Subjective and objective improvement occurred after the patient received a course of nitrogen mustard and acth followed by chlorambucil. It usually begins during fourth decade of life with isolated polyarthritis 50%, cutaneous lesions 25% or both concurrently 25%. What is the treatment for multicentric reticulohistiocytosis. Tumor necrosis factor antagonists in the treatment of multicentric. Multicentric reticulohistiocytosis is a multi system disorder which mainly involves the skin, joints and mucous membrane. Multicentric reticulohistiocytosis is a rare systemic disease with proliferation of histiocytes which leads to destructive polyarthritis and skin lesions. 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. Skin involvement generally establishes with translucent reddishbrown to fleshcolored papulonodules varying from 12 mm to 1 cm in diameter or larger. Multicentric reticulohistiocytosis rao ag, lakshmi ts, vani v. Authoritative facts about the skin from dermnet new zealand. Cutaneous nodules and distinctive arthritis, are the most prominent clinical features. If you have problems viewing pdf files, download the latest version of adobe. A rare histiocytic proliferative disorder, first described in 1954 by goltz and laymon, in which joints, skin and mucous membranes are affected.
Multicentric reticulohistiocytosis an overview sciencedirect topics. Multicentric reticulohistiocytosis mrh is a rare disease in which papulonodular skin lesions containing a proliferation of true histiocytes macrophages are associated with arthritis. Diagnosis is based on clinical findings and skin or. Introduction multicentric reticulohistiocytosis mrh is a rare disease with frequent involvement of bones, tendons, muscles and joints. The most typical lesions are arranged in a string of pearls. Multicentric reticulohistiocytosis mrh is a rare and debilitating. Cutaneous nodules and distinctive arthritis are the most prominent clinical features. Mod rheumatol downloaded from by catholic university of. It is a systemic condition but the cause is not known. Reticulohistiocytosis is a rare histiocytic proliferative disorder one form of which, multicentric reticulohistiocytosis, may. In the past solitary or multiple cutaneous reticulohistiocytoma without joint. A case report article pdf available january 2010 with 460 reads. Pdf multicentric reticulohistiocytosis with s100 protein. Multicentric reticulohistiocytosis is a rare form of nonlangerhans cell histiocytosis presenting with skin changes and erosive arthritis.
Multicentric reticulohistiocytosis is a disease that is characterized by the presence of papules and nodules and associated with arthritis mutilans. Multicentric reticulohistiocytosis mrh is a rare disease characterized by the presence of multiple. Multicentric reticulohistiocytosis with extramammillary. Skin, mucosa, synovia, bone and internal organs may be involved. Backgroundmulticentric reticulohistiocytosis mrh is a rare disease of uncertain etiology that most commonly presents as a papulonodular cutaneous eruption. An otherwise healthy 46yearold man presented with a 1year history of an illdefined acral eruption that had evolved over several weeks into reddishbrown papules and nodules, together with a progressive inflammatory polyarthritis. By continuing to use our website, you are agreeing to our use of cookies. Pdf multicentric reticulohistiocytosis mrh is a disease of unknown etiology that affects primarily women in the fourth decade of life. Presentation with the cutaneous features of dermatomyositis, rheumatology, volume 33, i we use cookies to enhance your experience on our website. If you do not see its contents the file may be temporarily unavailable at the journal website or you do not have a pdf plugin installed and enabled in your browser.
There is a recognized associated with various malignancies up to 25% of cases 9. Multicentric reticulohistiocytosis genetic and rare diseases nih. Reticulohistiocytoma rh is a rare benign lesion of the soft tissue. Multicentric reticulohistiocytosis rao ag, lakshmi ts. Histiocytosis is a condition in which there is rapid production proliferation of histiocytes immune cells in the skin or soft tissues. The arthritis involves the interphalangeal joints and in 45% of cases causes severe joint destruction known as arthritis mutilans. Derhami3 1 department of rheumatology, ghaem hospital, school of medicine, mashhad university of.
Presentation mode open print download current view. The patient was diagnosed with multicentric reticulohistiocytosis mrh and initially managed with 20 mg of methotrexate weekly and 20 to 40 mg of prednisone. For language access assistance, contact the ncats public information officer. Hand surgery for multicentric reticulohistiocytosis. 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 is a multisystem disease beginning usually around the age of 50 years, and is twice as common in women. Females are affected more than men, with the ratio of woman to man being 3. Unexpectedly, a biopsy of the forehead skin revealed numerous multinucleated giant cells. Multicentric reticulohistiocytosis mrh, also known as lipoid dermatoarthritis, is a rare systemic disorder. Pdf multicentric reticulohistiocytosis is a rare systemic disease described as a lipoid.
Multicentric reticulohistocytosis is characterized by cutaneous or mucosal papular lesions often associated with severe polyarthritis and arthralgias. Skin lesions ranging from several millimeters to several. Apr 01, 2000 read multicentric reticulohistiocytosis. Pdf multicentric reticulohistiocytosis researchgate. Although mrh is rare, it should be remembered that mrh can mimic dm. Here we describe a patient with mrh and extramammillary pagets disease empd, a diagnosis that has not previously been described in the literature. Multicentric reticulohistiocytosis primary care dermatology.
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 with dermatomyositis. 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. Mrh support the multicentric reticulohistiocytosis. Article pdf available in indian journal of rheumatology 3 september 2018 with 169 reads. Download fulltext pdf download fulltext pdf a case of multicentric reticulohistiocytosis article pdf available in modern rheumatology 111. Due to its unknown etiology, the treatment of mrh varies with. 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. Pathology multicentric reticulohistiocytosis is considered a form of granulomatous disease 10 characterized by infiltration of multinu. The skin, mucosa, synovial, bone and internal organs may be involved.
Apr 23, 2015 the literature reports approximately 300 cases of multicentric reticulohistiocytosis mrh worldwide, mostly women of caucasian origin. Skin lesions are pleomorphic, may be pruritic, and often occur around the joints. Multicentric reticulohistiocytosis is a rare disease affecting skin and joints primarily and rarely other organs. Abstract multicentric reticulohistiocytosis mrh is a rare, multisystemic nonlangerhans cell histiocytosis characterized by skin and articular involvement, and rarely involves various other organs.
Alternatively, you can download the file locally and open with any standalone pdf reader. Multicentric reticulohistiocytosis pubmed central pmc. Diagnosis is based on clinical findings and skin or synovial biopsy results. The mcp joints and carpal bones are involved less frequently. Multicentric reticulohistiocytosis mr, mrh, previously also called lipoid dermatoarthritis is a rare disease, primarily affecting patients in their adulthood characterized by the rapid appearance of multiple papules and nodules arising in conjunction with severe arthritis. Associated paraproteinemia has also been described. Treatment of multicentric reticulohistiocytosis with. The cause of multicentric reticulohistiocytosis is unknown but in about 2030% of cases, the disease is associated with an underlying malignancy.
Histopathology helps to differentiate multicentric reticulohistiocytosis from eruptive xanthogranuloma and juvenile xanthogranulomas. The multicentric reticulohistiocytosis support site. A case of multicentric reticulohistiocytosis is reported. Granuloma annulare mimicking multicentric reticulohistiocytosis. Multicentric reticulohistiocytosis kaul a, tolat sn.
The rarity of multicentric reticulohistiocytosis precludes the possibility of a doubleblind study. Multicentric reticulohistiocytosis is a rare disease of unknown etiology. Multicentric reticulohistiocytosis radiology reference. Multicentric reticulohistiocytosis is a rare systemic disease with proliferation of histiocytes which leads to destructive polyarthritis and skin lesions distribution. 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 is not life threatening and, after an average course of 78 years, the. There is currently no consensus for the treatment of mrh.
Pdf a case of multicentric reticulohistiocytosis researchgate. It belongs to a group of disorders called nonlangerhans cell histiocytosis and is a type of reticulohistiocytosis, all of which are types of histiocytosis. 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 case of photodistributed multicentric reticulohistiocytosis. Treatment of multicentric reticulohistiocytosis with etanercept. We describe a patient with granuloma annulare ga who presented with firm periungual papules mimicking coral beads, a characteristic sign of multicentric reticulohistiocytosis mrh. Lesions may occur in isolation reticulohistocytoma or in multiplicity multicentric reticulohistiocytosis.
Oct 05, 2010 part 2 of 2 of multicentric reticulohistiocytosis mystery diagnosis uncut version. Reticulohistiocytosis article about reticulohistiocytosis. Multicentric reticulohistiocytosis differential diagnoses. Mrh manifests as a symmetric erosive polyarthritis mimicking rheumatoid arthritis with the subsequent appearance of typical papulonodular skin lesions. What is the cause of multicentric reticulohistiocytosis. There is no specific treatment for multicentric reticulohistiocytosis. Multicentric reticulohistiocytosis also known as lipoid dermatoarthritis has a worldwide distribution with a female preponderance 6075%.
Sep 28, 2012 multicentric reticulohistiocytosis is a disease that is characterized by the presence of papules and nodules and associated with arthritis mutilans. The disease can involve the skin, the bones, the tendons, the muscles, the joints, and nearly any other organ e. First broadcast on the discovery health channel on august 16, 2010 this mystery diagnosis episode is about. Multicentric reticulohistiocytosis mrh is an uncommon systemic disease of unknown aetiology, which is characterized by the. 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. Multicentric reticulohistiocytosis is a rare systemic granulomatous disease of an unknown cause, characterized by distinct histopathology. The skin, mucosa, synovial, bone, and internal organs may. A collection of disease information resources and questions answered by our.
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. If there are multiple skin lesions but no arthritis then there is no association with cancer. Skin lesions can lead to significant deformity, and about half of affected patients develop a severe disabling arthri tis, with the remainder having arthritis of variable. Hydroxychloroquine was added to this regimen for 7 months with little benefit. If only the pancarpal destructive changes in the left wrist are. Download fulltext pdf download fulltext pdf multicentric reticulohistiocytosis with s100 protein positive staining. Controversy exists about whether multicentric reticulohistiocytosis is a true paraneoplastic disorder. If you have problems viewing pdf files, download the latest version of adobe reader. Multicentric reticulohistiocytosis is a multisystem disease beginning usually around the age of 50 years, and is twice as common in women 718. Pdf multicentric reticulohistiocytosis mrh is a rare. The literature reports approximately 300 cases of multicentric reticulohistiocytosis mrh worldwide, mostly women of caucasian origin. The rarity of multicentric reticulohistiocytosis precludes the possibility of.
Skin nonmelanocytic tumor multicentric reticulohistiocytosis. 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 reticulohistocytosis is characterized by cutaneous or mucosal papular lesions often. Feb 25, 2016 multicentric reticulohistiocytosis is a rare disease affecting skin and joints primarily and rarely other organs. 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. Backgroundpurpose multicentric reticulohistiocytosis mrh is a rare systemic inflammatory disease with skin nodules and arthritis. The pathogenesis of mrh is unknown but probably has an immunologic basis. Multicentric reticulohistiocytosis mr is a rare systemic granulomatous disease of unknown cause characterized by distinct histopathology. Multicentric reticulohistiocytosis case series from a. While the optimal treatment for multicentric reticulohistiocytosis associated pruritus is not currently known, you may find the following article to be a helpful resource.
Multicentric reticulohistiocytosis multicentric reticulohistiocytosis. Other organs may be involved and 20% of patients have an internal malignancy. We conclude that a response to an alkylating agent may be expected. Target areas of the hands primarily involve the interphalangeal joints, especially at the dips. First broadcast on the discovery health channel on august 16, 2010 this mystery diagnosis episode is. 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. Part 2 of 2 of multicentric reticulohistiocytosis mystery diagnosis uncut version. Papular xanthomas and erosive arthritis in a 3 year old. Diffuse optical spectroscopic imaging reveals distinct early breast tumor hemodynamic responses to metronomic and maximum tolerated dose regimens. The disease may rapidly progress towards mutilans arthritis in the majority of cases, but it generally. Oct 06, 20 multicentric reticulohistiocytosis mr, mrh, previously also called lipoid dermatoarthritis is a rare disease, primarily affecting patients in their adulthood characterized by the rapid appearance of multiple papules and nodules arising in conjunction with severe arthritis. Skin and joint symptoms dominate the clinical picture. Multicentric reticulohistiocytosis multicentric reticulohistiocytosis melton, john w irby, robert 19720301 00.
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