Multicentric reticulohistiocytosis in a Malaysian Chinese lady: a case report and review of literature .

Department of Dermatology, Sarawak General Hospital, Sarawak, Malaysia.
Dermatology online journal 02/2009; 15(1):2.
Source: PubMed

ABSTRACT A 36-year-old Malaysian woman with erosive arthritis presented with multiple asymptomatic skin colored papulonodules on the hands, elbows, knees, ears, and feet; many macules, papules, and plaques were photodistributed. Progressive, erosive arthritis began about 18 months after the onset of skin involvement. Initially the patient was diagnosed to have dermatomyositis due to her skin findings, but later proved to have multicentric reticulohistiocytosis based on the typical histopathological features of histiocytic multinucleated giant cells with eosinophilic ground glass cytoplasm on skin biopsy. Workup for associated diseases including malignancies was negative. Her skin lesions and joint pain responded to treatment with methotrexate and prednisolone.

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    ABSTRACT: Multicentric reticulohistiocytosis (MRH) is a rare systemic inflammatory granulomatous disease that primarily manifests clinically with severe erosive arthritis and widespread papulonodular skin lesions but can involve multiple other organ systems. Despite the fact that this condition can become aggressive, debilitating as well as deforming with significant detrimental consequences, the etiology of this disease remains poorly understood. Moreover, the fact that MRH is such an uncommon disease has created an obstacle in the path of adequate clinical trials that are needed for better understanding of this phenomenon and for the development of treatment options for this patient population. In this review, we will attempt to discuss the epidemiology, pathophysiology, clinical features, associated conditions, differential diagnoses, diagnostic workup, and available treatments of MRH with the hope of creating a better understanding of this very challenging yet elusive disease process.
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    ABSTRACT: Multicentric reticulohistiocytosis (MRH) is a rare systemic and devastating disease with main involvement of skin and joints, frequently related with malignancies. Macrophages and TNFα are the main targets of multiple treatment options with variable results. Although MRH has the tendency of self-resolve in an average of 8 years, treatment must be started early to avoid sequelae, mainly in joints with severe deformities and daily life activity impairment. We describe a 50-year-old man with skin and joint involvement, with a non-previously described muscle affectation, that failed to different drugs and achieved skin improvement with infliximab. We review for the first time in medical literature the available therapeutic options of MRH and its outcomes and propose possible future targets.
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    ABSTRACT: The literature reports approximately 300 cases of multicentric reticulohistiocytosis (MRH) worldwide, mostly women of Caucasian origin. MRH manifests as a symmetric erosive polyarthritis mimicking rheumatoid arthritis with the subsequent appearance of typical papulonodular skin lesions. The disease may rapidly progress towards mutilans arthritis in the majority of cases, but it generally remits spontaneously in 10 years. The only diagnostic hallmark for MRH is the observation at histology of numerous histiocytes and multinucleated giant cells and ground glass eosinophilic cytoplasm. Despite its rarity, clinical and pathogenetic challenging features characterize the condition. First, the differential diagnosis with other forms of arthritis and the frequent coexistence of neoplasms or autoimmune diseases warrant a careful evaluation of suspected cases. Second, data from isolated MRH cases are consistent in supporting a role for monocyte/macrophage cells and for an enhanced osteoclastic activity in the affected tissues. Third, beside anti-inflammatory treatments, promising reports suggest that biologics targeting TNF-alpha and bisphosphonates may prove beneficial in MRH. Based on these observations, we are convinced that our understanding of this rare condition may prove beneficial in mechanistic advancement for other more prevalent inflammatory conditions such as arthritidies and other bone diseases.
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