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Hunter Syndrome. Presented by S.G. Ghatia, MD, and J. Blum MD.
The patient is an 8-year-old boy with Hunter syndrome (mucopolysaccharidosis type II) and a pruritic skin eruption on the upper back for one year. There are multiple pale, nontender, white dermal papulonodules 3 to 8 mm in diameter over and below both scapuli. There is also increased hair growth on trunk and extremities. The head is enlarged with scaphocephalic contour and with prominent supraorbital ridges and a wide nasal ridge. There is a short thick neck and a high arched palate. Hands and feet are short and stubby. The skeletal age is about 3 years. Urinalysis was positive for mucopolysaccharides. A skin biopsy specimen with special stains showed normal skin.
Dr. Lester Weiss: Hunter syndrome is one of the disorders of mucopolysaccharide metabolism. Much of the new data regarding these diseases have been acquired through the use of
Mehregan AH. MICHIGAN DERMATOLOGICAL SOCIETY. Arch Dermatol. 1974;109(5):749-750. doi:10.1001/archderm.1974.01630050075035