Histiocytoses – Multicentric Reticulohistiocytosis

April 29th, 2012

Multicentric Reticulohistiocytosis is a rare Non-X Histiocytosis that I occasionally encounter in both my The Woodlands dermatology and Conroe dermatology offices.  Multicentric Reticulocytosis usually appears after age 50 and is most common in women.  Patients present with reddish brown papules typically on the fingers around the nailfold and the clinical appearance has been termed “coral beads.”  The skin can also be involved on the extremities (especially over joints), face, ears, neck and trunk.  Multicentric Reticulohistiocytosis is a systemic disease and osteoarthritis is a hallmark finding.  This arthritis can be very destructive and disabling.  When the arthritis affects the fingers the term “opera glass shortening” has been used to describe the telescopic shortening of the phalange bones.  Other organ systems involved are: heart, lungs, liver, lymph nodes, liver, muscles and gastrointestinal tract.  Approximately, 1/4 of all patients with Multicentric Reticulohistiocytosis also have an internal malignancy.  Thus, the use of immunosuppresant therapy to treat this inflammatory disease must be weighed against lowering the body’s defenses against fighting malignancy.