Piette, in dermatological signs of internal disease fourth edition, 2009 variants of langerhans cell disease. Backgroundpurpose multicentric reticulohistiocytosis mrh is a rare systemic inflammatory disease with skin nodules and arthritis. Multicentric reticulohistiocytosis mrh is a rare, multisystemic nonlangerhans cell histiocytosis characterized by skin and articular involvement, and rarely involves various other organs. Multicentric reticulohistiocytosis with extramammillary pagets. Skin lesions are pleomorphic, may be pruritic, and often occur around the joints. Multicentric reticulohistiocytosis clinics in dermatology. Histiocytosis represents a group of rare disorders characterized by nonneoplastic proliferation of histiocytes and solitary cutaneous reticulohistiocytosis scr is a form of nonlangerhans histiocytosis. First case report with tenosynovial fluid analysis and tenosynovial histology demonstrating the classic histologic findings, open journal of rheumatology and autoimmune diseases, 10. Multicentric reticulohistiocytosis with arthralgia and red. The disease can involve the skin, the bones, the tendons, the muscles, the joints, and nearly any other organ e. 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 rare multisystem disease, presenting with skin lesions and erosive polyarthritis, which is often associated with malignancy. Multicentric reticulohistiocytosis radiology reference. It belongs to a group of disorders called nonlangerhans cell histiocytosis and is a type of reticulohistiocytosis, all of which are types of histiocytosis. Luz fb, gasper nk, gasper ap, carneiro s, ramosesilva m. Efficacy of vinblastine and prednisone in multicentric. Thalidomide, a rational agent for treatment of multicentric. Multicentric reticulohistiocytosis is a rare systemic disease described as a lipoid dermatoarthritis. Multicentric reticulohistiocytosis mr is a rare disorder of skin with systemic granulomatous involvement of no known cause with distinct histopathology. On administration of systemic prednisolone and azathioprine the skin lesions resolved and the. Multicentric reticulohistiocytosis is more common in women than men, with a ratio of 3.
Histology of the both lesions disclosed an infiltrate composed primarily of histiocytic cells. Multicentric reticulohistiocytosis our dermatology online journal. Reticulohistiocytoma genetic and rare diseases information. Cutaneous reticulohistiocytosis is a rare form of nonlangerhans cell histiocytosis. Multicentric reticulohistiocytosis mrh is a rare systemic disease, presenting with typical skin abnormalities and erosive polyarthritis, which is often associated with malignancy. Congenital selfhealing reticulohistiocytosis hashimotopritzker disease is an uncommon but perhaps underdiagnosed pediatric syndrome.
Examination showed multiple papules and nodules with periarticular predisposition. 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. Oct 10, 2019 histopathology of multicentric reticulohistiocytosis mrh skin lesions. Reticulohistiocytosis is primarily a histopathological category, and. Multicentric reticulohistiocytosis longdom publishing sl. 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. A 86yearold woman presented with a 3month history of painless reddish nodules on fingers, nose and earlobes, together with polyarthralgia that had preexisted for 9 months before. Multicentric reticulohistiocytosis presenting with.
Multicentric reticulohistiocytosis is a very rare multisystem arthropathic form of reticulocytosis. Histopathology showed the characteristic histiocytic and multinucleated giant cell infiltrate with ground glass cytoplasm, and immunohistochemical analysis showed markers evocative of a monocytemacrophage origin of mrh. Histopathology was pathognomonic of multicentric reticulohistiocytosis. Mrh can coexist with various autoimmune disorders, tuberculosis, and malignancy. Scr consists of small papule, usually single, varying in color from yellow to brownishred, more frequent in young adults, and uncommon in childhood.
Skin nonmelanocytic tumor multicentric reticulohistiocytosis. Higher power demonstrating multinucleated giant cells with eosinophilic groundglass cytoplasm. Multicentric reticulohistiocytosis responding to tumor necrosis factor. Pdf s100 positive multicentric reticulohistiocytosis. Pathology outlines multicentric reticulohistiocytosis. Pdf multicentric reticulohistiocytosis a case with. In the past solitary or multiple cutaneous reticulohistiocytoma without joint. 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. The skin, mucosa, synovial, bone, and internal organs may. The diagnosis of multicentric reticulohistiocytosis was made on the basis of typical histopathological features which showed multinucleated. Characteristic involvement includes multiple skin nodules and progressive arthritis. The arthritis involves the interphalangeal joints and in 45% of cases causes severe joint destruction known as arthritis mutilans.
Multicentric reticulohistocytosis is characterized by cutaneous or mucosal papular lesions associated with severe polyarthritis and arthralgias. A retrospective cohort of mrh patients treated at our institution from 01. Reticulohistiocytoma and multicentric reticulohistiocytosis. Multicentric reticulohistiocytosis resembling dermatomyositis. Balachandran2 1 department of pathology, kasturba medical college, manipal, karnataka, india source of support. It is classified as a nonlangerhans cell histiocytosisclass iib. Multicentric reticulohistiocytosis shah sp, shah am. Apr 23, 2015 the literature reports approximately 300 cases of multicentric reticulohistiocytosis mrh worldwide, mostly women of caucasian origin. Abstract multicentric reticulohistiocytosis is a rare granulomatous disease of unknown etiology, characterized by cutaneous nodules and destructive arthritis. Multicentric reticulohistiocytosis kaul a, tolat sn. 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. It usually begins during fourth decade of life with isolated polyarthritis 50%, cutaneous lesions 25% or both concurrently 25%. 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 case series from a.
Case report a 70 year old noninsulin dependent diabetic white woman was referred to us for a progressive deformity. Metrics links files go to a case of multicentric reticulohistiocytosis misdiagnosed as rheumatoid arthritis. Multicentric reticulohistiocytosis is a rare disease affecting skin and joints. 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. Lesson of the month multicentric reticulohistiocytosis. Mucosal involvement occurs in approximately 50% of patients and includes the presence of nodules in the oral, nasal, and pharyngeal mucosae, as well as eye structures. Multicentric reticulohistiocytosis with elevated cytokine serum levels. Pdf multicentric reticulohistiocytosis is a rare systemic disease described as a. Multicentric reticulohistiocytosis mrh is a rare histiocytic proliferative disease in which joints, skin, mucous membranes and internal organs are affected. Multicentric reticulohistiocytosis mrh is a rare systemic disease that characteristically produces.
Mar 31, 2016 reticulohistiocytoma rh is a rare benign lesion of the soft tissue. Reticulohistiocytoma and multicentric reticulohistiocytosis are designations for uncommon, incompletely characterized histiocytic proliferations of the skin or soft tissues. Multicentric reticulohistiocytosis is the more frequently described form of rh. Histiocytic proliferative syndromes such as multicentric reticulohistiocytosis must be considered in the differential diagnosis of emphysema occurring in young adult smokers. Multicentric reticulohistiocytosis mrh is a rare disease affecting joints, skin and internal organs. Cutaneous nodules and distinctive arthritis are the most prominent clinical features. Multiple cutaneous reticulohistiocytomas successfully treated. 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.
The patient finally received a diagnosis of multicentric reticulohistiocytosis after histopathological examination of the. Case presentation this case report describes a newly diagnosed case of multicentric reticulohistiocytosis in a healthy 26year. It has been described to involve the skin, mucosa, joints and internal organs but all the involvement may not be found in a single patient 1. Reticulohistiocytosis an overview sciencedirect topics. Skin lesions can cause significant deformity, and approximately half of affected patients develop a severe disabling arthritis. Very little osteoporosis was evident despite the marked destruction. In this study, we analyzed a uniform group of 44 lesions composed of epithelioid histiocytes, comprising a subset of lesions o. Princess alexandra hospital, department of pathology, brisbane, australia jason wu, mbbs, princess alexandra hospital, department of dermatology.
Discussion multicentric reticulohistiocytosis mrh is a rare nonlangerhans histiocytosis which manifests mainly with cutaneous nodules, destructive arthritis and has distinct histopathological features. Here, we report the case of a 60yearold woman found to have features of both mrh. Multicentric reticulohistiocytosis is a disease that is characterized by the presence of papules and nodules and associated with arthritis mutilans. Indian journal of dermatology, venereology and leprology. Reticulohistiocytoses are a type of non langerhans cell histiocytosis. F 1 synonyms for multicentric reticulohistiocytosis. T the most prominent clinical features are distinctive cutaneous nodules and destructive arthritis.
Multicentric reticulohistiocytosis in a malaysian chinese lady. Diagnosis is based on histopathologic findings of histiocytic. Histology of reticulohistiocytoma in reticulohistiocytoma, sections show a diffuse infiltration of numerous large, mononucleated or multinucleated histiocytes in the dermis figures. Reticulohistiocytoma solitary epithelioid histiocytoma. Multicentric reticulohistiocytosis mrh is a rare disease with multisystem involvement. It has been described to involve the skin, mucosa, joints and internal organs but all the. 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. Multicentric reticulohistiocytosis mrh is a rare nonlangerhans histiocytosis which manifests mainly with cutaneous nodules, destructive arthritis and has distinct histopathological features. Dec 14, 2004 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. Pdf multicentric reticulohistiocytosis researchgate. Multicentric reticulohistiocytosis mrh is a systemic disease characterized by papulonodular skin eruptions and progressive, deforming arthritis.
In most cases, it can be misdiagnosed as rheumatoid arthritis. Multicentric reticulohistiocytosis mrh is a rare histiocytic proliferative disorder of uncertain etiology, characterized by mucocutaneous papulonodular lesions and progressive, symmetric erosive arthritis. Aggregations of histiocytes with ground glass cytoplasm. Diagnosis is based on clinical findings and skin or synovial biopsy results. Mrh manifests as a symmetric erosive polyarthritis mimicking rheumatoid arthritis with the subsequent appearance of typical papulonodular skin lesions. Treatment of multicentric reticulohistiocytosis with. Multicentric reticulohistiocytosis mrh is an idiopathic multisystemic. Infiltration of histiocytes and multinucleated giant cells are typical histological findings and confirm the diagnosis.
Multicentric reticulohistiocytosis mrh is a rare histiocytic proliferative disorder of. It predominantly affects caucasian females in their fifth and sixth decade of life 2, 3. 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. 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. The histopathologic differential diagnosis includes solitary reticulohistiocytoma and diffuse cutaneous histiocytosis. Multicentric reticulohistiocytosis is a rare multisystem disorder of unknown etiology that is. Histopathologic and immunophenotypic distinct entities. 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. It usually occurs in the elderly and is very rare in children. Multicentric reticulohistiocytosis mrh is a rare histiocytic proliferative abstract disorder of uncertain etiology, characterized by mucocutaneous papulonodular lesions and progressive, symmetric erosive arthritis. Cureus a rare case of multicentric reticulohistiocytosis. Multicentric reticulohistiocytosis mrh is a nonlangerhans cell histiocytosis that can mimic other rheumatic conditions and cause destructive arthritis. Multicentric reticulohistiocytosis mrh is a rare histiocytic proliferative disorder involving skin, mucosa and joints.
The mean age of people affected by multicentric reticulohistiocytosis is 47 years age range 874 years 5 in approximately 1 in 4 cases, multicentric reticulohistiocytosis is associated with an internal malignancy 1. The disease may rapidly progress towards mutilans arthritis in the majority of cases, but it generally. A 2 year untreated natural course of multicentric reticulohistiocytosis, in which the typical nodular skin manifestation of the disease and polyarticular arthritis was present, is. Multicentric reticulohistiocytosis responding to tumor. Megha sawhney, peter levitin, multicentric reticulohistiocytosis mrh.
Systemic glucocorticoids, conventional dmards and antitnf biologics are the most frequently used treatments in mrh. Multicentric reticulohistiocytosis genetic and rare. The diagnosis of mrh is established on the basis of histopathologic features of. Multicentric reticulohistiocytosis is characterised by skin and mucosal lesions, and arthritis. The radiographic features of the case were most marked in the interphalangeal joints of the hands. Swelling of knees and elbows with flexion deformity of distal interphalangeal joints was present. Multicentric reticulohistiocytosis is a rare syndrome of adulthood. Multicentric reticulohistiocytosis of lower in the vitiligo patients, there was no statistically sig the skin and synovia. Erythematous to brown papules overlying the right dorsal hand and wrist and erythematous to violaceous patches over the right dorsal hand and fingers. Multicentric reticulohistiocytosis has not been shown to have a familial component. Predominant involvement of the distal interphalangeal joint occurs in the hands. The patient was put on short course of systemic steroids after which the patient was lost for follow up.
While it has been noted worldwide, a higher incidence has been. Targetable driver mutations in multicentric reticulohistiocytosis. Background multicentric reticulohistiocytosis is a rare form of nonlangerhans cell histiocytosis presenting with skin changes and erosive arthritis. Involvement of skin, mucosa, and internal organs has been described. Zelger b1, cerio r, soyer hp, misch k, orchard g, wilsonjones e. Multicentric reticulohistiocytosis clinical presentation. Multicentric reticulohistiocytosis mrh is a very rare systemic disease with variable phenotypic presentation and a high rate of misdiagnosis. Most often, it is misdiagnosed as rheumatoid arthritis ra. However, pathology of the xanthomatous lesions was more suggestive of papular xanthoma, a disease that is not associated with arthritis. Multicentric reticulohistiocytosis also known as lipoid dermatoarthritis has a worldwide distribution with a female preponderance 6075%.
1229 1380 1773 235 313 1028 1612 324 122 1339 578 793 1100 1225 91 1352 580 1324 1767 1494 249 1402 1628