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Picture of the Month—Quiz Case
Basil J. Zitelli, MD
Author Affiliations: The Paul C. Gaffney Diagnostic Referral Service, Department of Pediatrics, Children’s Hospital of Pittsburgh, Pittsburgh, Pa.
Arch Pediatr Adolesc Med. 2005;159:721.
A 28-year-old man presented with numerous musculoskeletal findings, the following of which were noted (Figure 1): in the head and neck: dolichocephaly, malar hypoplasia, highly arched palate, retrognathia, and ectopia lentis with iridodonesis; in the extremities: long limbs (dolichostenomelia), long, tapered fingers (arachnodactyly), contractures of several joints, and pes planus, as well as "thumb sign" (Figure 2) and "wrist sign" (Figure 3); and in the torso: pectus carinatum, scoliosis, and striae atrophicae. His medical history was notable for spontaneous pneumothorax and aortic root dilatation.
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Figure 1. Physical findings in a 28-year-old man.
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Figure 2. "Thumb sign": when the hand is clenched without assistance, the entire thumbnail projects beyond the border of the hand.
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Figure 3. "Wrist sign": when the wrist is grasped by the contralateral hand, the thumb overlaps the terminal phalanx of the fifth digit.
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Picture of the Month—Diagnosis
Arch Pediatr Adolesc Med. 2005;159(8):722-723.
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