Abstract
A four-month-old baby girl presented with eruptions which started on the trunk a month ago. A physical examination showed scaly erythematous maculopapular eruptions of rice-bean size on the trunk and the thighs. The lesions were distinct, with purpura and erosion evident in places.
The histology showed diffuse infiltration of large hystiocytic cells with bean-shaped nuclei in the dermis and epidermis. These cells were immunohistochemically positive for S-100 protein and CD 1 a. Electron microscope findings revealed the presence of Birbeck granules in the cytoplasm of the histiocytic cells. The patient was diagnosed with Langerhans cell histiocytosis, a single-system, multisite type, after systemic check up. The patient responded well to systemic chemotherapy, and the symptoms have since been in remission.
Isolated skin lesions in LCH occur primarily in early infancy, and show mostly benign prognosis. We reported a case of a single-system skin lesion in LCH, with an overview of some of the literature concerning its treatment and prognosis. [Skin Cancer (Japan) 2006; 21; 327-331]
