Pediatric Erdheim-Chester disease with aggressive skin manifestations

Abstract

Erdheim-Chester disease (ECD), one type of systemic non-Langerhans cell histiocytosis, is uncommon and characterized by the accumulation of CD68+/CD1a- foamy histiocytes. It is extremely rare in children. The skin lesions of pediatric ECD was not systemically described before. We report a case of ECD in a 3.5 year old Chinese boy. The patient present with generalized skin and bone involvement for 3 years duration. Marked generalized annular maculopapular lesions with central atrophy which differed from previously reported adult xanthoma-like papules or periorbital xanthelasma-like lesions. Computed tomography revealed diffuse pulmonary fibrosis and generalized skeletal involvement including osteolysis and osteosclerosis. The presence of CD68+/CD1a- histiocytes allowed the diagnosis of ECD to be made. According to our review of the literatures, this is pediatric ECD cases with the youngest age of onset. The generalized skin involvement made our case unique in comparison to previously reported.

This article is protected by copyright. All rights reserved.

http://ift.tt/2iJxHUz