By Gary M. White, MD

CDAGS syndrome is the constellation of craniosynostosis and clavicular hypoplasia; delayed fontanel closure, cranial defects (such as parietal foramina), deafness; imperforate anus or anterior anal placement; genitourinary abnormalities (such as hypospadias and urethrorectal fistula); and skin eruption, especially porokeratosis-like lesions.


Circinate, erythematous, erosive plaques on the skin occur. They may have a firm, raised, thread-like border reminiscent of porokeratosis.


A 2-year-old developmentally delayed girl presented with a skin eruption that had delayed corrective surgery for craniosynostosis. She had been born with an imperforate anus. The skin lesions began shortly after birth, progressively worsened, and were variably pruritic and tender. [JAAD May 2013;Volume 68, Issue 5, Pages 881–884 ]


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