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