Multicentric Reticulohistiocytosis: A Report of Two Cases

Multicentric reticulohistiocytosis (MR) is a rare multisystemic disorder of unknown aetiology characterized by cutaneous and joint manifestations. It is associated with malignancy in up to 31% of cases. Common radiological findings are peri-articular erosions and osteolytic punched-out areas. We present a case of MR with cutaneous nodules, joint pains, and multiple lytic skull lesions--a combination that has not been described before. Osteolytic activity of proinflammatory cytokines (tumour necrosis factor-alpha and interleukin-1) may explain the peri-articular erosions often seen in MR, and the multiple lytic skull lesions seen in our patient.

Multicentric reticulohistiocytosis (MRH) is a very rare multisystemic syndrome.1,2 The first case of MRH was described by Goltz and Layman in 1954 and so far only less than 200 cases have been reported.3-5 It is characterized by the insidious onset of polyarthritis that often evolves into a severe erosive deforming arthritis and characteristic skin lesions composed of nodules and plaques containing lipid-laden (periodic acidSchiff-positive) histiocytes and multinucleated giant cells.6 It most commonly affects the handsand cervical spine.7 MRH is also known as lipoid dermatoarthritis, lipoid rheumatism, and giant cell reticulohistiocytosis.4 MRH is occured due to infiltration of multinucleated giant cells and histiocytes into various tissues. The typical pictures include skin nodules and destructive polyarthritis.3 This entity is frequently mistaken for rheumatoid arthritis (RA).3 MRH is often associated with systemic complication and various types ofmalignancy. Therefore, sometimes it is considered a paraneoplastic syndrome

Multicentric reticulohistiocytosis (MRH) is a rare non-Langerhans histiocytosis of unknown etiology with a predilection for joint and skin. The characteristic clinical features are papulonodular skin eruptions and inflammatory polyarthritis, sometimes progressive to arthritis mutilans, a severe destructive arthropathy. Although these manifestations can present at the same time, it is more common that one feature precedes the others. Notably, these features are similar to those found in some rheumatic diseases, such as rheumatoid arthritis or dermatomyositis, and this can lead to a misdiagnosis, especially during periods where only one feature is present. Herein, we report a female patient with polyarthralgia and subsequent skin eruptions, who was eventually diagnosed with MRH. Her symptoms seemed to resemble those of some rheumatic diseases, but several features such as affected joints and the characteristic shape of the skin lesions did not correspond to that. The histological result of infiltration of histiocytes and multinucleated giant cells in the skin ultimately facilitated the correct diagnosis.In this paper, we review MRH briefly and highlight several differential points which enable us to increase the likelihood of correctly diagnosing MRH.

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. 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 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 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 positivity and lower-titer RF, uncommon presence of anti-CCP antibodies and ESR elevation may be helpful to distinguish MRH from RA.

Multicentric reticulohistiocytosis presenting with clinical features of dermatomyositis

Multicentric reticulohistiocytosis (MRH) is a rare systemic disorder belonging to non-Langerhans cell histiocytosis, which is characterized by severe destructive arthritis and multiple skin nodules. It is thought that proinflammatory cytokines, such as tumour necrosis factor-α (TNF-α), interleukin 1β (IL-1β), IL-6 and IL12 secreted by monocytes and macrophages, may be associated with the pathogenesis of skin lesions and destructive arthritis in MRH, and that the inhibition of these cytokines may be useful for therapy (1, 2). There are several case reports of successful or unsuccessful treatments using anti-TNF-α reagents, such as infliximab (3–6), etanercept (7–9) and adalimumab (10, 11). We report here a case of MRH that was resistant to combination therapy of prednisolone, methotrexate (MTX) and golimumab (another anti-TNF-α drug administrated subcutaneously every 4 weeks), but finally responded to prednisolone, MTX and adalimumab.

&lt;p&gt;Multicentric reticulohistiocytosis (MRH) is a rare disease characterized by nodular skin lesions and severe erosive polyarthritis which is associated with malignancy in some cases. The diagnosis is confirmed by the presence of oncocytic histiocytes and multinucleated giant cells on histopathology of the cutaneous nodules and the synovial membrane. It usually remits spontaneously after 5-8 years but it can provoke destructive arthritis. We report a case of a 49-year-old female who presented with numerous nodules on the both hands, face and abdomen and progressive destructive polyarthritis of 3 years duration and has been diagnosed with rheumatoid arthritis. The lesion showed large histiocytes with ground-glass eosinophilic cytoplasm, typical of MRH. Clinical manifestation and radiological pattern of MRH may be misdiagnosed as other disease like rheumatoid arthritis and psoriatic arthritis, but histopathologic findings of our case can differentiate MRH from any other conditions.&lt;/p&gt;

Multicentric reticulohistiocytosis accompanied by oral and temporomandibular joint manifestations

We report the case of a 42-year-old Japanese woman who developed multicentric reticulohistiocytosis (MR) complicated by systemic sclerosis (SSc) and Sjögren syndrome (SS). The patient complained of tender nodules on the left hand, polyarthralgia in the finger joints and knees, and xerostomia. The skin nodules were distributed mainly on her hands and fingers with skin sclerosis. The serum anti-nuclear test revealed anti-centromere antibody and the discrete speckled pattern of anti-nuclear antibody. The biopsy specimens from the finger nodule and the sclerotic finger skin showed a perivascular infiltration of multinucleated giant cells with ground-glass cytoplasm and dermal thick collagen proliferation, respectively. The lip biopsy and sialography specimens showed periductal lymphocyte infiltration and apple tree-like changes. Systemic corticosteroid treatment improved the polyarthritis, xerostomia, and skin sclerosis rapidly but suppressed the nodular lesions only gradually. This is the first report of a combined case of MR, SSc and SS. This multiple autoimmune complication suggests the involvement of an immunological disturbance in the pathogenesis of MR.

Multicentric reticulohistiocytosis: A report of a case and a review of the pathology

Multicentric reticulohistiocytosis is a special form of an erosive, destructive arthropathy. It can be distinguished from other forms of erosive polyarthropathies by the characteristic x-ray appearances, although the final proof of the diagnosis is histological in association with the clinical features and the presence of nodular skin lesions. A case of multicentric reticulohistiocytosis is described. The typical radiological appearances are discussed and the differential diagnosis from other forms of chronic polyarthritis, such as gout, psoariatic arthropathy, Reiter's disease and erosive osteoarthritis is considered.

A case of multicentric reticulohistiocytosis in an 8-year-old girl, which is a diagnosis rarely seen in children, is presented. Multicentric reticulohistiocytosis is a disorder of unknown aetiology, predominantly affecting the joints, skin and mucosa. Joint symptoms, but not cutaneous lesions, have improved with treatment with methotrexate.

Journal Article Multicentric reticulohistiocytosis a review of eight cases Get access M.D. CATTERALL M.D. CATTERALL Department of Dermatology, Royal Naval Hospital, Haslar, Gosport, Hants PO12 2AA Search for other works by this author on: Oxford Academic Google Scholar Clinical and Experimental Dermatology, Volume 5, Issue 3, 1 September 1980, Pages 267–279, https://doi.org/10.1111/j.1365-2230.1980.tb01705.x Published: 01 September 1980 Article history Accepted: 07 November 1979 Published: 01 September 1980

A case of multicentric reticulohistiocytosis is reported. It is a systemic condition but the cause is not known. Subjective and objective improvement occurred after the patient received a course of nitrogen mustard and ACTH followed by chlorambucil. Results of blood lipid studies were normal, and histochemical examination of the material in the giant cells showed a polysaccharide and a phospholipid present. There is no evidence of abnormal lipid metabolism, so rather than using the term ``lipoid dermato-arthritis,'' we prefer the term ``multicentric reticulohistiocytosis.''

Multicentric reticulohistiocytosis: Report of a case, with atypical features and electron microscopic study of skin lesions