1/6. Phacomatosis pigmentovascularis type IIa successfully treated with two types of laser therapy.We describe a 28-year-old Japanese woman with phacomatosis pigmentovascularis type IIa who was treated by 27 sessions of Q-switched ruby laser irradiation to the site of dermal melanosis on her face and three sessions of dye laser irradiation to the port-wine stain on her left cheek. This is the first report of the successful treatment of a cutaneous lesion in a patient with phacomatosis pigmentovascularis. As the outcome of the treatments was excellent, we conclude that phacomatosis pigmentovascularis type IIa can be treated successfully by the combination of the two types of laser therapy.- - - - - - - - - - ranking = 1keywords = phacomatosis (Clic here for more details about this article) |
2/6. Nevi flammei affecting two contralateral quadrants and nevus depigmentosus: a new type of phacomatosis pigmentovascularis?Here, we report on a 64-year-old-woman with nevi flammei affecting two contralateral quadrants, venous insufficiency of the right leg, and asymmetry of the arms, in association with a linear nevus depigmentosus. This combination of vascular and pigmentary abnormalities may represent a novel type of phacomatosis pigmentovascularis caused by non-allelic twin-spotting.- - - - - - - - - - ranking = 1.6666666666667keywords = phacomatosis (Clic here for more details about this article) |
3/6. Treatment of phacomatosis pigmentovascularis: a combined multiple laser approach.BACKGROUND: Phacomatosis pigmentovascularis (PPV) consists of a capillary malformation with a variety of melanocytic lesions, which involve various regions of the body and are difficult to treat with conventional therapeutic tools. OBJECTIVE: We described two cases with PPV (type IIa and IIb) that were successfully treated with different lasers. methods: The areas involved by both melanocytic lesions and port-wine stains were treated using the Q-switched ruby laser, the Q-switched Alexandrite laser, and the flashlamp pumped pulsed-dye laser. RESULTS: Removal of a good portion of cutaneous and vascular lesions using combined multiple laser approach was achieved after 6 sessions in the first case under general anesthesia and after 31 sessions under local anesthesia in the second case. CONCLUSION: PPV type II can be treated successfully by laser treatment. We prefer to start combined multiple laser treatment of PPV in childhood period under general anesthesia because it will reduce the number of treatment, improve the patient's quality of life, and increase the cost-effectiveness of the treatment.- - - - - - - - - - ranking = 1.3333333333333keywords = phacomatosis (Clic here for more details about this article) |
4/6. nevus roseus: a distinct vascular birthmark.The new term nevus roseus is proposed to denote a lateralized telangiectatic birthmark with a light-red or pale-pink color, unlike the dark hue of nevus flammeus. It appears to be a distinct entity rather than just a color variant of nevus flammeus. Remarkably, nevus flammeus is a characteristic component of phacomatosis pigmentovascularis type II ("phacomatosis cesioflammea"), whereas nevus roseus represents a distinguishing feature of phacomatosis pigmentovascularis type III ("phacomatosis spilorosea"). In analogy to "port-wine stain" that is used as a synonym for nevus flammeus, nevus roseus could also be called "rose-wine stain". This lateralized vascular birthmark should be distinguished from the salmon patch that always involves the midline of the body. nevus roseus belongs to the rather broad category of telangiectatic nevi, as well as to the even larger group of "capillary malformations". For obvious reasons these terms are not suitable to designate any specific type of vascular birthmark such as nevus roseus.- - - - - - - - - - ranking = 1.3333333333333keywords = phacomatosis (Clic here for more details about this article) |
5/6. Phacomatosis pigmentovascularis type IIb associated with sturge-weber syndrome and pyogenic granuloma.A case of phacomatosis pigmentovascularis (PPV) in a 6-year-old girl with sturge-weber syndrome, pyogenic granuloma, and other complications is described. It is relatively rare that a complete form of sturge-weber syndrome was associated with PPV. A review of the literature on PPV, focusing on total number of reported cases and etiological speculations, is presented. To our knowledge, a total of 118 cases of PPV, including the present one, have been reported to date. Regardless of many speculations, the true etiology remains unknown. The average "density" of mast cells (MCs) per mm2 appearing in the central region of the pyogenic granuloma was calculated to be 86.3/mm2 and that in the adjacent nevus flammeus was 37.9/mm2. The "density" of mast cells in pyogenic granuloma separately calculated from ten other cases was 105.5 /- 28.6/mm2 (mean /- SD), compared with that in normal skin, 6.85 /- 4.9/mm2 (n = 20). There was a significant difference between the two, indicating that MCs are closely associated with angiogenesis in pyogenic granuloma.- - - - - - - - - - ranking = 0.33333333333333keywords = phacomatosis (Clic here for more details about this article) |
6/6. Phacomatosis pigmentovascularis type II.Phacomatosis pigmentovascularis (PPV) is described as the association of cutaneous vascular malformations and different pigmentary disorders. The different associations are classified into four types, with localized and systemic forms. The constant alteration in each type is a nevus flammeus. Associated pigmented abnormalities are nevus pigmentosus and verrucosus in type I, blue spots in type II, nevus spilus in type III, blue spots and nevus spilus in type IV. Type II is the most frequently reported. In this type, half of the patients have visceral involvement, more often Klippel-Trenaunay or/and sturge-weber syndrome. We report two cases of phacomatosis pigmentovascularis type II. The particularity of the first case is the association with a cranio-cervical junction malformation (Arnold-Chiari type I), which to our knowledge, has never been described with PPV. The second case presents with a bilateral scleral melanosis without visceral involvement. Definition of visceral involvement and ophthalmologic manifestations are discussed.- - - - - - - - - - ranking = 0.33333333333333keywords = phacomatosis (Clic here for more details about this article) |