What are the clinical features of multicentric reticulohistiocytosis. Pdf multicentric reticulohistiocytosis is a rare systemic disease described as. Specific treatment for multicentric reticulohistiocytosis. The utility of fdgpetct imaging in the evaluation of multicentric reticulohistiocytosis. Skin nonmelanocytic tumor multicentric reticulohistiocytosis.
Mrh support the multicentric reticulohistiocytosis. Patients are left with crippling, deformed joints and disfigured facial appearance. Images were acquired using a cooled ccd camera attached to a light. Multicentric reticulohistiocytosis is a rare systemic disease with proliferation of histiocytes which leads to destructive polyarthritis and skin lesions. Multicentric reticulohistiocytosis is a disease that is characterized by the presence of papules and nodules and associated with arthritis mutilans.
There is a recognized associated with various malignancies up to 25% of cases 9. Multicentric reticulohistiocytosis mrh is a rare disease characterized by the. Cureus a rare case of multicentric reticulohistiocytosis. Cutaneous nodules may be seen as soft tissue swellings. Multicentric reticulohistiocytosis clinical presentation. What is the treatment for multicentric reticulohistiocytosis. The disease can involve the skin, the bones, the tendons, the muscles, the joints, and nearly any other organ e.
Multicentric reticulohistiocytosis is a rare granulomatous disease of unknown etiology, characterized by cutaneous nodules and destructive arthritis. Multicentric reticulohistiocytosis mrh is a rare disease in which papulonodular skin lesions containing a. Multicentric reticulohistiocytosis is a rare disorder characterized by cutaneous papulonodular lesions and destructive arthritis. Multicentric reticulohistiocytosis is more common in women than men, with a ratio of 3. Multicentric reticulohistiocytosis mrh is a rare nonlangerhans. There is a predilection for females and people of northern european descent. Although initial histological findings did not match the clinical image, additional biopsies were valuable to confirm the diagnosis. A rare case of multicentric reticulohistiocytosis with. Case of the week 324 on awardwinning, radiologic teaching site for medical students and those starting out in radiology focusing on chest, gi, cardiac and musculoskeletal diseases containing over 200. We describe the common and uncommon clinical and imaging. Multicentric reticulohistiocytosis mrh is a rare and debilitating systemic disorder. Skin lesions are characterized by cutaneous papules and nodules.
The utility of fdgpetct imaging in the evaluation of. Other organs may be involved and 20% of patients have an. Pdf multicentric reticulohistiocytosis mrh is a rare nonlangerhans histiocytosis of unknown etiology with a predilection for. Multicentric reticulohistiocytosis mrh is a rare histiocytic disorder that involves the skin, joints, and visceral organs. Multicentric reticulohistiocytosis mrh is a rare disease in which papulonodular skin lesions containing a proliferation of true histiocytes macrophages are associated with arthritis. The combination of radiographic findings and the patients presentation in this case suggest multicentric reticulohistiocytosis mcrh as a diagnosis, but do not rule out other differential considerations such as rheumatoid arthritis and other inflammatory arthritides or gout. Multicentric reticulohistiocytosis genetic and rare. The patient was put on short course of systemic steroids after which the patient was lost for follow up.
If only the pancarpal destructive changes in the left wrist are. This case report describes a newly diagnosed case of multicentric reticulohistiocytosis. In approximately 1 in 4 cases, multicentric reticulohistiocytosis is associated with an internal malignancy 1. Multicentric reticulohistiocytosis mrh is a rare systemic inflammatory granulomatous disease that primarily manifests clinically with severe erosive arthritis and widespread papulonodular. The combination of radiographic findings and the patients presentation in this case suggest multicentric reticulohistiocytosis mcrh as a diagnosis, but do not rule out other differential considerations such as rheumatoid arthritis. Multicentric reticulohistiocytosis mrh is a rare disease in which papulonodular.
We describe a 42yearold man with a fiveyear history of arthritis mutilanslike destructive joint changes and with a oneyear history of nodules on the fingers, ears, oral mucosa, pharynx, larynx, vocal cords, some being ulcerated and haemorrhagic. Backgroundpurpose multicentric reticulohistiocytosis mrh is a rare systemic inflammatory disease with skin nodules and arthritis. Infiltration of histiocytes and multinucleated giant cells are typical histological findings and confirm the diagnosis. Imaging should include xray, magnetic resonance imaging of involving joints, skin or synovial tissue biopsy for mrh, which usually. The mean age of people affected by multicentric reticulohistiocytosis is 50 years old 2. Multicentric reticulohistiocytosis is a rare disease characterized by raised, red bumps on the skin and arthritis. Autoimmune diseases including sjogren syndrome and primary biliary cirrhosis 3 hyperlipidaemia.
It is classified as a nonlangerhans cell histiocytosisclass iib. Thalidomide, a rational agent for treatment of multicentric. Multicentric reticulohistiocytosis radiology reference. Multicentric reticulohistiocytosis an overview sciencedirect topics. Multicentric reticulohistiocytosis mrh learningradiology. Multicentric reticulohistiocytosis definition glossary. Multicentric reticulohistiocytosis mcr is a rare disease characterized by papulonodular skin lesions of the hands, forearms, face, and ears associated with erosive arthritis. Diffuse cutaneous reticulohistiocytosis jama dermatology.
Multicentric reticulohistiocytosis is a multisystem disease beginning usually around the age of 50 years, and is twice as common in women 718 see also. Skin lesions are characterized by cutaneous papules and. Pathology outlines multicentric reticulohistiocytosis. Discussion multicentric reticulohistiocytosis mrh is a rare non. Magnetic resonance mr imaging is increasingly being used in the assessment of rheumatoid arthritis due to its capacity to help identify the key pathologic features of this disease entity at presentation. The morphologic, light microscopic, and ultrastructural features of the skin lesions were typical of multicentric reticulohistiocytosis.
Multicentric reticulohistiocytosis mrh is a rare disease with multisystem involvement. Multicentric reticulohistiocytosis radiology reference article. Multicentric reticulohistiocytosis in a patient with. The definitive diagnosis depends on biopsy of the affected tissues. Note the lack of osteoporosis, the marked destruction of not only the carpals but of all the interphalangeal joints, the noncalcified soft tissue masses white arrows. Pdf a case of multicentric reticulohistiocytosis researchgate. Smith field, beaufort, north carolina, united states and 69 more what is the abbreviation for multicentric reticulohistiocytosis. Multicentric reticulohistiocytosis is a rare disease affecting skin and joints primarily and rarely other organs.
Multicentric reticulohistiocytosis mrh, also known as lipoid dermatoarthritis, is a rare systemic disorder. Magnetic resonance imaging mri and computed tomography can detect abnormal iron accumulation in involved abdominal organs and the heart. Multicentric reticulohistiocytosis is a disease that is characterized by the. However, imaging continued to show effusions in the hand joints and. Multicentric reticulohistiocytosis acr note distribution and soft tissue swelling shoulder views. Magnetic resonance imaging mri and computed tomography ct scanning. A rare histiocytic proliferative disorder, first described in 1954 by goltz and laymon, in which joints, skin and mucous membranes are affected. On skin or joint biopsy the hallmark is the presence of multinucleated. Treatment of multicentric reticulohistiocytosis with. Mrh stands for multicentric reticulohistiocytosis also iata code for michael j. Tumor necrosis factor antagonists in the treatment of multicentric. Multicentric reticulohistiocytosis was first named by goltz and laymon in 1954, although the literature is muddled with prior reports of conditions with similar features. Multicentric reticulohistiocytosis mrh is a rare systemic disease that can produce skin changes usually papulonodular eruption, mucosal lesions, and arthritis, generally with erosive. Multicentric reticulohistiocytosis mrh is a rare inflammatory disease characterized by skin nodules and associated with destructive arthritis.
Multicentric reticulohistiocytosis case series from a. Note the lack of osteoporosis, the marked destruction of not only the carpals but of all the interphalangeal joints, the noncalcified soft. Successful treatment of multicentric reticulohistiocytosis. Multicentric reticulohistiocytosis is a rare form of nonlangerhans cell histiocytosis presenting with skin changes and erosive arthritis. Multicentric reticulohistiocytosis mrh is a rare, multisystemic nonlangerhans cell histiocytosis characterized by skin and articular involvement, and rarely involves various other organs. Hemochromatosis, wilsons disease, ochronosis, fabry. Multicentric reticulohistiocytosis is a multisystem disease affecting adults, with a mean age of 50 years. Targetable driver mutations in multicentric reticulohistiocytosis. Practice essentials multicentric reticulohistiocytosis mrh is a rare disease in which papulonodular skin lesions containing a proliferation of true histiocytes macrophages are associated with. In many patients, after an average course of 8 years, the disease can go into remission, however, by this time considerable joint destruction may have occurred. A case is described of multicentric reticulohistiocytosis or lipoid dermatoarthritis with early radiographic findings of subchondral erosions of the distal interphalangeal joint space, more marked.
The arthritis involves the interphalangeal joints and in 45% of cases causes severe joint destruction known as arthritis mutilans. Case report multicentric reticulohistiocytosis presenting with papulonodular skin lesions and arthritis mutilans rayasaba,shawng. Multicentric reticulohistiocytosis mrh is a rare multisystem macrophage disorder of unknown etiology characterized by papulonodular skin and mucosal lesions, rapidly progressive erosive symmetric. Specific treatment for multicentric reticulohistiocytosis has not yet been established 3, 4. A patient with multifocal reticulohistiocytosis mrh of skin and joints failed treatment.
Multicentric reticulohistiocytosis mr, mrh, previously also called lipoid dermatoarthritis is a rare disease, primarily affecting patients in their adulthood characterized by the. The skin, mucosa, synovial, bone, and internal organs may. Multicentric reticulohistiocytosis is a rare multisystem disorder of unknown etiology that is characterized by erosive polyarthritis and papulonodular lesions on the skin, mucous membranes, and internal. Multicentric reticulohistiocytosis is considered a form of granulomatous disease 10 characterized by infiltration of multinucleated histiocytes into various tissues, primarily affecting skin and synovium. Multicentric reticulohistiocytosis is considered a form of granulomatous disease 10 characterized by infiltration of multinucleated histiocytes into various tissues, primarily affecting skin and synovium, which results in an erosive, deforming polyarthritis associations. Multicentric reticulohistiocytosis presenting with. Multicentric reticulohistiocytosis is relatively resistant to glucocorticoids and to diseasemodifying antirheumatic drugs dmards such as methotrexate and hydroxychloroquine. There is no specific treatment for multicentric reticulohistiocytosis. Infiltration of histiocytes and multinucleated giant.
601 614 814 1262 197 191 446 798 7 345 978 6 1542 557 1252 35 326 1241 419 1404 410 207 510 668 923 1423 848 522 514