PMC10118899_01
(a) Macroglossia and lateral scalloping of the tongue, (b) flexion contracture of the knee joint, and (c) skin stiffness of palms and sclerodactyly.
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Macroglossia and lateral tongue scalloping were noted while the patient was unable to protrude her tongue (Figure 1(a)). The findings of extremity examination consisted of fascia hardening of soles and palms, sclerodactyly (Figure 1(c)), arthritis of both wrists and right ankle, lower extremity pitting edema, and muscle stiffness which was particularly evident in biceps and triceps. Furthermore, flexion contracture of knees (Figure 1(b)), elbows, shoulders, and hip was found.
(a and b): MRI of both legs; T1W (a) and fluid sensitive fat sat (b) sequences; perimuscular and fascial edema (curved arrow) and intra muscular edema (arrow) within gastrocnemius muscles; (c and d): MRI of both thighs T1W (c) and fluid sensitive fat sat (d) sequences; perimuscular and fascial edema (arrow) more in anterior compartment.
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For bilateral thigh and arms, magnetic resonance imaging (MRI) revealed widespread perimuscular fluid and edema with concomitant subcutaneous fluid around muscles, as well as grade 1-2 fatty changes in all pelvis and leg and arm muscles along with mild to moderate intramuscular edema (Figures 2(a)-2(d)).
MRI of right arm T1W (a) and fat sat (b) and (c)-(d) MRI of left shoulder T1W (c) and fat sat (d) sequences. Note to the diffuse perimuscular and fascial edema more along the latissimus dorsi and rotator cuff muscles (arrows).
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Another finding of upper extremity MRI was bilaterally diffuse subcutaneous fat thickening (Figures 3(a)-3(d)).
Bone marrow shows sheets of plasmacytoid cells. (a) H&E. (b) CD138. (c) Lambda. (d) Kappa.
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The majority of cells were positive for CD138 with lambda light chain restriction (Figure 4).
Muscle biopsy of the medial head of the gastrocnemius muscle demonstrating deposition of hyalinized amorphous extracellular eosinophilic material in vessel walls and endomysial connective tissue. (a) Hematoxylin and eosin. (b) Congo red.
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Also, endomysial and perimysial connective tissue was expanded with foci of deposition of the same material, which was confirmed to be amyloid by Congo red staining (Figure 5).