Multicentric reticulohistiocytosis presenting with a rash and arthralgia

Abstract

A case of multicentric reticulohistiocytosis in a 53-year-old woman with a history of generalized cutaneous eruptions followed by arthralgia is presented. A thorough malignancy screen was performed with negative results. Treatment was commenced with oral prednisone 20 mg daily, which rapidly resulted in marked improvement of joint symptoms and resolution of self-perceived functional disability. As there was little improvement of her rash, oral azathioprine was added at 100 mg daily. Both drugs were well-tolerated and the patient was discharged from hospital on these medications. Two months later the azathioprine was reduced to 50 mg per day orally, following a small rise in hepatic transaminases. Within 4 months there had been dramatic improvement in the rash and cutaneous nodules with complete remission of the arthralgia and pruritus. During this time the oral prednisone had gradually been decreased to 10 mg daily. To date only the periungual nodules persist.

Multicentric reticulohistiocytosis (MRH): case report with review of literature between 1991 and 2014 with in depth analysis of various treatment regimens and outcomes

“…Consistent findings included nodular infiltrate of plump histiocytes with abundant finely granular eosinophilic cytoplasm and multinucleated giant cells, and usually PAS positivity, diastase-resistant. Other finding noted cells with CD68 immunoreactivity, which failed to stain with S100 or CD1a (Hiramanek et al 2002 ). One case mentioned that there was villous hypertrophic synovitis.…”

“…These articles were then individually screened for inclusion criteria including a diagnosis of MRH, written in the English language, and discussed treatment regimen and outcome. We reviewed the treatment options available (Islam et al 2013 ; Zelger et al 1996 ; Weber and Freudenthal 1937 ; Goltz and Laymon 1954 ; Barrow and Holubar 1969 ; Lesher and Allen 1984 ; Tajirian et al 2006 ; Luz et al 2001 ; Trotta et al 2004 ; Havill et al 1999 ; Lonsdale-Eccles et al 2009 ; Muñoz-Santos et al 2007 ; Goto et al 2003 ; Bennàssar et al 2011 ; Iwata et al 2012 ; Hiramanek et al 2002 ; Sakamoto et al 2002 ; Cox et al 2001 ; Flaming and Weigand 1993 ; Olson et al 2015 ; Aouba et al 2015 ; Eagle et al 1995 ; Han et al 2012 ; Teo and Goh 2009 ; Kishikawa et al 2007 ; Valencia et al 1998 ; Shiokawa et al 1991 ; Moreau et al 1992 ; Lambert and Nuki 1992 ; Qureshi et al 1993 ; Gibson et al 1995 ; Franck et al 1995 ; Liang and Granston 1996 ; Kocanaogullari et al 1996 ; Gorman et al 2000 ; Morris-Jones et al 2000 ; Hsu et al 2001 ; Saito et al 2001 ; Santilli et al 2002 ; Blanco et al 2002 ; Outland et al 2002 ; Hsiung et al 2003 ; Matejicka et al 2003 ; Liu and Fang 2004 ; Kovach et al 2004 ; Shannon et al 2005 ; Mavragani et al 2005 ; Malik et al 2005 ; Gajic-Veljic et al 2006 ; Adamopoulos et al 2006 ; Chen et al 2006 ; Chauhan et al 2007 ; de Zwart-Storm et al 2007 ; Codriansky et al 2008 ; Satoh et al 2008...…”

Multicentric Reticulohistiocytosis with Dermatomyositis-Like Features: A More Common Disease Presentation than Previously Thought

“…All patients had biopsies consistent with MRH. Using these methods, 27 cases of MRH with dermatomyositis-like features were identified (table 1) [4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27]. …”

A case of multicentric reticulohistiocytosis responsive to azathioprine in a patient with no underlying malignancy

“…[10][11][12][13] Our case suggests a role for azathioprine in the treatment of the cutaneous and arthritic manifestations of MRH. Further support for this is provided by the disease recurring upon cessation of azathioprine and resolution with its re-introduction.…”