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