Inheritance of Hunter syndrome is X-linked recessive. Iduronate sulfatase is deficient. Heparan sulfate and dermatan sulfate are excreted in the urine at increased levels.


Firm, ivory white papules and nodules which may coalesce to form ridges or a rippled pattern are markers of Hunter syndrome. They characteristically occur on the back overlying both the scapula and the pectoral area but also occur on the nape, arms, and thighs. They begin appearing in the second or third year of life.


Cutaneous biopsy may aid in the diagnosis but treatment involves a multidisciplinary approach.


Mongolian spot and Hunter syndrome. JAAD December 1998;Volume 39, Issue 6, Pages 1013–1015

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