Case Report: Multicentric Reticulohistiocytosis Associated With Posterior Mediastinal Adenosquamous Carcinoma, Antinuclear Antibody Positivity and Lupus Anticoagulant Positivity

Multicentric reticulohistiocytosis (MRH) is a rare systemic disease of non-Langerhans cell histiocytosis. A number of studies in the literature have documented that it can coexist with malignancy or autoimmune disease, making it difficult to determine the most appropriate therapy. Here, we present a case study of MRH associated with posterior mediastinal adenosquamous carcinoma along with antinuclear antibody positivity and lupus anticoagulant positivity. The patient experienced 6 months of clinical benefit after surgical resection and chemoradiotherapy of the mediastinal malignancy. This case adds to the available literature on multicentric reticulohistiocytosis associated with different types of malignancy and provides supplementary clinical data on the coexistence of this syndrome with malignancy and immune system abnormalities. To the best of our knowledge, this is the first case study describing MRH accompanied by posterior mediastinal adenosquamous carcinoma and lupus anticoagulant positivity. The unknown aetiology and polymorphic clinical presentation of MRH warrants further investigation.

Clinical Characteristics of Multicentric Reticulohistiocytosis and Distinguished Features from Rheumatoid Arthritis: A Single-Centre Experience in China

ObjectiveTo investigate the clinical features of multicentric reticulohistiocytosis (MRH). MethodsThe clinical manifestations, laboratory examination results and histologic characteristics of eleven patients with MRH were collected and compared with those of 33 patients with rheumatoid arthritis. ResultsIn total, 72.7% of the MRH patients were women. The median age was 46 years (range: 33-84 years). Diagnosed by specific pathologic features, all MRH patients exhibited cutaneous involvement. The dorsa of the hands, arms, face and auricle were the most commonly affected areas. Nodules were also located on the legs, scalp, trunk, neck, and even the hypoglossis and buccal mucosa. Ten MRH patients (90.9%) had symmetric polyarthritis. Compared with rheumatoid arthritis (RA) patients, MRH patients were more likely to have distal interphalangeal joint (DIP) involvement (63.6% vs 24.2%, P=0.017) and less likely to have elbow (36.4% vs 72.7%, P=0.003), ankle (45.5% vs 93.9%, P<0.001) and metacarpophalangeal joint (MCP) (36.4% vs 78.8%, P=0.009) involvement. The positivity for rheumatoid factor (RF) (36.4% vs 84.6%, P=0.001) and anti-CCP antibody (9.1% vs 81.8%, P=0.000), as well as the median RF titer [43.8 (31.7-61.0) vs 175.4 (21.3-940.3), P = 0.021], in MRH patients was lower than that in RA patients. Elevation of the erythrocyte sedimentation rate (ESR) was also less common in MRH patients than in RA patients (36.4% vs 72.7%, P=0.030). After treatment with median- to large-dose corticosteroids and disease-modifying antirheumatic drugs, 8 patients achieved complete remission, and 2 patients achieved partial remission (skin lesions ameliorated, joint lesions not ameliorated). ConclusionAlways pathologically diagnosed, MRH is a systemic disease involving RA-like erosive polyarthritis and a specific distribution of skin nodules characterized by "coral beads". More DIP involvement and less elbow, ankle and MCP involvement are seen in MRH than in RA. In addition, less positive and lower-titer RF, uncommon presence of anti-CCP antibodies and ESR elevation may be helpful to distinguish MRH from RA.

Multicentric Reticulohistiocytosis - A Rare Clinicopathological Entity

This is a rare case report of a thirty-eight-year-old male who presented with multiple asymptomatic hyper-pigmented papulonodular lesions on the face for the past two years without the association of pain or pruritus and with restriction of elbow movements. The diagnosis of multicentric reticulohistiocytosis was made on histopathological findings of Touton type of giant cells and sheets of foamy histiocytes along with immunohistochemistry (IHC) studies. Workup was done for other associated diseases. Multicentric reticulohistiocytosis (MRH) is also known as lipoid dermatitis,1 a rare disease which is characterised by the presence of extensive papulonodular cutaneous eruptions and severe, sometimes destructive arthropathy, followed by eruption of the skin and mucous membrane lesion.2,3 It’s a rare idiopathic nonLangerhans cell histiocytosis.4 This disorder is characterised by predominant cutaneous manifestation and joint involvements. The lesions may show regression and recurrence, many case studies show an association of this lesion with internal malignancies, autoimmune diseases, hyperlipidaemias, and tuberculosis.5,6 Few cases have shown musculoskeletal involvement with features such as myositis. The disease was described initially as reticulocytosis granuloma in 1952 by Caro and Senear, later the term was coined by Goltz and Laymon as multicentric reticulohistiocytosis in 1954.4 It is also known by different names such as giant cell histiocytosis, lipoid dermato-arthritis, lipoid-rheumatism and reticulohistiocytosis granuloma. The disease incidence is very low worldwide, less than 200 cases have been reported in literature5 and reports from India are limited.