Multicentric reticulohistiocytosis with prominent skin lesions and arthritis: A case report

Abstract

BACKGROUND Multicentric reticulohistiocytosis (MRH) is a rare non-Langerhans histiocytosis of unknown etiology characterized by papulonodular skin lesions and progressive, erosive arthritis. To date, there have been approximately 300 cases of MRH reported worldwide. The majority of patients are Caucasian from western countries, and Asian patients are rare. Here, we report a case of MRH in a Chinese patient. CASE SUMMARY A 38-year-old male was admitted to the hospital with a rash that had persisted for over 2 years and bilateral knee pain for over 1 year. The patient’s symptoms had previously been misdiagnosed as eczema when there were only skin symptoms and was finally diagnosed as MRH after a skin biopsy of the left upper back. The patient was treated with glucocorticoids combined with an immunosuppressive regimen. While the skin lesions on both arms, abdomen, and back subsided, the skin lesions on the rest of the body did not increase. The interphalangeal joints of both thumbs and bilateral knee joints remained swollen and painful. CONCLUSION The case will help clinicians better identify and treat this disease in the absence of epidemiological studies or randomized controlled data.

Multicentric Reticulohistiocytosis Presenting As Acute Pericarditis With Moderate-Sized Pericardial Effusion: A Case Report and Review of Multicentric Reticulohistiocytosis Treatment

“…Skin nodules of MRH have a characteristic coral bead appearance and are more often observed around the ungual region and may also be observed on the mucosa, face, arms, and torso. Facial lesions were described as having a lion-like appearance in the literature [ 8 ]. Systemic MRH may present as pleural and pericardial effusion (Figures 5 , 6 ), congestive heart failure, urogenital lesions, and mesenteric lymphadenopathy [ 5 ].…”

“…Histologically, multinucleated giant cells and histiocytes are seen with eosinophilic ground glass cytoplasm. These cells stain positive for periodic acid-Schiff (PAS), CD163, HAM56, and CD68 and negative for Langerhans markers such as S-100, CD1a, CD19, and CD20 [ 7 , 8 ]. Recent studies have demonstrated positive Mac387, vimentin, CD43, CD45, and lysozyme in a few patients [ 8 ].…”

“…These cells stain positive for periodic acid-Schiff (PAS), CD163, HAM56, and CD68 and negative for Langerhans markers such as S-100, CD1a, CD19, and CD20 [ 7 , 8 ]. Recent studies have demonstrated positive Mac387, vimentin, CD43, CD45, and lysozyme in a few patients [ 8 ]. Imaging of MRH shows a characteristic absence of periarticular osteoporosis and periosteal osteoblastic activity, unlike inflammatory and juvenile idiopathic arthritis [ 4 ].…”

“…In addition, patients with solid or hematologic malignancies can present with MRH as the paraneoplastic syndrome. Such presentation is observed in 25-33% of MRH patients, and the symptoms usually abate with the management of the malignancies [7][8][9]. Due to the common features between MRH and other systemic autoimmune conditions, MRH is more prone to misdiagnosis.…”