Case of disseminated Langerhans' cell histiocytosis presenting with sclerosing cholangitis

DOGANCI, FERDA; SAYLI, T; Gulderen, F; ERDEN, E; SENCER, H

doi:10.1111/j.1365-4632.2004.02160.x

Case of disseminated Langerhans' cell histiocytosis presenting with sclerosing cholangitis

Atıf İçin Kopyala

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

INTERNATIONAL JOURNAL OF DERMATOLOGY, cilt.43, sa.9, ss.673-675, 2004 (SCI-Expanded)

Yayın Türü: Makale / Tam Makale
Cilt numarası: 43 Sayı: 9
Basım Tarihi: 2004
Doi Numarası: 10.1111/j.1365-4632.2004.02160.x
Dergi Adı: INTERNATIONAL JOURNAL OF DERMATOLOGY
Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
Sayfa Sayıları: ss.673-675
Çukurova Üniversitesi Adresli: Hayır

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.