Case of the Month, December, 2010, Page 2

Does the following image narrow your differential?
 

Single-contrast esophagram:


 

Findings:
 

  • AP radiograph of bilateral hands shows numerous confluent areas of dense calcification primarily centered around joint spaces.
  • Single contrast esophagram shows a short-segment stricture with a dilated proximal esophagus.

Diagnosis:

Scleroderma with CREST Syndrome

  • Scleroderma is an uncommon disorder with an unknown etiology that usually presents in the 3rd to 5th decades of life. It has a female:male ratio of 3:1. Early symptoms include Raynaud’s phenomenon, skin thickening and edema of the extremities, arthralgia of the small joints and knees, and dysphagia. Progression of the disease leads to taut, shiny, atrophic skin with tapering of the distal digits.
  • Two very important clues to making the diagnosis of scleroderma radiographically include soft tissue calcification and acro-osteolysis. Almost 75% of patients with calcinosis have calcification in the hands which may occur in either the subcutaneous tissue or the joint capsule. Erosions are a common finding and typically involve the DIP and PIP joints. Severe resorption of the first CMC joint with radial subluxation of the first metacarpal is nearly pathognomonic for scleroderma. Flexion contractures are a very common finding of the hands and feet.
  • CREST syndrome is an acronym for calcinosis, Raynaud’s phenomenon, esophageal abnormalities, sclerodactyly, and telangiectasia. The entire GI tract may be involved but the esophagus is the most frequently affected. Dysmotility, esophagitis, and stricturing are common. The “hide-bound” appearance of small bowel is a classic sign of scleroderma on small bowel series. It is caused by fibrosis of the inner circular smooth muscle with narrow packing of the valvulae conniventes which are themselves normal in thickness.
  • Sclerodactyly refers to thickening of the skin, but generally patients with scleroderma progress from edematous, to indurative, to atrophic phases. Telangiectases are lesions formed by collections of dilated vessels and typically occur on the face, hands, upper trunk, and mucosal surfaces (which may result in hemorrhage).
  • Other less common associations include pulmonary hypertension, myocardial fibrosis, primary biliary cirrhosis, renal involvement, entrapment neurologic syndromes, and an increased risk of cancer (particularly lung cancer).

References:

  • Yu, Joseph S. Musculoskeletal Imaging: Case Review Series. Mosby, 2008.
  • Bassett LW, Blocka KLN, Furst DE, Clements PJ, Gold RH. “Skeletal Findings in Progressive Systemic Sclerosis (Scleroderma).” American Journal of Roentgenology 136:1121-1126, June 1981.
  • Pickhardt PJ. “The Hide-bound Bowel Sign.” Radiology 213:837-838, December 1999.
  • Horner KL. “CREST Syndrome.”  eMedicine. 22 Sept. 2009. 12 Dec. 2010. <http://emedicine.medscape.com/article/1064663-overview>
  • Federle MP, Venkata A. “Scleroderma, Esophagus.” STATdx 12 Dec. 2010. <https://my.statdx.com/>
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