Multicentric reticulohistiocytosis (MRH) is categorized as a rare non-Langerhans cell histiocytosis most commonly seen in women in the fourth to fifth decade of life. This systemic inflammatory condition affects multiple organ systems and can result in severe joint destruction which can progress to arthritis mutilans. To date, various underlying malignancies have been discovered in patients with MRH including breast, gastric, thymic, hepatic, and melanoma. There has been 1 case of underlying renal cell carcinoma reported in a patient diagnosed with MRH. Additionally, there is no consistently recognized treatment for MRH described in the literature. The rarity of the disease contributes to the difficulty in defining a standardized treatment. We present the case of a patient with extensive joint and skin involvement who was successfully treated with infliximab and methotrexate, experienced clinical improvement, and was later diagnosed with clear cell renal cell carcinoma. The synergistic effects of infliximab and methotrexate, in combination with the low side-effect profile, appear to be promising in the setting of MRH and in our patient resulted in the resolution of symptoms and cutaneous manifestations. We suggest this regimen as an effective combination therapy. We emphasize thorough and continuous screening for underlying malignancy associated with MRH, despite clinical improvement or negative malignancy work-up upon initial diagnosis.
BACKGROUND
Henoch-Schönlein purpura is a leukocytoclastic small vessel vasculitis with both cutaneous and systemic manifestations. Although Henoch-Schönlein purpura is most common in childhood it may manifest in adults with more extensive systemic manifestations. Additionally, the disease is present worldwide with differences in epidemiologic and systemic characteristics.
OBJECTIVE
The main objective of this study was to examine the features of extracutaneous manifestations in adults with Henoch-Schönlein purpura as distinguished by geographic region worldwide.
METHODS
A search of Pubmed, Embase, Cochrane Library, and Web of Science was performed for articles published January 1, 1970 through December 1, 2019. Keywords included “Henoch-Schönlein purpura" OR "henoch schonlein purpura + adult”, “IgA vasculitis + adult”, “HSP + adult”, and “IgAV”. A total of 995 publications were found. 42 studies, comprised of 3388 patients were included with the vast majority of reported cases being from continents Asia, Europe, and the Americas.
RESULTS
Of adults with Henoch-Schönlein purpura, European patients were more likely to be male (P<0.001), have gastrointestinal involvement (P<0.001), and have musculoskeletal involvement (P<0.001). Patients from America were the least likely to have genitourinary involvement (P<0.001).
CONCLUSIONS
The distribution and extracutaneous manifestations of Henoch- Schönlein purpura vary between geographical boundaries. Among adult patients with Henoch-Schönlein purpura, Europeans affected were more likely to be males, and were more likely to experience gastrointestinal and musculoskeletal involvement. Asian patients were more likely to experience genitourinary involvement than those in America. Prospective studies utilizing standardized reporting measures are needed to confirm the relationships identified in this study.
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