The cause has not been fully elucidated, and no consistently effective treatment has been identified. Histopathologically, the lesion was composed of oncocytic macrophages and multinucleate giant cells with abundant, eosinophilic and granular cytoplasm with. The literature reports approximately 300 cases of multicentric reticulohistiocytosis mrh worldwide, mostly women of caucasian origin. There is no specific treatment for multicentric reticulohistiocytosis. Histopathologically, the lesion was composed of oncocytic macrophages and multinucleate giant. Multicentric reticulohistiocytosis presenting with. Mrh can coexist with various autoimmune disorders, tuberculosis, and malignancy.
Multicentric reticulohistiocytosis mrh is a rare histiocytic. About 25% of multicentric reticulohistiocytosis patients have an underlying malignancy, with reported cases involving most types of solid and haematological cancers. 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. To our knowledge, this is the first case report of tenosynovial fluid and tenosynovium histopathology demon. Multicentric reticulohistiocytosis is a multisystem disease beginning usually around the age of 50 years, and is twice as common in women 718.
There is no known etiology, and it often remits spontaneously within 510 years, but can result in severe. A retrospective cohort of mrh patients treated at our institution from 01. Khan departmentofinternalmedicine,saintjosephhospital,n. Mutilating arthropathy, multicentric reticulohistiocytosis, histiocytosis.
Multicentric reticulohistiocytosis genetic and rare. 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. Discussion multicentric reticulohistiocytosis mrh is a rare nonlangerhans histiocytosis which manifests mainly with cutaneous nodules, destructive arthritis and has distinct histopathological features. 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. Other organs may be involved and 20% of patients have an internal malignancy. Multicentric reticulohistiocytosis is a rare syndrome of adulthood. Multicentric reticulohistiocytosis mrh is a rare histiocytic proliferative disorder of uncertain etiology, characterized by mucocutaneous papulonodular lesions and progressive, symmetric erosive arthritis. Nov 19, 2019 multicentric reticulohistiocytosis mrh is a rare disease with multisystem involvement. Backgroundpurpose multicentric reticulohistiocytosis mrh is a rare systemic inflammatory disease with skin nodules and arthritis. Case report multicentric reticulohistiocytosis presenting with papulonodular skin lesions and arthritis mutilans rayasaba,shawng. What is the treatment for multicentric reticulohistiocytosis. 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.
Cutaneous nodules and distinctive arthritis are the most prominent clinical features. Efficacy of vinblastine and prednisone in multicentric. 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. Cureus a rare case of multicentric reticulohistiocytosis. Most often, it is misdiagnosed as rheumatoid arthritis ra.
The skin, mucosa, synovial, bone, and internal organs may. Multicentric reticulohistiocytosis mrh, also known as lipoid dermatoarthritis, is a rare systemic disorder. Multicentric reticulohistiocytosis case series from a. 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. The patient was put on short course of systemic steroids after which the patient was lost for follow up. Multicentric reticulohistiocytosis annals of the rheumatic. Rapini, md m ulticentric reticulohistiocytosis mrh is a rare condition in which papular and nodu lar skin lesions containing characteristic bi zarre giant cells with groundglass cyto plasm are associated with a form of severe arthritis. The histopathologic differential diagnosis includes solitary reticulohistiocytoma and diffuse cutaneous histiocytosis. Swelling of knees and elbows with flexion deformity of distal interphalangeal joints was present. 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.
Multicentric reticulohistiocytosis pubmed central pmc. 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. Introduction multicentric reticulohistiocytosis mrh is a rare disease with frequent involvement of bones, tendons, muscles and joints. However, in 50 to 66% of patients, these diagnostic nodules follow the onset of arthritis by months to years. Multicentric reticulohistiocytosis successfully treated with.
Lesions may occur in isolation reticulohistocytoma or in multiplicity multicentric reticulohistiocytosis. In approximately 1 in 4 cases, multicentric reticulohistiocytosis is associated with an internal malignancy 1. There were 26 males and 18 females with a median age of 35 years range, 2. Histopathology of the skin shows dermal infi ltration of histiocytes and.
A retrospective cohort of mrh patients treated at our. Characteristic histopathology with welldefined dermal infiltrate consisting of multinucleated giant. In conclusion, our case is unique with tenosynovial fluid and tenosynovium histopathology showing histiocytes. The diagnosis of multicentric reticulohistiocytosis was made on the basis of typical histopathological features which showed multinucleated. Pathology multicentric reticulohistiocytosis is considered a form of granulomatous disease 10 characterized by infiltration of multinu. It is classified as a nonlangerhans cell histiocytosisclass iib. The clinicopathological and immunohistochemical features of four patients with systemic multicentric reticulohistiocytosis mr were compared with five cases of solitary and one case of multiple reticulohistiocytoma rh, which were confined to the skin only. First case report with tenosynovial fluid analysis and tenosynovial histology demonstrating the classic histologic findings. The most typical lesions are arranged in a string of pearls.
A rare histiocytic proliferative disorder, first described in 1954 by goltz and laymon, in which joints, skin and mucous membranes are affected. Pathology outlines multicentric reticulohistiocytosis. First case report with tenosynovial fluid analysis and tenosynovial histology demonstrating the classic histologic findings author megha sawhney, peter levitin. Pdf multicentric reticulohistiocytosis researchgate.
Characteristic histopathology with welldefined dermal infiltrate consisting of multinucleated giant cells and large macrophages having abundant. If only the pancarpal destructive changes in the left wrist are. Sep 28, 2012 multicentric reticulohistiocytosis is a disease that is characterized by the presence of papules and nodules and associated with arthritis mutilans. Multicentric reticulohistiocytosis our dermatology online journal. Herein, we describe a 48yearold smoker with severe panlobular emphysema occurring in association with multicentric reticulohistiocytosis, a papular, infiltrative cutaneous eruption that is a member of the proliferative histiocytic cd68 spectrum of diseases. Reticulohistiocytosis is a rare histiocytic proliferative disorder one form of which, multicentric reticulohistiocytosis, may mimic rheumatoid arthritis. Aggregations of histiocytes with ground glass cytoplasm. Multicentric reticulohistiocytosis mrh is a rare disease in which papulonodular skin lesions containing a proliferation of true histiocytes macrophages are associated with arthritis. Histopathology of skin biopsy showed thinned out epidermis overlying a lesion in dermis composed of oncocytic macrophages, few multinucleate giant cells with abundant. In the past solitary or multiple cutaneous reticulohistiocytoma without joint. Multicentric reticulohistiocytosis mrh is a rare systemic disease characterized by the combination of typical papular and nodular skin lesions and a severe and destructive polyarthritis, although virtually any organ system of the body can be involved.
Multicentric reticulohistiocytosis primary care dermatology. Multicentric reticulohistiocytosis mrh is a rare disease with multisystem involvement. The skin, mucosa, synovial, bone, and internal organs may be involved. All patients had a superficial, circumscribed, mildly elevated, solitary lesion size range. Megha sawhney,peter levitin, 2016 multicentric reticulohistiocytosis mrh. Multicentric reticulohistiocytosis mrh is a rare histiocytic proliferative disorder involving skin, mucosa and joints. Histopathology was pathognomonic of multicentric reticulohistiocytosis. S100 positive multicentric reticulohistiocytosis our. Multicentric reticulohistiocytosis mrh is a rare histiocytic proliferative disorder of unknown etiology. 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.
Multicentric reticulohistiocytosis mrh is a rare systemic disease that characteristically produces severe erosive arthritis of the distal and proximal interphalangeal joints along with cutaneous nodules over the upper extremities and face, but it can be found in other organs. Diagnosis is based on clinical findings and skin or. Diagnosis is based on clinical findings and skin or synovial biopsy results. The arthritis involves the interphalangeal joints and in 45% of cases causes severe joint destruction known as arthritis mutilans. Case report multicentric reticulohistiocytosis presenting. 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. Mrh manifests as a symmetric erosive polyarthritis mimicking rheumatoid arthritis with the subsequent appearance of typical papulonodular skin lesions. Diagnosis diagnosis is confirmed by histopathological examination. Multicentric reticulohistocytosis is characterized by cutaneous or mucosal papular lesions often associated with severe polyarthritis and arthralgias. 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. Multicentric reticulohistiocytosis oxford medicine.
Multicentric reticulohistiocytosis mr is a rare multisystem granulomatous disease manifesting cutaneous eruption, destructive arthritis, various systemic involvement, and associated malignancy. Multicentric reticulohistiocytosis mrh is a systemic disease characterized by papulonodular skin eruptions and progressive, deforming arthritis. Multicentric reticulohistiocytosis radiology reference. Hydroxychloroquine was added to this regimen for 7 months with little benefit. Examination showed multiple papules and nodules with periarticular predisposition. Earlyonset symptomatic emphysema has been described to occur in susceptible individuals under age 50. Reticulohistiocytoma solitary epithelioid histiocytoma. It usually occurs in the elderly and is very rare in children.
We aimed to examine the clinical correlates and outcomes of mrh seen at our center, and study the association with malignancy and autoimmunity. Multicentric reticulohistiocytosis is a disease that is characterized by the presence of papules and nodules and associated with arthritis mutilans. Associated paraproteinemia has also been described. Skin lesions are pleomorphic, may be pruritic, and often occur around the joints. Multicentric reticulohistiocytosis has not been shown to have a familial component. Multicentric reticulohistiocytosis is a rare systemic inflammatory disease, featuring destructive polyarthritis and papulonodular skin lesions. 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. A 45 year old patient presented with nonpruritic papules over the body and multiple joint pains. Multicentric reticulohistiocytosis is a rare disease affecting skin and joints primarily and rarely other organs. Apr 23, 2015 the literature reports approximately 300 cases of multicentric reticulohistiocytosis mrh worldwide, mostly women of caucasian origin. Treatment of multicentric reticulohistiocytosis with etanercept. Efficacy of vinblastine and prednisone in multicentric reticulohistiocytosis with onset in infancy vijay kumar jha, md, a ravindra kumar, md, b abhijeet kunwar, ms, c ankur singh, md, b mahendra kumar, md, a mohan kumar, md, a rajniti prasad, mdb departments of.
The mean age of people affected by multicentric reticulohistiocytosis is 50 years old 2. Multicentric reticulohistiocytosis is more common in women than men, with a ratio of 3. Feb 25, 2016 multicentric reticulohistiocytosis is a rare disease affecting skin and joints primarily and rarely other organs. Multicentric reticulohistiocytosis mrh is a rare systemic disease, presenting with typical skin abnormalities and erosive polyarthritis, which is often associated with malignancy. Pdf multicentric reticulohistiocytosis mrh is a rare nonlangerhans. Multicentric reticulohistiocytosis mrh is a rare, histiocytic disorder that primarily affects the skin and joints. Here, we report the case of a 60yearold woman found to have features of both mrh. Multicentric reticulohistiocytosis associated with thymic. The patient finally received a diagnosis of multicentric reticulohistiocytosis after histopathological examination of the patients left knee. The disease may rapidly progress towards mutilans arthritis in the majority of cases, but it generally. 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. It predominantly affects caucasian females in their fifth and sixth decade of life 2, 3. Multicentric reticulohistiocytosis responding to tumor.
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 an overview sciencedirect topics. Multicentric reticulohistiocytosis presenting with papulonodular. Multicentric reticulohistiocytosis clinical presentation. Pdf a case of multicentric reticulohistiocytosis researchgate. Diagnosis is confirmed by histopathological examination. Multicentric reticulohistiocytosis mrh is a rare, multisystemic nonlangerhans cell histiocytosis characterized by skin and articular involvement, and rarely involves various other organs. Reticulohistiocytoma and multicentric reticulohistiocytosis. 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. Multicentric reticulohistiocytosis is a rare systemic granulomatous disease of an unknown cause, characterized by distinct histopathology.