Primary cutaneous mucinoses are a heterogeneous group of diseases characterized by the abnormal deposition of glycosamminoglycans (mucin) in the skin as the main histopathological clue resulting in distinctive clinical presentations. They are usually diseases of adult age and are uncommon in children in whom the diagnosis and the management are a difficult task. In particular, the classification of primary cutaneous mucinoses occurring in childhood is a difficult and confusing challenge because of the very limited number of described cases and overlaps in their clinicopathological features in the absence of reports of homogeneous case series. The aim of this review is to help dermatologists and pediatricians identify patients with cutaneous mucinoses in pediatric age and enable institution of appropriate management. A classification of primary pediatric cutaneous mucinoses is also proposed.

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