[Skip to Content]
Access to paid content on this site is currently suspended due to excessive activity being detected from your IP address 50.16.79.246. Please contact the publisher to request reinstatement.
Sign In
Individual Sign In
Create an Account
Institutional Sign In
OpenAthens Shibboleth
[Skip to Content Landing]
Citations 0
Special Feature
August 2005

Picture of the Month—Quiz Case

Author Affiliations

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(8):721-723. doi:10.1001/archpedi.159.8.721

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.

Figure 1.
Physical findings in a 28-year-old man.

Physical findings in a 28-year-old man.

Figure 2.
“Thumb sign”: when the hand is clenched without assistance, the entire thumbnail projects beyond the border of the hand.

“Thumb sign”: when the hand is clenched without assistance, the entire thumbnail projects beyond the border of the hand.

Figure 3.
“Wrist sign”: when the wrist is grasped by the contralateral hand, the thumb overlaps the terminal phalanx of the fifth digit.

“Wrist sign”: when the wrist is grasped by the contralateral hand, the thumb overlaps the terminal phalanx of the fifth digit.

What is your diagnosis?

×