Multicentric Reticulohistiocytosis Associated with an Early Form of Systemic Lupus Erythematosus: A Case Report of a Rare Disease, with Mini Review of the Literature

Abstract

Multicentric reticulohistiocytosis (MRH) is the most frequently described form of reticulohistiocytosis (RH), and it is classified as a class IIb non-Langerhans cell histiocytosis. It has been designated as multicentric, being characterized by multisystemic involvement. In fact, although mainly involving the skin, along with the joints, it is a systemic inflammatory condition potentially involving every internal organ. As MRH-related skin findings can mimic rheumatoid nodules or Gottron papules, the histopathology of the cutaneous lesions is often necessary for the correct diagnosis. Approximately one-third of MRH patients have confirmed concomitant autoimmune disorders. A wide variety of autoimmune disorders associated with the disease have been reported in the literature, suggesting immune dysfunction as a factor in the pathophysiology of MRH. A case of MRH associated with autoimmune manifestation is reported in the context of a mini review of the literature, with a focus on clinical presentation, treatments, and treatment outcomes. Moreover, eight cases of MRH associated with autoimmune diseases are briefly discussed.

Systemic lupus erythematosus with multicentric reticulohistiocytosis: A case report

“…Sarilumab and tocilizumab have been proposed as specific immunomodulators to treat MRH refractory patients based on the overexpression of IL-6 in the giant multinucleated cells of the MRH inflammatory infiltrate, which play a vital role in improving joints and dermatological manifestations. Meanwhile, a recent case report mentions that using anakinra (IL-1 inhibition) allowed for control of the disease[ 1 ]. In our patient, we initially treated with prednisone, hydroxychloroquine, leflunomide and MTX, but the patient failed to respond to MTX, with new nodules detected on her hands and feet after 6 mo of treatment.…”

“…It has been reported that CTX was of significant benefit in 20% of cases, with complete arthritis resolution, and in 27% of cases of skin lesions in MRH. Additionally, partial arthritis and skin disease control was seen in 40 and 45% of cases, respectively[ 1 ]. However, the precise mechanism of CTX was not documented, but it can be postulated.…”

“…Multicentric reticulohistiocytosis (MRH) is a rare, multisystem non-Langerhans cell disorder of unknown etiology that is characterized by erosive polyarthritis and skin papulonodular lesions. MRH is associated with underlying malignancy and autoimmune disease, including breast cancer, ovarian adenocarcinoma, melanoma, rheumatoid arthritis (RA), Sjogren’s syndrome, systemic lupus erythematosus (SLE) and multiple sclerosis[ 1 , 2 ]. Many cases are misdiagnosed as RA due to the manifestation of arthritis mutilans, including symmetric articular involvement, marginal erosion in bone and severe arthritis destruction.…”