Pebbled skin over axilla and umbilicus

Pseudoxanthoma elasticum or Grönblad-Strandberg syndrome is characterized by progressive calcification and degeneration of the elastic fibers throughout the body. They are noted in the second or third decade and comprise flat-topped, discrete-to-confluent yellowish papules in a linear or reticular...

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Bibliographic Details
Main Authors: Ankita Agrawal (Author), Yogesh Kalyanpad (Author)
Format: Book
Published: Wolters Kluwer Medknow Publications, 2019-01-01T00:00:00Z.
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Summary:Pseudoxanthoma elasticum or Grönblad-Strandberg syndrome is characterized by progressive calcification and degeneration of the elastic fibers throughout the body. They are noted in the second or third decade and comprise flat-topped, discrete-to-confluent yellowish papules in a linear or reticular pattern over flexures, and periumbilical skin giving a "cobblestone," "plucked chicken skin," or "Moroccan leather" appearance. We report a case of 13-year-old-child with yellowish pebbled skin over axilla and umbilicus. Angioid streaks were present on ophthalmological examination. On histopathology, fragmented and curled fibers were present in the dermis giving ravelled wool appearance. Von Kossa and Verhoeff-van gieson stain fibers stains showed calcified and fragmented elastic fibers in the mid-dermis.
Item Description:2319-7250
10.4103/ijpd.IJPD_58_18