A 37-year-old woman with a personal history of appendicectomy,
cholecystectomy, left
oophorectomy secondary to an
ovarian cyst complication, nephritic
colic with repeated episodes of
pyelonephritis, alcoholic hepatopathy, Raynaud's phenomenon and bilateral
exophthalmos showed an increase in volume in the root of the upper limbs and in the base of the neck over a period of 4 years, painful to the touch and of a soft consistency. She presented with a pseudo-athletic appearance (Fig. 1) produced by an increase in the volume at the root of the upper limbs, upper back and the back of the neck (Fig. 2). The lesions produced a pulling sensation and were associated with
paresthesia,
hyperesthesia, and a moderate loss of strength in both arms. A biopsy taken from the upper third of the right arm showed a diffuse proliferation of the subcutaneous adipose tissue, which appeared normal, and extended between the
collagen fibers, reaching in some cases into the most superficial zones of the reticular dermis (Fig. 3). Laboratory evaluation revealed a chronic
anemia,
leukopenia with moderate
lymphopenia, increased erythrocyte sedimentation rate, elevation of
enzymes of hepatic function, decrease in total
proteins, and increase in
ferritin, all in the context of hepatopathy.
Antinuclear antibodies and the hormonal profile were normal. Abdominal and gynecologic echography revealed a right
ovarian cyst of no clinical relevance. Cranial nuclear magnetic resonance (NMR) revealed an increase in the periorbital fat responsible for bilateral
exophthalmos.