Complete remission of multicentric reticulohistiocytosis with combination therapy of steroid, cyclophosphamide, and low‐dose pulse methotrexate. Case report, review of the literature, and proposal for treatment
Abstract:We describe a patient with multicentric reticulohistiocytosis in whom complete remission was achieved by sequential combination therapy with steroid, methotrexate, and cyclophosphamide. We reviewed all cases of complete or near-complete remissions of multicentric reticulohistiocytosis reported in the English-language literature and propose a new drug regimen for this rare but potentially disabling disease.Multicentric reticulohistiocytosis is a rare systemic disorder of unknown etiology, characterized by tissu… Show more
“…MRH also occurs in association with autoimmune diseases in 5-20 % of patients including lupus, scleroderma, hypothyroidism, diabetes, primary biliary cirrhosis, idiopathic inflammatory myopathy, and celiac disease, and has also been reported in association with rheumatoid arthritis and Sjogren's syndrome [11]. Treatment is with steroids or chemotherapeutic agents [12]. Anti-tumor necrosis factor α agents and alendronate may also be efficacious [7].…”
“…MRH also occurs in association with autoimmune diseases in 5-20 % of patients including lupus, scleroderma, hypothyroidism, diabetes, primary biliary cirrhosis, idiopathic inflammatory myopathy, and celiac disease, and has also been reported in association with rheumatoid arthritis and Sjogren's syndrome [11]. Treatment is with steroids or chemotherapeutic agents [12]. Anti-tumor necrosis factor α agents and alendronate may also be efficacious [7].…”
“…3 As lesões cutâneas freqüentemente se resolvem antes do quadro articular e podem deixar seqüe-las permanentes. 8 Nos casos mais graves, a desfiguração da face é conhecida como "face leonina". As lesões de mucosa podem afetar de 34% a 50% dos pacientes e atingem principalmente língua, lábios, gengivas e septo nasal.…”
Section: Discussionunclassified
“…12 As neoplasias associadas são: carcinomas de mama, estômago, cérvix, pleura, pulmão, ovário, cólon, pân-creas e laringe, bem como o mesotelioma, melanoma maligno, sarcoma, linfoma e leucemia. 8 Não há relato de associação com adenoma hipofisário, que foi diagnosticado na paciente aqui apresentada.…”
A retículo-histiocitose multicêntrica é doença sistêmica rara e de etiologia desconhecida. Caracterizase por poliartrite simétrica, que pode evoluir para artrite mutilante, e por lesões papulonodulares na pele e nas mucosas. O diagnóstico definitivo é histopatológico. Em aproximadamente um terço dos casos de retículo-histiocitose multicêntrica em adultos, observa-se associação com malignidade. Ainda não foi estabelecida uma terapia-padrão. Descreve-se o caso de mulher de 46 anos com quadro clínico característico de retículo-histiocitose multicêntrica. Realizou-se tratamento inovador à base de infliximabe, obtendo-se ótimos resultados.
“…All patients had biopsies consistent with MRH. Using these methods, 27 cases of MRH with dermatomyositis-like features were identified (table 1) [4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27]. …”
Multicentric reticulohistiocytosis (MRH) is a rare form of non-Langerhans histiocytosis that presents with erosive arthritis and skin nodules. Approximately 25% of patients with MRH have an associated malignancy. Dermatomyositis is an inflammatory autoimmune condition that has also been associated with malignancy. To date, 7 cases of MRH have been reported to present with cutaneous features of dermatomyositis. We describe an eighth patient with MRH who presented with dermatomyositis-like features (V-neck erythema, shawl sign, Gottron’s papules and periungual erythema), who developed metastatic breast cancer 1 year after diagnosis. We hypothesized that clinical overlap between MRH and dermatomyositis was not as uncommon as review of the literature suggested. Careful review of the physical exam findings and photographs of the 234 papers reporting MRH revealed 27 cases of MRH with dermatomyositis-like features. Of these 27 cases, 7 (26%) were associated with a malignancy. Skin biopsies of the cutaneous features mimicking dermatomyositis revealed pathologic features of MRH. This is a descriptive analysis of published case reports. Based on a review of published case reports, MRH presenting with dermatomyositis-like features is likely fairly common. Histological examination of skin biopsies allows for disease differentiation. Differentiating MRH from dermatomyositis is important for management decisions and comorbidity screening.
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