Multicentric reticulohistiocytosis (MRH)/systemic lupus erythematosus (SLE) overlap syndrome is an uncommon disease in the clinic and is diagnosed through characteristic clinical manifestations, histopathology, and immunopathology. Here, we report the case of a 30-year-old woman with SLE who developed MRH.

A 30-year-old woman with a history of polyarthritis for the past 12 years had multiple skin nodules on her body for 10 years, including the sacrococcygeal area, dorsum of the hands, interphalangeal joint of the feet and sternoclavicular joint. The histopathology of a biopsy of the distal interphalangeal joint of the hands revealed granulomatous inflammation, fibrous hyperplasia with ground-glass degeneration, inflammatory cell exudation and focal necrosis. The immunohistochemical stains showed positive staining for CD68 and negative staining for S100 and acid-fast staining. The patient was diagnosed with SLE with MRH. Her symptoms were improved after a combined treatment of prednisone, hydroxychloroquine and cyclophosphamide.

MRH/SLE overlap syndrome is difficult to diagnose and treat. Cyclophosphamide may be an alternative choice for the treatment of MRH.

• Multicentric reticulohistiocytosis
• Systemic lupus erythematosus
• Cyclophosphamide
• Systemic disorder
• Case report

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.

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.

The case will help clinicians better identify and treat this disease in the absence of epidemiological studies or randomized controlled data.

• Multicentric reticulohistiocytosis
• Papulonodular skin lesions
• Arthritis
• CD68
• Case report

• Systemic lupus erythematosus
• antiphospholipid syndrome
• histiocytosis
• indeterminate cell histocytosis

Multicentric reticulohistiocytosis is a rare disease affecting skin and joints primarily and rarely other organs. We present a case report of this disease and an extensive review of the literature. We reviewed the data between 1991 and 2014 and extracted 52 individual cases. Only articles in English were chosen after checking for relevance. The articles were studies and data was extracted into excel spread sheets and later used to compute such variables like frequency, mean and percentage of distribution of various clinical manifestations. The treatments used in these articles were critically analyzed and graded for their relative efficacy for skin and joint manifestations. The grades were 0 = worse, 1 = no benefit/condition remained same, 2 = improvement without resolution, and 3 = resolution. This article also reports the demographic, clinical, laboratory and pathological data from the reviewed articles. Authors attempted to discuss the findings of this review in depth to help manage this condition and proposed a treatment algorithm to help clinicians approach this rare and challenging disease.

• Arthritis
• Autoimmune disease
• Immunosuppressive medications
• Skin disease

• Anti-Inflammatory Agents/therapeutic use
• Cyclophosphamide/therapeutic use
• Fatal Outcome
• Female
• Heart Diseases/physiopathology
• Histiocytosis, Non-Langerhans-Cell/diagnosis
• Histiocytosis, Non-Langerhans-Cell/drug therapy
• Histiocytosis, Non-Langerhans-Cell/physiopathology
• Humans
• Immunosuppressive Agents/therapeutic use
• Methotrexate/therapeutic use
• Methylprednisolone/therapeutic use
• Tumor Necrosis Factor-alpha/antagonists & inhibitors

• Adenocarcinoma/complications
• Aged
• Celiac Disease/complications
• Celiac Disease/diagnosis
• Celiac Disease/therapy
• Female
• Histiocytosis, Non-Langerhans-Cell/complications
• Histiocytosis, Non-Langerhans-Cell/diagnosis
• Histiocytosis, Non-Langerhans-Cell/therapy
• Humans
• Ovarian Neoplasms/complications
• Paraneoplastic Syndromes/complications
• Paraneoplastic Syndromes/diagnosis
• Paraneoplastic Syndromes/therapy
• Treatment Outcome

• Adult
• Diagnosis, Differential
• Female
• Histiocytosis, Non-Langerhans-Cell/diagnosis
• Histiocytosis, Non-Langerhans-Cell/drug therapy
• Histiocytosis, Non-Langerhans-Cell/pathology
• Humans
• Immunosuppressive Agents/therapeutic use
• Methotrexate/therapeutic use
• Skin Diseases/diagnosis
• Skin Diseases/drug therapy
• Skin Diseases/pathology

• Fenofibrate/adverse effects
• Fenofibrate/therapeutic use
• Histiocytosis, Non-Langerhans-Cell/complications
• Humans
• Hyperlipidemias/complications
• Hyperlipidemias/drug therapy
• Hypolipidemic Agents/adverse effects
• Hypolipidemic Agents/therapeutic use
• Lipid Metabolism
• Musculoskeletal Diseases/etiology

• Adrenal Cortex Hormones/therapeutic use
• Adult
• Antirheumatic Agents/therapeutic use
• Arthritis/drug therapy
• Arthritis/etiology
• Biopsy
• Drug Therapy, Combination
• Female
• Heart Failure/drug therapy
• Heart Failure/etiology
• Histiocytosis, Non-Langerhans-Cell/complications
• Histiocytosis, Non-Langerhans-Cell/drug therapy
• Histiocytosis, Non-Langerhans-Cell/pathology
• Humans
• Hydroxychloroquine/therapeutic use
• Skin Diseases/drug therapy
• Skin Diseases/etiology
• Skin Diseases/pathology
• Treatment Outcome