A 27-year-old man consulted with clinical and radiological features of chronic erosive
oligoarthritis of large joints (hips and knee), associated with diffuse lymph-node enlargement and
diabetes insipidus. Lymph-node biopsy provided the diagnosis of systemic
Langerhans' cell histiocytosis, for which synovial involvement remains a diagnostic challenge.
Infectious diseases search and immunological tests were all negative. Skeleton radiographs, hip and cerebral magnetic resonance imaging showed, respectively, erosive
arthritis of the hips and stigmates of pituitary-stalk involvement. Hip-synovium biopsy exhibited the main histological features of
Erdheim-Chester disease, a
non-Langerhans' cell histiocytosis. An extensive literature review found that
Langerhans' cell histiocytosis and
non-Langerhans' cell histiocytosis (mainly
Erdheim-Chester disease) coexistence is rare and synovial involvements in them even more so, these latter presenting mainly as large joint monoarthritis. The absence of typical clinical and radiographic signs of
Erdheim-Chester disease led to consideration of the rheumatologic diagnosis of unclassified
non-Langerhans' cell histiocytosis (or
Erdheim-Chester disease-type)
oligoarthritis, associated with multiorgan
Langerhans' cell histiocytosis. The differential diagnosis of large joint erosive
arthritis should then include both entities, particularly when multiorgan manifestations are present.
Non-Langerhans' cell histiocytosis synovial involvements responded poorly to
vinblastine and
corticosteroids, while
Langerhans' cell histiocytosis involvements responded completely but transiently. Both entities regressed under
cladribine, with only mild relapses of the
non-Langerhans' cell histiocytosis involvements.