Dermatology Online Journal

Phacomatosis pigmento-pigmentaria: Aberrant dermal melanocytosis and nevus spilus
Ronni Wolf MD¹, Eleonora Ruocco MD PhD², Adone Baroni MD PhD²
Dermatology Online Journal 17 (8): 9

1. Kaplan Medical Center, Rehovot, Israel. Associate Clinical Professor of Dermatology at the School of Medicine, Hebrew University and Hadassah Medical Center, Jerusalem, Israel
2. Department of Dermatology, 2nd University of Naples, Naples, Italy

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Abstract

We present a dermal melanocytosis with superimposed nevus spilus on the arm of a 5-year-old boy. We recently introduced a new type of phacomatosis, which we termed "phacomatosis pigmentopigmentalis," that is analogous to phacomatosis pigmentokeratotica or pigmentovascularis, for describing the association of two pigmented nevi. The present case is an example of this type of phacomatosis, which now we prefer to define phacomatosis pigmento-pigmentaria according to the correct Latin terminology.

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Case report

Figure 1	Figure 2

A 5-year-old boy presented to our clinic with a congenital, well-demarcated, and homogeneous grayish-blue hyperpigmented macule measuring 10 x 5 cm on the right forearm. He was an only child and there was no history of parental consanguinity or of any remarkable hereditary disorders. Pregnancy and the postnatal period had been uncomplicated and the child was apparently healthy and well developed. A detailed dermatologic examination revealed the presence of a superimposed melanocytic nevus (Figure 1). Videodermoscopy showed a more superficial, diffuse and irregular brown pigmentation on a diffuse grayish-blue pigmentation background. In addition, a patchy, fine, and poorly circumscribed reticular pattern and many globular structures were present (Figure 2). Dermal melanocytosis with superimposed nevus spilus was diagnosed based on the history, clinical, and videodermoscopic data.

Figure 3	Figure 4

A biopsy performed later showed findings of lentigo simplex (pertaining to nevus spilus) in the epidermis and of dermal melanocytosis in the deep reticular dermis (Figures 3 and 4).

Discussion

The term "phacomatosis pigmentovascularis" (PPV) is used to describe the association of a vascular nevus with an extensive pigmentary nevus. PPV had originally been classified into four types and a fifth type was added later [1]. Happle [2] 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 1996, Happle et al [3] introduced another type of phacomatosis, "phacomatosis pigmentokeratotica," for describing the association of speckled-lentiginous nevus with organoid nevus and extracutaneous anomalies.

We [4] recently introduced another type of phacomatosis, which we termed "phacomatosis pigmentopigmentalis," which is analogous to phacomatosis pigmentokeratotica or pigmentovascularis, for describing the association of two pigmented nevi. The present case of an aberrant macule of dermal melanocytosis and a superimposed nevus spilus is an example of this type of phacomatosis, which now we prefer to define as phacomatosis pigmento-pigmentaria according to the correct Latin terminology. In fact, pigmentalis is not a Latin word. The two Latin adjectives concerning pigment (in Latin pigmentum) are pigmentatus, -a, -um (for something which has received pigment) and pigmentarius, -a, -um (for something that produces pigment).

References

1. Torrelo A, Zambrano A, Happle R. Cutis marmorata telangiectatica congenita and extensive mongolian spots: type 5 phacomatosis pigmentovascularis. Br J Dermatol 2003;148(2):342-345. [PubMed]

2. Happle R. Phacomatosis pigmentovascularis revisited and reclassified. Arch Dermatol 2005;141(3):385-388. [PubMed]

3. Happle R, Hoffmann R, Restano L, Caputo R, Tadini G. Phacomatosis pigmentokeratotica: a melanocytic-epidermal twin nevus syndrome. Am J Med Genet 1996;65(4):363-365. [PubMed]

4. Wolf R, Wolf D, Davidovici B. Phacomatosis pigmentopigmentalis: aberrant Mongolian spots and segmental cafe au lait macules. Pediatr Dermatol 2009;26(2):228-229. [PubMed]

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