Skin lesions on the hands, especially at the base of the nails lesions may also be on the face, ears, arms, scalp or mucosal surfaces. Multicentric reticulohistocytosis is characterized by cutaneous or mucosal papular lesions often associated with severe polyarthritis and arthralgias. Histopathologically, the lesion was composed of oncocytic macrophages and multinucleate giant. Hydroxychloroquine was added to this regimen for 7 months with little benefit. A case of mrh arthropathy, in which the typical nodular skin manifestation of the disease was absent, is described in a patient with a past history of breast cancer and no evidence. Recently, she presented with symmetric distal interphalangeal joint. Sep 28, 2012 multicentric reticulohistiocytosis is a disease that is characterized by the presence of papules and nodules and associated with arthritis mutilans. If only the pancarpal destructive changes in the left wrist are. Cureus a rare case of multicentric reticulohistiocytosis. Meaning of multicentric reticulohistiocytosis medical term.
This is a systemic disease in which weight loss and episodes of. Background multicentric reticulohistiocytosis mrh is a rare. Multicentric reticulohistiocytosis presenting with. We report the case of a 54yearold female who was misdiagnosed as having rheumatoid arthritis and underwent numerous joint replacement surgeries for progressively destructive arthritis. Pathology multicentric reticulohistiocytosis is considered a form of granulomatous disease 10 characterized by infiltration of multinu. Multicentric reticulohistiocytosis mrh is a disease of unknown etiology characterized by diffuse skin lesions and destructive polyarthritis. We report a case of a 31 year old female who presented. Because up to 20% of cases may stem from an underlying malignancy, evaluation for a possible malignancy is essential. Multicentric reticulohistiocytosis mrh is a rare histiocytic proliferative disease in which joints, skin, mucous.
Multicentric reticulohistiocytosis is a rare condition of unknown aetiology characterised by the infiltration of histiocytes and multinucleated giant cells into a variety of tissues including the heart, lungs, skeletal muscle and the gastrointestinal system. Multicentric reticulohistiocytosis is a multisystem disease beginning usually around the age of 50 years, and is twice as common in women. To better understand the disorder and its other associations, we aimed to evaluate clinical correlates and outcomes of all patients with mrh seen at mayo clinic. The rarity of multicentric reticulohistiocytosis precludes the possibility of a doubleblind study.
Oct 10, 2019 multicentric reticulohistiocytosis mrh is a rare disease in which papulonodular skin lesions containing a proliferation of true histiocytes macrophages are associated with arthritis. Multicentric reticulohistiocytosis also known as lipoid dermatoarthritis is a rare multisystem disorder that affects skin, mucosa and joints and has distinct histopathological features. Skin lesions are pleomorphic, may be pruritic, and often occur around the joints. Only articles in english were chosen after checking for relevance. The patient was diagnosed with multicentric reticulohistiocytosis mrh and initially managed with 20 mg of methotrexate weekly and 20 to 40 mg of prednisone daily for 2 months.
Reticulohistiocytoma rh is a rare benign lesion of the soft tissue. Part 1 of 2 of multicentric reticulohistiocytosis mystery diagnosis uncut version. Multicentric reticulohistiocytosis is a rare form of nonlangerhans cell histiocytosis presenting with skin changes and erosive arthritis. Multicentric reticulohistiocytosis case series from a.
Pdf multicentric reticulohistiocytosis mrh is a rare nonlangerhans histiocytosis of unknown. This report describes the case of a patient with multicentric reticulohistiocytosis. First broadcast on the discovery health channel on august. About 25% of multicentric reticulohistiocytosis patients have an underlying malignancy, with reported cases involving most types of solid and haematological cancers. Multicentric reticulohistiocytosis associated with thymic. There is currently no consensus for the treatment of mrh. Characteristically, both entities consisted of oncocytic mononuclear histiocytes with granular eosinophilic cytoplasm similar to oncocytic thyroid cells and multinucleated histiocytes with a groundglass appearance, which appeared to be much larger 200 um and bizarre in cases of rh compared with cases of.
Looking for online definition of multicentric reticulohistiocytosis in the medical dictionary. Mar 31, 2016 reticulohistiocytoma rh is a rare benign lesion of the soft tissue. In the past solitary or multiple cutaneous reticulohistiocytoma without joint. On skin or joint biopsy the hallmark is the presence of multinucleated giant cells and histiocytes with a ground glass appearance of the cytoplasm secondary to lipid inclusions. Typical onset is during the fourth decade of life, but individuals of any age can be affected. This patient had been misdiagnosed with rheumatoid arthritis for many years. Free fulltext pdf articles from hundreds of disciplines, all in one place multicentric reticulohistiocytosis mrh. Treatment of multicentric reticulohistiocytosis with etanercept. Backgroundpurpose multicentric reticulohistiocytosis mrh is a rare systemic inflammatory disease with skin nodules and arthritis. Multicentric reticulohistiocytosis clinical presentation. Feb 25, 2016 multicentric reticulohistiocytosis is a rare disease affecting skin and joints primarily and rarely other organs.
This case report describes a newly diagnosed case of multicentric reticulohistiocytosis in a healthy 26yearold female originally from. Multicentric reticulohistiocytosis mrh, a rare histiocytic disease that can mimic other rheumatic conditions, may be associated with cancer and other autoimmune disorders. Multicentric reticulohistiocytosis is a rare disorder characterized by cutaneous papulonodular lesions and destructive arthritis. Part 2 of 2 of multicentric reticulohistiocytosis mystery diagnosis uncut version. Multicentric reticulohistiocytosis wikimili, the free. The most typical lesions are arranged in a string of pearls. Diagnosis is based on clinical findings and skin or synovial biopsy results.
A case of mrh arthropathy, in which the typical nodular skin manifestation of the disease was absent, is described in a patient with a past history of breast cancer and no evidence of. Mrh can coexist with various autoimmune disorders, tuberculosis, and malignancy. Most often, it is misdiagnosed as rheumatoid arthritis ra. Infiltration of histiocytes and multinucleated giant cells are typical histological findings and confirm the diagnosis. There is no specific treatment for multicentric reticulohistiocytosis. Multicentric reticulohistiocytosis is more common in women than men, with a ratio of 3. It usually occurs in the elderly and is very rare in children.
Treatment trial of multicentric reticulohistiocytosis with a combination of predonisolone, methotrexate and alendronate. Multicentric reticulohistiocytosis mrh is a rare histiocytic proliferative disorder involving skin, mucosa and joints. Multiple cutaneous papules and nodules arise most distinctly on the dorsum of the hands and the face but also less commonly involving the extensor extremities, scalp, and mucosa. Pdf multicentric reticulohistiocytosis is a rare systemic disease described as a lipoid dermatoarthritis. 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 organs. The rarity of multicentric reticulohistiocytosis precludes the possibility of a. A 45 year old patient presented with nonpruritic papules over the body and multiple joint pains.
Multicentric reticulohistiocytosis mrh is a rare multisystem macrophage disorder of unknown etiology characterized by papulonodular skin and mucosal lesions, rapidly progressive erosive symmetric polyarthritis, and inflammation of internal organs. The skin, mucosa, synovial, bone, and internal organs may be involved. Treatment of multicentric reticulohistiocytosis with. Multicentric reticulohistiocytosis mrh is a rare disease in which papulonodular skin lesions containing a proliferation of true histiocytes macrophages are associated with arthritis. Pdf multicentric reticulohistiocytosis researchgate. Histologically, cutaneous and synovial lesions reveal infiltration of multinucleated giant cells and histiocytes of monocytemacrophage origin 1, 2. The disease can involve the skin, the bones, the tendons, the muscles, the joints, and nearly any other organ e. Multicentric reticulohistiocytosis genetic and rare.
Multicentric reticulohistiocytosis is a disease that is characterized by the presence of papules and nodules and associated with arthritis mutilans. Reticulohistiocytoma genetic and rare diseases information. Mrh manifests as a symmetric erosive polyarthritis mimicking rheumatoid arthritis with the subsequent appearance of typical papulonodular skin lesions. A study of multicentric reticulohistiocytosis jama. The arthritis involves the interphalangeal joints and in 45% of cases causes severe joint destruction known as arthritis mutilans. Reticulohistiocytosis definition of reticulohistiocytosis. Multicentric reticulohistiocytosis mrh is a rare disorder of unknown etiology that primarily affects the skin, mucous membranes, and joints. Download multicentric reticulohistiocytosis download free online book chm pdf. The disease may rapidly progress towards mutilans arthritis in the majority of cases, but it generally. Reticulohistiocytoma and multicentric reticulohistiocytosis. Multicentric reticulohistiocytosis is a rare systemic inflammatory disease, featuring destructive polyarthritis and papulonodular skin lesions. Multicentric reticulohistiocytosis mrh is a rare histiocytic proliferative disease in which joints, skin, mucous membranes and internal organs. Successful treatment of multicentric reticulohistiocytosis.
Diagnosis is based on clinical findings and skin or. Pdf a case of multicentric reticulohistiocytosis researchgate. Multicentric reticulohistiocytosis mrh is a rare proliferative histiocytic disease of. Immunohistochemical analysis revealed prominent markers of monocytemacrophage origin, as well as the presence of tumor necrosis factor. A rare histiocytic proliferative disorder, first described in 1954 by goltz and laymon, in which joints, skin and mucous membranes are affected. We plan to continue tapering prednisone and leflunamide dosages if symptoms remain controlled. There is a recognized associated with various malignancies up to 25% of cases 9. Multicentric reticulohistiocytosis is a rare systemic granulomatous disease of an unknown cause, characterized by distinct histopathology. In approximately 1 in 4 cases, multicentric reticulohistiocytosis is associated with an internal malignancy 1. Skin lesions on the hands, especially at the base of the nails lesions may also be on the face, ears, arms, scalp or mucosal surfaces coral beads and vermicular erythematous lesions bordering nostrils are pathognomonic j eur acad dermatol venereol 2001. Cutaneous nodules and distinctive arthritis are the most prominent clinical features.
It can be a limited disease with a single yellowishred skin lump through to multiple skin lesions with internal organ involvement. Multicentric reticulohistiocytosis is a rare disease affecting skin and joints primarily and rarely other organs. Associated paraproteinemia has also been described. Sat0600 multicentric reticulohistiocytosis 19802017. 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. Lesions may occur in isolation reticulohistocytoma or in multiplicity multicentric reticulohistiocytosis. Multicentric reticulohistiocytosis mrh is an important entity to recognize given its association with underlying malignancy and irreversible destructive arthritis. In the literature, there are now 23 welldocumented examples. Multicentric reticulohistiocytosis genetic and rare diseases nih.
Distinctive features of multicentric reticulohistiocytosis mrh were present on the hand radiographs figure, including erosions across the endplate, apparent widening of the joint space, predominance of interphalangeal involvement, and relatively retained bone density. Her symptoms were consistent with an inflammatory polyarthropathy, with symmetrical pain and stiffness of both hands, wrists, elbows, shoulders and knees. Mrh is not life threatening and, after an average course of 78 years, the. Apr 01, 2016 multicentric reticulohistiocytosis mrh is a rare, multisystemic nonlangerhans cell histiocytosis characterized by skin and articular involvement, and rarely involves various other organs. The literature reports approximately 300 cases of multicentric reticulohistiocytosis mrh worldwide, mostly women of caucasian origin. This article cites 18 articles, 0 of which you can access for free at. The characteristic clinical features are papulonodular.
Multicentric reticulohistiocytosis mrh is a rare nonlangerhans histiocytosis of unknown etiology with a predilection for joint and skin. Whether after treatment for 6 to 18 months most patients may be able to discontinue the drug and remain in remission has yet to be shown. Multicentric reticulohistiocytosis mrh is a rare histiocytic proliferative disorder of uncertain etiology, characterized by mucocutaneous papulonodular lesions and progressive, symmetric erosive arthritis. Multicentric reticulohistiocytosis is a multisystem disease beginning usually around the age of 50 years, and is twice as common in women 718. Here, we report the case of a 60yearold woman found to have features of both mrh. Skin nonmelanocytic tumor multicentric reticulohistiocytosis. The articular destruction can lead to permanent joint deformities. Thorough history taking and physical examination with ageappropriate cancer screening is recommended.
Diagnosis is confirmed by histopathological examination. Multicentric reticulohistiocytosis mrh is a rare systemic disease, presenting with typical skin abnormalities and erosive polyarthritis, which is often associated with malignancy. We conclude that a response to an alkylating agent may be expected. 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 radiology reference. Multicentric reticulohistiocytosis mrh is a rare disease in which papulonodular skin lesions containing a proliferation of true histiocytes macrophages are associated with arthritis that primarily affects the interphalangeal joints. Reticulohistiocytosis is a rare form of histiocytosis that can affect the skin and other organs reticulohistiocytosis has a wide range of severity. Multicentric reticulohistiocytosis with arthralgia and red. Multicentric reticulohistiocytosis mrh is a rare histiocytic proliferative disorder of. Sir, we present the case of a 65yrold female smoker with a 2month history of peripheral arthralgia, referred to a tertiary rheumatology service as a possible case of ra. Multicentric reticulohistiocytosis pubmed central pmc. It belongs to a group of disorders called nonlangerhans cell histiocytosis and is a type of reticulohistiocytosis, all of which are types of histiocytosis. This is a systemic disease in which weight loss and episodes of pyrexia occur frequently.
A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for 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 disorder of unknown etiology, characterized by erosive polyarthritis and papulonodular. The skin, mucosa, synovial, bone, and internal organs may. Multicentric reticulohistiocytosis mrh, also known as lipoid dermatoarthritis, is a rare systemic disorder. Thalidomide, a rational agent for treatment of multicentric reticulohistiocytosis daniel j zinn1, olive eckstein 2, mary l olsen3, carl e allen and kenneth l mcclain2 1department of pediatrics, lehigh valley reilley childrens hospital, allentown, pennsylvania, usa 2texas childrens cancer and hematology centers, baylor college of medicine, houston, texas, usa. What is the treatment for multicentric reticulohistiocytosis. Multicentric reticulohistiocytosis mrh is a rare histiocytic proliferative disease in which joints, skin, mucous membranes and internal organs are affected. Other organs may be involved and 20% of patients have an internal malignancy. The patient has remained symptom free for 2 months. Multicentric reticulohistiocytosis is a multisystem disease beginning usually around the age of 50 years, and is twice as common in women718. Histiocytosis is a condition in which there is rapid production proliferation of histiocytes immune cells in the skin or soft tissues.
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