Hand surgery for Multicentric Reticulohistiocytosis: A new avenue of treatment and review of the literature.

INTRODUCTION

Multicentric Reticulohistiocytosis (MRH) is a rare non-Langerhans cell histiocytosis characterised by destructive polyarthritis and violaceous skin papules.

PRESENTATION OF CASE: In 2010, a 70-year-old woman with Palindromic Rheumatism was diagnosed with MRH. Within a few months, she developed ankylosis of the small joints of both hands which resulted in severe fixed flexion deformities of the fingers and thumbs. The joint disease failed to respond to medical therapies and the palmar skin of her left hand was becoming increasingly macerated. Therefore, she elected to undergo arthrodesis of the metacarpophalangeal joints to allow hand hygiene.

DISCUSSION: To-date, this is the first report of a surgical intervention for this rare condition and represents a novel avenue of potential therapy. Medical therapies for MRH are usually ineffective in preventing the debilitating small joint disease which often develops and there is on-going research into newer agents and alternative surgical techniques.

CONCLUSION

Once medical therapies are exhausted, clinicians should consider the input of Hand Surgeons in managing the inevitable and mutilating joint disease of this rare condition.