PMC4766148_01
The patient's right dorsal hand with visible swelling of all the distal, proximal interphalangeal and metacarpophalangeal joints
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Fig1
On clinical exam, the patient had red-brown macules and papules symmetrically distributed on the dorsum of hands, elbows, periocularly, and periauricularly (Figs. 1, 2).
The right dorsal hand with multiple red-brown macules and papules
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Fig2
On clinical exam, the patient had red-brown macules and papules symmetrically distributed on the dorsum of hands, elbows, periocularly, and periauricularly (Figs. 1, 2).
Skin biopsy from a red-brown papule on the right dorsal hand (Fig. 2) demonstrated numerous multinucleated histiocytes (arrows) infiltrating between the collagen bundles in the superficial dermis. There is a Grenz zone separating the epidermis from the dermal tumor. Additionally, there are an increased number of blood vessels amongst the histiocytes, as well as scattered lymphocytes
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Fig3
A skin biopsy demonstrated numerous multinucleated histiocytes infiltrating between the collagen bundles in the superficial dermis (Fig. 3).
The multinucleated histiocytes (arrows) are large with an eosinophilic and finely granular "ground-glass" cytoplasm. The nuclei are haphazardly arranged, but tend to favor the center of the cells. A CD163 stain was diffusely positive and the cells were focally PAS-positive diastase-resistant
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Fig4
The nuclei are haphazardly arranged, but tend to favor the center of the cells (Fig. 4).
Radiographs of the hands showed erosions in the left third DIP and right second DIP, mild joint space narrowing with mild diffuse osteopenia, and erosions of the scaphoid bone with surrounding soft tissue swelling
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Fig5
There were also erosions seen in the scaphoid bone with surrounding soft tissue swelling (Fig. 5).
Radiograph of the left shoulder revealing marked glenohumeral joint space narrowing, small humeral head osteophytes, subchondral sclerosis, and subchondral cysts
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Fig6
The patient's left shoulder X-ray showed marked glenohumeral joint space narrowing, small humeral head osteophytes, subchondral sclerosis, subchondral cysts (Fig. 6), and large erosions of the humeral head.
MRI of the shoulder showing moderate glenohumeral joint effusion with synovitis and large well-demarcated erosions on the humeral head
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Fig7
Magnetic resonance imaging (MRI) of the shoulder showed moderate glenohumeral joint effusion with synovitis and well-demarcated erosions of the humeral head (Fig. 7).
Color Doppler imaging demonstrating a minimal effusion but marked synovial proliferation with moderate Doppler flow
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Fig8
We performed an ultrasound on the second right MCP (Fig. 8) and first Interphalangeal joint which showed minimal effusion but marked synovial proliferation with moderate Doppler flow, suggesting disease activity.
Large anechoic effusion and echogenic synovial proliferation with hypervascularity
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Fig9
An ultrasound of the knee showed a large anechoic effusion, echogenic synovial proliferation with grade 2 Doppler flow (Fig. 9).