Elsevier

Joint Bone Spine

Volume 76, Issue 6, December 2009, Pages 701-704
Joint Bone Spine

Case report
Spumous histiocytic oligoarthritis coexisting with systemic Langerhans’ cell histiocytosis: Case report and literature review

https://doi.org/10.1016/j.jbspin.2009.02.007Get rights and content

Abstract

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.

Introduction

Langerhans’ cell histiocytosis (LCH) is a disease of the dendritic Langerhans’cell lineage. Like other entities corresponding to the heterogeneous group of non-Langerhans’ cell histiocytosis (nLCH) including Erdheim–Chester disease (ECD), multicentric reticulohistiocytosis (MCRH), Rosai-Dorfman disease and juvenile xanthogranuloma that correspond to monocyte–macrophage disorders, its etiology and pathophysiology are poorly known and remain controversial [1]. However, LCH and certain nLCH, mainly ECD, share some similarities, suggesting a possible common cellular origin, background and/or pathogenic mechanisms. These entities are rare and exhibit a broad spectrum of specific or common but different organ tropisms of predilection, ranging from multisystemic life-threatening disease to localized and benign bone involvement. First–line therapy for LCH is consensual and consists of the combination of vinblastine and corticosteroids, while treatment of nLCH is less codified. Various chemotherapies and/or immunotherapies, including interferon-alpha, have been used with variable responses [1], [2].

The performance of extensive English literature review using the Medline database found that the coexistence of LCH and nLCH group disorders (mainly ECD) in the same patient and their location on joints has rarely or exceptionally been reported. We report here and discuss about a patient, managements and nosological classifications of an oligoarthritis picture with histological features of ECD, mimicking inflammatory rheumatic diseases and coexisting with an histological proven systemic LCH.

Section snippets

Case report

A 27-year-old man, referred from Algeria to our department in September 2000, had a 10-year history of painless, enlarged cervical and axillary lymph-nodes associated with moderate occasional fever and anemia. A lymph-node biopsy performed in June 1996 showed unspecified histiocytic disease based on light microscopy histology. The patient received several cycles of oral or intravenous dexamethasone until July 2000, with only moderate and transient attenuation. His medical history was

Discussion

LCH and ECD differ in their morphological and immunohistochemical labeling patterns and their ultrastructural features. Both diseases are characterized by positive in situ labeling of CD68, the common marker of the monocyte–macrophage lineage [1], [2], [3], [4], [5].

Our patient's LCH diagnosis was based on morphological and immunohistochemical features in a lymph-node biopsy: pathological histiocyte features with indented nuclei, and protein S-100 and CD1a positivity, the latter being

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