Variant of phacomatosis pigmentokeratotica

An atypical variant of phacomatosis pigmentokeratotica: verrucous epidermal nevus, speckled lentiginous nevus, and Spitz nevus associated with scoliosis

“…Phacomatosis pigmentokeratotica is often associated with neurological (mental retardation, epilepsy, deafness, hemiatrophy, dysesthesia, and hyperhidrosis), ophthalmological (strabismus and ptosis), and musculoskeletal (kyphosis, scoliosis, limb asymmetry, and hypertrophy) abnormalities, and rarely with hypertension, atrioventricular block, hypophosphatemic rickets, and pheochromocytoma 1–5,7–9 . Our patient also had mild scoliosis.…”

“…Nevertheless, both types of SLN were present in our patient. In addition, miscellaneous types of melanocytic nevi such as junctional, compound, and dermal nevi, blue nevi, Spitz nevi, congenital melanocytic nevi, and dysplastic nevi may develop in SLN 2 . Although our case had a Spitz nevus and multiple compound and dermal nevi within SLN, few melanocytic nevi occurred in other regions, and a dome‐shaped, dark brown dermal nevus was observed on the right palmar hypothenar.…”

“…Our patient also had mild scoliosis. Although there have been eight PPK cases without extracutaneous abnormalities, 2,6 some potential extracutaneous anomalies can be found by a long‐term close follow‐up 7…”

“…Two different mosaic spots are often arranged in close proximity to each other, while EN lesions follow a linear pattern along Blaschko lines, and SLN is arranged in a checkerboard pattern 3 . The accumulated data demonstrated that malignant melanoma and basal cell carcinoma can develop in SLNs and organoid nevi, respectively 2,9 . Therefore, even though the malignancy has been undetected thus far, the patient should be advised to monitor lesions at regular intervals.…”

“…There have been 33 cases of PPK since it was first described by Happle et al. in 1996 2 . However, verrucous EN is an uncommon component of PPK 3 .…”