MULTICENTRIC RETICULOHISTIOCYTOSIS

By Gary M. White, MD


Multicentric reticulohistiocytosis (MRH) Multicentric reticulohistiocytosis (MRH) is a rare, non-Langerhans histiocytosis of unknown etiology with a predilection for joints and skin. Systemic involvement including fever, weight loss, fatigue, muscle and bone involvement, lymphadenopathy, and cardiopulmonary complications occur rarely. MRH can be fatal, especially from cardiopulmonary involvement. A significant association with internal malignancy (e.g., lung, stomach, breast, colon, ovary, sarcoma, lymphoma) has been found.

Clinical

Pink, red, or purple dome-shaped papules on the fingers and the periungual area are characteristic. Multiple papules may occur elsewhere and rarely a photodistribution may be seen. Approximately half of patients have mucosal involvement. There is a slight female predominance and a progressive and destructive arthritis may be associated.

Treatment

Systemic steroids and NSAIDs may be given initially. Hydroxychloroquine, leflunomide, MTX, mycophenolate mofetil, cyclophosphamide, cyclosporine, and azathioprine have also been administered with some success. Etanercept, adalimumab, and infliximab have all been used with good results. Another promising option is the use of bisphosphonates, which act on the osteoclastic activity associated with MRH. Spontaneous resolution may occur.

References

Dermatology Online Journal 15(1)

Coral Bead Sign. Indian J Dermatol 2012;57:302-3

Multiple nodules on the elbows. Indian J Dermatol 2012;57:302-3

Destructive arthritis in MRH. JAAD February 2007 Volume 56, Issue 2, Pages 302–316

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