Case of disseminated Langerhans' cell histiocytosis presenting with sclerosing cholangitis


DOGANCI T. , SAYLI T., Gulderen F., ERDEN E., SENCER H.

INTERNATIONAL JOURNAL OF DERMATOLOGY, cilt.43, ss.673-675, 2004 (SCI İndekslerine Giren Dergi) identifier identifier identifier

  • Cilt numarası: 43 Konu: 9
  • Basım Tarihi: 2004
  • Doi Numarası: 10.1111/j.1365-4632.2004.02160.x
  • Dergi Adı: INTERNATIONAL JOURNAL OF DERMATOLOGY
  • Sayfa Sayıları: ss.673-675

Özet

Langerhans' cell histiocytosis is an uncommon disorder of childhood with a wide clinical spectrum. Although liver involvement is common in the disseminated form, presentation with hepatic disfunction is unusual. We describe an 18-month-old girl who presented with intrahepatic cholestasis. The patient was shown to have Langerhans' cell histiocytosis with sclerosing cholangitis by liver biopsy and skin biopsy showing S-100, CD1a positivity, and Birbeck granules by electron microscopy.