis. CONCLUSIONS: Clinical types of Langerhans cell histiocytosis could not be differentiated only from the histopathological findings of the skin. The distinction between Langerhans cell histiocytosis confined to the skin showing spontaneous resolution and congenital self-healing reticulohistiocytosis remains to be clarified.