Phacomatosis Pigmentokeratotica

Abstract

For reconstruction of the pelvic floor after sacrectomy, Localio et al. 6 advocated obliteration of the dead space by tight closure of the gluteus maximus. However, if the resection has been extensive, the residual soft tissue is often insufficient for adequate closure. In these circumstances Santora et al. 1 suggest that prosthetic mesh should be used prophylactically to reconstruct the pelvic floor and reduce the likelihood of sacral herniation 1. Symptomatic sacral hernia following sacrectomy is a rare but important complication, and during the initial surgical resection an attempt should be made to close the soft tissues adequately. Like other groups we have found that polypropylene mesh offers a simple, adaptable, and effective method of hernia repair and pelvic floor reconstruction.

Two Concurrent Facial Epidermal Nevi without Systemic Abnormalities: Nevus Sebaceus and Nevus Comedonicus

“…However, the macules of our patient were in close proximity to the NS and the NC. Melanocytic nevus is considered one of the cutaneous abnormalities in EN14. Moreover, considering that phacomatosis pigmentokeratotica is characterized by the presence of an NS and a contralateral or ipsilateral speckled lentiginous nevus, hyperpigmented macules may be associated with NS or NC15.…”

Phacomatosis pigmentokeratotica associated with hemihypertrophy and a rhabdomyosarcoma of the abdominal wall

“…Spatial overlap of the SLN and ON was observed in 33% (10/30) of the cases. 2,6,9,12,18,20,21,22 Melanocytic nevi of various types have been noted to develop within the SLNs of patients with PPK, including blue, 2,18,22,23 Spitz, 9,12,16,20 and dysplastic nevi 15 as well as banal junctional, compound, and dermal nevi. Several patients (including ours) were noted to have larger, hypertrichotic plaques with clinical and histologic features of a ''classic'' congenital melanocytic nevus (CMN) within their SLNs, 11,17,18,22 supporting the hypothesis that SLNs represent a subtype of CMN.…”

“…Extracutaneous features of PPK can include a variety of musculoskeletal, neurologic, and ophthalmologic abnormalities (Table I). [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] In addition, at least 6 of the 30 patients with PPK reported to date had ONassociated hypophosphatemic vitamin Deresistant rickets. 2,6,12,17,19 Herein we describe a patient with PPK associated with right hemihypertrophy who developed an ipsilateral embryonal rhabdomyosarcoma during the first year of life.…”

Phacomatosis pigmentokeratotica associated with compound melanocytic nevus of the conjunctiva

“…Other abnormalities, including rhabdomyosarcoma, 5 pheochromocytoma, 14 renal artery stenosis, 22 aortic stenosis, 19 arterial hypertension 19,22 , and hypophosphatemic rickets 4,14 have also been reported in PPK, but PPK is known to sometimes occur without systemic involvement. 3,23,24,29 To date, approximately 30 patients with PPK have been reported. To our knowledge, this is the first case of PPK reported in Iran.…”