Case of disseminated Langerhans' cell histiocytosis presenting with sclerosing cholangitis

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Doganci T., Sayli T., Gulderen F., Erden E., Sencer H.

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

• 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
• Ankara 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.