Multicentric reticulohistiocytosis

Case contributed by Matt Skalski
Diagnosis almost certain

Presentation

Several month history of non-tender skin papules and tongue lesions, now with progressing joint pain in the extremities.

Patient Data

Age: 45 years
Gender: Female

Left hand: There are extensive erosive changes of the distal articular surfaces distal radius and ulna, as well as of the carpals and carpometacarpal joints. The radiocarpal, intercarpal and carpometacarpal joint spaces demonstrate severe uniform loss of joint space.

Right hand: There are mild erosive changes involving the scaphoid in the region of the scaphoid tubercle, as well as marginally throughout the interphalangeal joints; however, the associated joint spaces are maintained.  

Annotated image

The marginal erosions in the right hand are indicated with blue arrows. 

There are multiple marginal erosions randomly distributed throughout the metatarsal heads bilaterally, as well as at the lateral aspect of the right interphalangeal joint, all of which have maintained joint spaces at the involved sites without angular deformity. 

On the left the navicular-cuneiform joints appear ankylosed with degnerative and inflammatory changes throughout Chopart's joint. 

Annotated image

Some of the periarticular erosions are indicated with blue arrows. 

Case Discussion

The combination of radiographic findings and the patient's presentation in this case suggest multicentric reticulohistiocytosis (MCRH) as a diagnosis, but do not rule out other differential considerations such as rheumatoid arthritis and other inflammatory arthritides or gout. If only the pan-carpal destructive changes in the left wrist are identified, septic arthritis would be an important consideration. 

This case was biopsy proven MCRH. 

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