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Natural history of extensive Mongolian spots in MPS2 (Hunter syndrome): a survey among 52 Japanese patients.
We experienced difficult intubation in a case with a medical history of inguinal hernia operation; the patient exhibited an ectopic Mongolian spot, coarse face, stiff joints, and claw hand deformity, some of which are clearly characteristic symptoms of MPS.
The most common site for mongolian spots was lower back (58.4%) and gluteal region (35.2%) (Fig.
In the papillary and upper reticular dermis, dendritic melanocytes are present and surrounded by fibrous sheaths (which are not present in other dermal melanocytosis, such as blue nevus or mongolian spots).
93 children showed Mongolian spots. The signs of miniature puberty were found in 82 children.105 children had sebaceous hyperplasia.
Typical and limited Mongolian spots are benign skin markings, commonly appear at birth or shortly after, and are not associated with any disorder.
(1) Sebaceous hyperplasia, milia, Epstein's pearls and Mongolian spots are present in a majority of neonates at birth or a few hours thereafter.
* The differential diagnosis for CMN includes cafe au lait macules, blue-gray spots (aka Mongolian spots), nevus of Ota, nevus spilus, and vascular malformations (table).

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