We read with great interest Oiso et al.’s1 description of a 13-year-old Japanese girl with hyperpigmented spots (1–3 mm in diameter) within and partly around a hypopigmented macule on her left lower torso. The hypopigmented macule presented at birth was diagnosed as nevus depigmentosus. The multiple hyperpigmented spots were consistent with lentigines. We agree entirely with the authors’ observation that the simultaneous occurrence of lentigines within nevus depigmentosus is rare, and we suggest that it should be considered a new form of phacomatosis (“pigmento-pigmentaria”) introduced by us in 2009.2

The term “phacomatosis pigmentovascularis” (PPV) is used to describe the association of a vascular nevus with a pigmentary nevus. PPV had originally been classified into four types, and a fifth type was added later on.3 Happle4 recently proposed a new classification of PPV, in which he condensed those five types into four, using three descriptive terms for three of the types and referring to the fourth type as “unclassifiable”. In an earlier paper, his group5 had proposed another type of phacomatosis, “phacomatosis pigmentokeratotica”, for describing the association of speckled-lentiginous nevus with organoid nevus and extracutaneous anomalies.

We2 recently reported yet another type of phacomatosis, a form analogous to phacomatosis pigmentokeratotica or PPV and descriptive of the association of two pigmented nevi. Oiso et al.’s1 case of lentigines within and partly around nevus depigmentosus is clearly an example of this type of phacomatosis, to which we suggest the name “phacomatosis pigmento-pigmentaria” for this new type of phacomatosis.


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