Clinical characteristics and treatment of 52 cases of phakomatosis pigmentovascularis.

Phakomatosis pigmentovascularis is a rare syndrome characterized by widespread capillary malformation and pigmented nevus. The objective of this study was to evaluate its characteristics and treatment. Fifty-two patients presenting between 2003 and 2017 were retrospectively reviewed. Type IIa (port-wine stain and dermal melanocytosis with oculocutaneous involvement) was most common. Systemic involvement was observed in 17.3% and it was not significantly correlated to extent of capillary malformation or pigmented nevus. However, systemic involvement was significantly frequent in patients with nevus of Ota and in patients with pigmented nevus located on the head and neck (P = 0.004 and 0.035, respectively). Capillary malformation was almost cleared in 28.6% of patients using pulsed dye laser, whereas pigmented nevus was almost cleared in 23.7% and completely cleared in 42.1% of patients using Q-switched neodymium:yttrium-aluminum-garnet laser. Treatment outcome score showed significant inverse correlation with the extent of port-wine stain or pigmented nevus (P = 0.047, ρ = -0.308 and P = 0.011, ρ = -0.410, respectively). Pigmented nevus demonstrated better treatment response to lasers than did capillary malformation. Smaller lesions tended to show better treatment outcomes for both capillary malformation and pigmented nevus.

Prospective pilot study on combined use of pulsed dye laser and 1% topical rapamycin for treatment of nonfacial cutaneous capillary malformation.

BACKGROUND: The regeneration or revascularization of blood vessels after pulsed dye laser (PDL) treatment is one of the causes of treatment failures of cutaneous capillary malformations (CM). Recently, topical administration of rapamycin was introduced as a possible adjunctive therapeutic option to minimize postlaser revascularization in facial CM. OBJECTIVES: We evaluated the effect of combined use of 1% topical rapamycin with PDL compared to PDL alone in cutaneous CM of trunk or extremities and tried to identify the optimal duration of topical rapamycin application. METHODS: Three adjacent areas of cutaneous CM that had never been treated before were selected in each patient and underwent the following regimens: (A) PDL + vehicle for 8 weeks post-PDL; (B) PDL + topical rapamycin for 1-week post-PDL and (C) PDL + topical rapamycin for 8 weeks post-PDL. Each test site was treated by PDL for two sessions with 8 weeks interval. RESULTS: Only one of six patients showed clinical improvement with combined rapamycin treatment. Overall, there was no statistically significant difference in erythema and blanching rate among PDL alone and combined rapamycin regimens. CONCLUSION: One percent topical rapamycin does not seem to be effective as a treatment modality for cutaneous CM of trunk or extremities.

Clinical experience in the treatment of port-wine stains with blebs.

BACKGROUND: The current modality of choice for the treatment of Port-wine stains (PWS) is laser photocoagulation. Laser therapy for the treatment of PWS, especially with a pulsed dye laser (PDL), has been proven safe and effective; however, because penetration of the PDL is too shallow for an effective ablation of the blebs, treatment of blebbed PWS, using PDL, may be insufficient. OBJECTIVE: We demonstrated the clinical efficacy of a 1,064 nm long pulsed Nd:YAG laser with contact cooling device for blebbed PWS. METHODS: Twenty one patients with blebbed PWS (Fitzpatrick skin types II-V) underwent a treatment, using a 1,064 nm long pulsed Nd:YAG laser with a contact cooling device at 8-week intervals. Treatments were done using 5~6 mm spot sizes at 20~30 ms and 95~170 J/cm(2). Laser parameters were adjusted in order to meet the needs of each individual patient's lesions. RESULTS: All subjects tolerated the treatments well, and showed clinical improvement from blebs. Of the 21 patients, 18 of them experienced either moderate or excellent response. CONCLUSION: Use of a 1,064 nm long pulsed Nd:YAG laser results in a greater depth of vascular coagulation. A 1,064 nm long pulsed Nd:YAG laser with contact cooling device may be regarded as a promising therapeutic option for the treatment of blebbed PWS.

Treatment using a long pulsed nd:yag laser with a pulsed dye laser for four cases of blebbed port wine stains.

Port wine stains (PWS) are congenital capillary malformations consisting of ectasia of capillaries and venules. At birth,lesions are flat and relatively uniform in color, but evolve with age to become raised, thickened, irregularly surfaced, and deeply colored. Therefore, it is considered optimal to begin treatment of patients at an early age. Conventional treatment modalities, such as electrocautery or excision, require considerable effort and may be cosmetically unsatisfactory. We have performed treatment of blebbed PWS of four patients using a 1,064 nm long pulsed Nd:YAG laser with a contact cooling device. According to their size, most blebs required three or fewer treatment sessions at 8-week intervals. Treatments were well tolerated by all subjects and patients showed moderate to good improvement of blebs. A 1,064 nm long pulsed Nd:YAG laser with contact cooling may be considered as a promising therapeutic option for treatment of blebbed PWS.

Angioblastoma showing aggravation after treatment with long-pulsed Nd:YAG laser (1064 nm).

Angioblastoma usually develops in infancy or early childhood on the neck or upper trunk. It is known to be slowly progressive and benign in nature, but treatment guidelines have not yet been established. Spontaneous regression has been occasionally documented, and treatment with pulsed dye laser, excision, high-dose steroids, and interferon alpha have been successful in individual patients. Our patient experienced partial response to interferon alpha injection, and for further treatment, long-pulsed Nd:YAG laser (1064 nm) treatment was performed. However, unexpectedly, the tumor was rapidly aggravated. We report this occurrence to increase awareness of trauma-induced aggravation phenomena in angioblastoma.