Sharma Utsav, Songmei Geng
Am J Case Rep 2011; 12:154-158
Available online: 2011-11-08
Background: Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of Langerhans cells, with a broad spectrum of clinical manifestation and prognosis. Here, we present 2 cases of cutaneous LCH in young children with good prognosis.
Case Report: A 6-month-old girl showed generalized multiple reddish-brown papules with crust over the trunk. The second patient was a boy aged 2.5 years who also presented with generalized papules with crust and ulcers over the trunk and was treated for eczema and psoriasis with no improvement. In both cases, routine laboratory and radiological examinations were normal, and skin biopsies revealed atrophic epidermis and a dense infiltration of large histiocytic cells with eosinophilic cytoplasm and reniform nuclei. The histiocytes were positive for S100 and CD1a, thus confirming the diagnosis of LCH. Both patients were prescribed chemotherapy including prednisone, vincristine, and azathioprine for 6 months.
Conclusions: Through this case report, we wish to emphasize the importance of both histopathological and immunohistochemical confirmation for the diagnosis of cutaneous manifestations of LCH, as well as recommending a long-term follow-up to monitor recovery and evidence of relapse.
Keywords: langerhans cell histiocytosis, cutaneous, children