Reticulohistiocitosis multicéntrica: una gran simuladora dentro de la Reumatología / Multicentric reticulohistiocytosis: a great pretender within Rheumatology

La reticulohistiocitosis multicéntrica (RHM) es una histiocitosis de células no Langerhans, multisistémica, rara, caracterizada por afectación cutánea y articular, que poco frecuentemente afecta a otros órganos. El diagnóstico se basa en hallazgos clínicos y resultados de biopsias de piel o sinovial, sin describirse un parámetro de laboratorio específico. Realizamos una revisión de la literatura en relación a las características clínicas de esta entidad desde el punto de vista reumatológico.

Multicentric reticulohistiocytosis (MHR) is a rare, multisystem, non-Langerhans cell histiocytosis characterized by skin and joint involvement, and rarely involving other organs. Diagnosis is based on clinical findings and results of skin or synovial biopsies; a specific laboratory parameter has not been described. We conducted a review of the literature regarding the clinical characteristics of this entity from a rheumatological point of view.

Reticulohistiocitosis multicéntrica: una gran simuladora dentro de la Reumatología / Multicentric reticulohistiocytosis: a great pretender within Rheumatology

La reticulohistiocitosis multicéntrica (RHM) es una histiocitosis de células no Langerhans, multisistémica, rara, caracterizada por afectación cutánea y articular, que poco frecuentemente afecta a otros órganos. El diagnóstico se basa en hallazgos clínicos y resultados de biopsias de piel o sinovial, sin describirse un parámetro de laboratorio específico. Realizamos una revisión de la literatura en relación a las características clínicas de esta entidad desde el punto de vista reumatológico.

Multicentric reticulohistiocytosis (MHR) is a rare, multisystem, non-Langerhans cell histiocytosis characterized by skin and joint involvement, and rarely involving other organs. Diagnosis is based on clinical findings and results of skin or synovial biopsies; a specific laboratory parameter has not been described. We conducted a review of the literature regarding the clinical characteristics of this entity from a rheumatological point of view.

Multicentric reticulohistiocytosis: A case report / 北京大学学报(医学版)

A 65-year-old woman developed erythema, papules and nodules over the body. Some nodules of her auricles and hands like string beads. Besides, she suffered from symmetrical swelling and pain of multiple joints, morning stiffness with deformity of joints; She had elevated erythrocyte sedimentation rate and C reactive protein levels; Her rheumatoid factor and antinuclear antibody were positive; Joints destruction was found with X-ray imaging; Skin pathology showed Dermal infiltrate of abundant histiocytes, part of them with a ground-glass appearance; A CD68 immunohistochemical stain was positive and the cells were negative for S100, CD1a. These findings were diagnostic evidences of multicentric reticulohistiocytosis (MRH). The patient received high-dose of glucocorticoids combinated with immunosuppressive agents, and achieved a satisfactory effect. MRH was a rare multisystem disease characterized by papulonodular mucocutaneous and destructive arthritis, and its pathogeny was not yet completely understood. The typical lesions of MRH were hard papules or nodules that usually occured on the hands, face and arms. Classic coral bead appearance from periungual cutaneous nodules that were characteristic of MRH. MRH was an inflammatory joint disease, affecting almost all the appendicular joints and characterized by joint multiple, symmetrical, destructive, progressive disability. Joints destruction of the distal interphalangeal joints was a unique feature of MRH. In addition to skin and joints, it could also involve other systems. There were no diagnostic laboratory markers for MRH. Laboratory examinations had often been found to be non-specific. Imageological examination mainly showed bone and joint destruction. Skin biopsy was the best test to diagnose MRH, the typical histopathological findings included an infiltrate with histiocytes and multinucleated giant cells with a ground-glass appearing in eosinophilic cytoplasm, and the immunohistochemical stain was positive for CD68. The diagnosis was typically made based on the clinical presentation, supportive radiographic findings and skin biopsy. MRH was easily possible to mistake for other more common autoimmune conditions, such as rheumatoid arthritis, psoriatic arthritis, osteoarthritis, and dermatomyositis, but the distinctive clinical, radiographic, and histologic features could aid in differentiating these diseases. MRH could mimic other rheumatic diseases, besides, it could also coexist with cancer or other autoimmune disorders. There was no standardized treatment for MRH. However, Nonsteroidal anti-inflammatory drugs, glucocorticoid, Immunosuppressant, biologic medications, and bisphosphonates had been used with varying degrees of curative effect. Treatment with glucocorticoid combined with immunosuppressants were effective for rash and arthritis, early use of them should be strongly considered, and refractory cases could be treated with biological agents. By reporting a MRH case and reviewing literature, this paper aims to help the clinicians improve the understanding of this rare disease, and suggests that when one diagnosis cannot explain the whole picture of the disease, and further evidence should be sought to confirm the diagnosis.

Reticulohistiocitosis multicéntrica de inicio súbito por quemadura solar / Multicentric reticulohistiocytosis, sudden onset after sunburn

Las reticulohistiocitosis son un grupo de enfermedades muy poco frecuentes que se caracterizan por la acumulación de histiocitos en piel y articulaciones principalmente. Se suelen asociar a condiciones y patologías muy variadas, predominantemente neoplasias. Presentamos un caso de inicio súbito luego de quemadura solar (AU)

Reticulohisticytosis is a group of diseases characterized by the accumulation of cells of histiocytic lineage primarily in the skin and joints. They have been associated with many conditions and diseases, mainly malignant neoplasms. A case of multicentric reticulohitiocytosis of sudden onset after sunburn is reported (AU)

Reticulohistiocitosis multicéntrica en paciente con artritis reumatoidea / Multicentric reticulohistiocytosis in a patient with rheumatoid arthritis

La reticulohistiocitosis multicéntrica (RHM) es una patología sistémica, de baja prevalencia, considerada dentro delgrupo de las histiocitosis no Langerhans. Clínicamente se caracteriza por el compromiso de la piel y las articulaciones.Se describe el caso de un paciente masculino con antecedente de diagnóstico de artritis reumatoidea (AR) de 10 años de evolución, que en 2008 se presenta a la consulta por un cuadro de poliartritis asociado a prurito cutáneo,hiperpigmentación facial y presencia de múltiples lesiones nodulares rojovioláceas en codos y manos. Mediante biopsia de las lesiones cutáneas se confirma el diagnóstico de RHM. Cabe destacar que se ha descripto hasta un 30% de asociación de la entidad con patologías neoplásicas; en nuestro paciente el tamizaje fue negativo. En cuanto al tratamiento no existen protocolos avalados científicamente dada la baja incidencia de la enfermedad, por lo que sigue siendo experimental. En nuestro paciente se realizó tratamiento exitoso con metotrexate.

Multicentric reticulohistiocytosis (MRH) is a systemic pathology of low prevalence within the group of histiocytosis no-Langerhans. It is clinically characterized by the involvement of skin and joints. The present article describes the case of a male subject with a diagnosis of rheumatoid arthritis of 10 years’ duration. In 2008 he was seen in our service with multiple nodular, red skin lesions in his hands and elbows. A biopsy of the cutaneous lesions confirmed the diagnosis of MRH. An association with neoplasic disease has been described in 30% of the cases; however, in our patient cancer screening was negative. With regard to the treatment, scientifically proven protocols do not exist. This is due to the low incidence of the disease. Our patient responded well to methotrexate.

A Case of Multicentric Reticulohistiocytosis Misdiagnosed as Rheumatoid Arthritis / 고신대학교의과대학학술지

Multicentric reticulohistiocytosis (MRH) is a rare disease characterized by nodular skin lesions and severe erosive polyarthritis which is associated with malignancy in some cases. The diagnosis is confirmed by the presence of oncocytic histiocytes and multinucleated giant cells on histopathology of the cutaneous nodules and the synovial membrane. It usually remits spontaneously after 5-8 years but it can provoke destructive arthritis. We report a case of a 49-year-old female who presented with numerous nodules on the both hands, face and abdomen and progressive destructive polyarthritis of 3 years duration and has been diagnosed with rheumatoid arthritis. The lesion showed large histiocytes with ground-glass eosinophilic cytoplasm, typical of MRH. Clinical manifestation and radiological pattern of MRH may be misdiagnosed as other disease like rheumatoid arthritis and psoriatic arthritis, but histopathologic findings of our case can differentiate MRH from any other conditions.

Paraneoplastic multicentric reticulohistiocytosis: A clinicopathologic challenge.

Multicentric Reticulohistiocytosis (MRH) is a rare, systemic non-Langerhans cell histiocytosis (non-LCH) with prominent joint and skin manifestations. It is mostly self limiting. However, 15-30% of the cases are associated with malignancy and carry a poor prognosis. We report the case of a 42-year-old man who presented with multiple reddish-brown papules that on biopsy showed aggregates of oncocytic histiocytes with several multinucleate giant cells. Immunostains were positive for CD 68, CD 45 and were negative for S-100, CD1a. An impression of multicentric reticulohistiocytosis (MRH) was made, with the recommendation to screen for malignancy. Electron microscopy of the skin lesions showed features consistent with non-Langerhans cell histiocytosis. The patient was later diagnosed with acute myeloid leukemia at a follow-up visit several months later. Thus, it appears prudent to screen and follow-up adults with MRH, to identify an underlying malignant condition.

Multicentric reticulohistiocytosis.

A 50-year-old male presented with fever, joint pain and skin lesions since eight months. Examination showed multiple papules and nodules with periarticular predisposition. Swelling of knees and elbows with flexion deformity of distal interphalangeal joints was present. Investigations revealed anemia and raised ESR. Histopathology was pathognomonic of multicentric reticulohistiocytosis. Patient was treated with bisphosphonates along with systemic steroids and methotrexate to which he responded well.

A Case of Multicentric Reticulohistiocytosis Mistaken as Rheumatoid Arthritis / 대한류마티스학회지

Multicentric reticulohistiocytosis (MRH) is a rare disease affecting joints, skin and internal organs. Yet the cause is still unknown. In most cases, it can be misdiagnosed as rheumatoid arthritis or psoriatic arthritis before typical change in the skin emerges, and precise diagnosis is essential because it may become severe enough to transform and destroy the joints. Therefore, skin or synovial biopsy can confirm the existence of this disease. This particular patient is a 39-year-old male who had been treated for rheumatoid arthritis on a wrist and hand and transferred to this hospital when arthritic pain continued with erythematous papules and nodules on the hands, the outer rims of the ears, hands and elbows. The X-ray examination of both hands revealed multiple marginal erosion of proximal and distal interphalangeal joints and destruction of subchondral bones. periarticular osteoporosis and joint space enlargement is combined, but no new bones were seen to be formed. Biopsy of erythematous nodules on the dorsum of hands showed that an infiltrate histiocytes and multi-nuclear giant cells were aligned irregularly, and immunological chemical staining showed potential for being positive to PAS and CD68. To control pain and regulate activity of the disease, tracing observation and treatment were started from the outside with NSAID, hydroxycholoquine, MTX and prednisolone. A month of treatment did not improve arthritis and skin problems, and increased dose of MTX, prednisolone did improve arthritis a little but not skin problems. Treatment with infliximab (3mg/kg), a anti-tumor necrosis factor, is in progress, showing improvement in both conditions.

Reticulohistiocitosis multicéntrica / Multicentric reticulohistiocytosis

La reticulohistiocitosis multicéntrica (RHM) es una enfermedad de etiología desconocida que afecta principalmente a mujeres en la cuarta década de la vida. La afección articular se caracteriza por la presencia de sinovitis simétrica de miembros superiores especialmente en manos y afección de piel presentando lesiones de diferente morfología más comúnmente nódulos y pápulas localizadas principalmente en cara y miembros superiores. Puede existir además compromiso de órganos internos como pulmón, corazón, tracto gastrointestinal y glándulas salivales. Esta patología se ha relacionado con la aparición de neoplasias malignas en diferentes órganos como mama, ovario, cérvix y linfomas llegando a ser considerada por diferentes autores como una manifestación de un síndrome paraneoplásico. Los exámenes de laboratorio en estos pacientes evidencian alteraciones en el cuadro hemático, perfil hepático así como marcadores de autoinmunidad. El diagnóstico confirmatorio de la enfermedad se determina por el estudio histológico donde se demuestran células histiocíticas (histiocitos mononucleares) gigantes multinucleadas (con diámetro de 50-100 µm) con citoplasma eosinofílico y gránulos finos con apariencia de vidrio esmerilado. Múltiples medicamentos han sido usados como ciclofosfamida, metotrexate, azatioprina, clorambucil y regímenes conjugados. Se informa el caso de una paciente de sexo femenino de 51 años, raza blanca, que consulta por cuadro clínico de cuatro meses de evolución caracterizado por astenia, adinamia, fiebre subjetiva nocturna, pérdida de peso, poliartritis inflamatoria de carpos, codos y rodillas, así como la aparición de lesiones cutáneas en forma de pápulas dolorosas de color violáceo que se localizaban en cara, codos, manos y flancos.

Multicentric reticulohistiocytosis (MRH) is a disease of unknown etiology that affects primarily women in the fourth decade of life. Articular involvement is characterized by the presence of symmetric synovitis of the upper extremities, particularly of the hands, and compromise of the skin with lesions of different morphology, more commonly nodules and papules localized mainly on the face and upper extremities. There can also be involvement of internal organs such as lung, heart, gastrointestinal tract and salivary glands. This disease has been linked to malignant neoplasms of different organs like breast, ovary, uterine cervix and lymphomas, leading some authors to consider this disease a manifestation of a paraneoplastic syndrome. Laboratory testing in these patients show abnormalities of the complete blood count, liver function tests as well as markers of autoimmunity. Histological studies confirm the diagnosis by demonstrating giant multinucleated histiocytic cells (mononuclear histiocytes-diammeter of 50 to 100 µm) with an eosinophilic cytoplasm and fine granules that give the cells a ground glass appearance. Multiple medications have been used to treat this disease including cyclophosphamide, azathioprine, methotrexate, chlorambucil and combined regimens. We document the case of a 51 year old caucasian female patient that was evaluated because of a 4 month history of subjective fever, weakness, fatigue, weight loss, inflammatory bilateral polyarthritis of the wrists, elbows and knees as well as the appearance of painful violaceous papules on the surface of the face, elbows, hands and flanks.

A Case of Muticentric Reticulohistiocytosis Misdiagnosed As Rheumatoid Arthritis / 대한피부과학회지

Multicentric reticulohistiocytosis (MRH) is a rare multisystemic granulomatous disease of unknown cause, characterized by severe debilitating polyarthritis and papular to nodular mucocutaneous lesions. This disease usually manifests as a progressive, destructive polyarthritis with mucocutaneous eruptions consisting of erythematous to brownish-colored papules and nodules. MRH is important, not only because of its disfiguring and disabling manifestations and systemic involvements, but also due to its association with internal malignancy. The histological features of skin and synovial lesions are infiltration of multinucleated giant cells, and histiocytes which contain abundant eosinophilic cytoplasm and have a ground glass appearance. Without the accompanying skin nodules, the patients can be misdiagnosed as having rheumatoid arthritis or psoriatic arthritis, and this inaccurate diagnosis will affect treatment. We report a case of MRH which was misdiagnosed as rheumatoid arthritis.

A Case of Multicentric Reticulohistiocytosis / 대한피부과학회지

Multicentric reticulohistiocytosis is a rare systemic disorder of unknown ethiology, characterized by development of cutaneous papulonodular lesions, and destructive arthritis. Most patients with this condition have been Caucasian. There have only been a few case reports in Korea. However there appears no racial difference in prevalence. We herein report a case of multicentric reticulohistiocytosis in a 47-year-old female, who presented with papulonodules on both hands and had symptoms of a rapidly progressive arthritis. During a trial with methotrexate, cyclosporin, prednisone and NSAID, the patient dropped out of follow-up treatment at the 2nd month, showing no improvement of the condition.

A Case of Multicentric Reticulohistiocytosis / 대한피부과학회지

Multicentric reticulohistiocytosis(MR) is a rare systemic disease of unknown etiology, characterized by destructive polyarthritis and mucocutaneous eruptions in various locations. In most cases rapidly progressive destructive polyarthritis is the presenting symptom, followed by typical skin eruptions. Skin lesions are characterized clinically by erythematous to brownish nodular lesions on the face, periungual areas and the trunk. The biopsy demonstrating the characteristic granulomatous infiltration of multinucleated giant cells and histiocytes with a ground-glass appearance is diagnostic. We report a case of multicentric reticulohistiocytosis in a 57-year-old female patient, who had skin lesions with polyarthritis and cardiac problem. To our knowledge, this is the first case report in Korea.