Multicentric reticulohistiocytosis
Synopsis

In the skin, MRH appears as papules and/or nodules that most frequently occur on the acral areas of the body, especially the head, hands, and juxta-articular regions of the extremities. Mucous membrane involvement occurs in up to half of patients. The typical mucosal eruption is characterized by multiple erythematous papules and nodules that affect the oral and nasopharyngeal mucosa. Mucocutaneous disease typically lasts 5-10 years with subsequent regression.
Joint disease is a prominent feature of MRH that can produce significant morbidity. The typical presentation is a symmetric erosive polyarthritis that may evolve into arthritis mutilans. Progressive destruction of joints occurs in most individuals for about 6-8 years with stabilization thereafter. The distal interphalangeal (DIP) joints are affected most commonly (about 75% of cases), although any joint can be involved.
There are numerous reported associations including malignancy (up to 25%), tuberculosis (12%-50%), autoimmune disorders, and systemic vasculitis. Carcinomas of the lung, breast, stomach, cervix, ovary, and colon are the most frequently associated malignancies, although others have been reported.
Codes
E78.81 – Lipoid dermatoarthritis
SNOMEDCT:
84241008 – Multicentric reticulohistiocytosis
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Last Updated:11/18/2018