Faculty Bibliography

The Rothmund-Thomson syndrome is a rare disorder characterized by poikiloderma, juvenile cataracts, bone abnormalities, short stature, sparse hair, hypogonadism, defective dentition, nail dystrophy, hyperkeratosis, and mental retardation. A 12-year-old boy with the syndrome was referred to us for evaluation and treatment of poikiloderma. This component of the disease was markedly improved with the use of pulsed dye laser photocoagulation

Twelve children, 6 to 30 weeks of age (average 14.9 weeks), with port-wine stains of the head and neck were treated with the flashlamp-pumped pulsed dye laser at 585 nm and 450 microsecond pulse duration. Ten of 12 patients (83%) showed more than 50% lightening of their port-wine stains after 2.9 treatment sessions (2.9 +/- 1.4 [+/- standard deviation]). Forty-five percent of the patients demonstrated 75% or more lightening of their lesions after a mean of 3.8 treatments (+/- 1.6). No lesions in this group cleared completely after a mean of 2.8 treatments. Treated skin was identical in texture to normal skin in all patients. There was no evidence of depressed scars, atrophy, hyperpigmentation, or hypopigmentation in the treated areas. These results indicate that pulsed dye laser treatment of port-wine stains can be undertaken safely in infancy

A patient with epidermodysplasia verruciformis died of intracranial invasion by squamous cell carcinoma (SCC). A biopsy of clinically normal skin had revealed perineural invasion by malignant keratinocytes. Because SCCs arising in patients with epidermodysplasia verruciformis may be biologically aggressive, it is best to excise them with careful control of the surgical tissue margins

Twelve children 2 to 10 years of age with spider telangiectases of the face were treated with a flashlamp-pumped pulsed dye laser at a wavelength of 585 nm and a pulse width of 450 microsec. Complete resolution of the telangiectases was noted in all 12 patients, without scarring or permanent pigmentary alteration of the skin

Strawberry, or capillary, hemangiomas are common vascular neoplasms, with an incidence of approximately 2.6% in neonates. They usually develop in the first few weeks of life, so that between 1 month and 1 year the incidence rises to between 8.7% and 10.1%. These lesions may grow quite large in the first year of life, and they may ulcerate or obstruct a vital organ or function. The great majority will spontaneously regress after the first year of life. Parents are often alarmed at the sight of these hemangiomas and need reassurance that the great majority will regress spontaneously. Treatments such as cryosurgery, irradiation, radium instillation, corticosteroid therapy, or surgical excision are often ineffective or cause significant morbidity. We describe 10 children with capillary hemangiomas treated with the flash lamp-pumped pulsed dye laser. The patients ranged in age from 7 weeks to 5.5 years at the beginning of laser therapy. The patients underwent 3.1 +/- 1 (mean +/- SD) laser treatments, with a mean regression of the lesions of 69.9% +/- 4.5%. All patients demonstrated some diminution in the size and color of their hemangiomas after the treatments, and there were no ill effects, such as ulceration, hemorrhage, infection, or scarring. There was no evidence of hyperpigmentation or hypopigmentation. Pulsed dye laser therapy should be considered as an option in the treatment of capillary hemangiomas, preferably prior to their full evolution. It is also a useful therapeutic approach in those hemangiomas that are slow to regress in older children

New lasers and improved laser delivery systems have allowed for the safe and effective treatment of port-wine stains in patients of all ages. The satisfactory results obtained by laser treatment have increased the number of patients seeking consultation regarding their birthmarks. It is imperative that physicians recognize the various medical syndromes and problems associated with port-wine stains. A review of 415 patients with facial port-wine stains has revealed hypertrophy and/or nodularity in 65% of patients by the fifth decade of life, which increases significantly the morbidity of these lesions. It is believed that laser treatment will minimize the medical and psychologic complications that result from the natural evolution of port-wine stains

Seventy-three patients between the ages of 3 months to 14 years (average age 6 years 2 months) with port-wine stains were treated with the flashlamp-pumped pulsed dye laser. More than 75% lightening was achieved with an average of 2.5 treatments in 33 patients (45%), 50% to 74% lightening after an average of 1.7 treatments in 31 (42%), 26% to 49% lightening after 2 treatments in 5 (7%), and less than 25% lightening after 1 treatment in 4 (5%). The overall average lightening after one treatment was 53%. The percentage of lightening increased as the number of treatments increased. Three patients had 100% clearance of the port-wine stain. Patients aged between 3 months and 6 years (44 patients) had a better response after the first treatment (55% lightening) than did patients aged between 7 and 14 years (29 patients with a 48% lightening; p = 0.027). Complications included cutaneous depressions in four patients, hyperpigmentation in 16 patients, and hypopigmentation in three patients. All complications were transient and disappeared completely

Porokeratosis of Mibelli is a disorder of epidermal proliferation in which an abnormal clone of cells expands in a centrifugal manner. We present a case of porokeratosis of Mibelli with an underlying hemangioma that was treated with a 585 nm flashlamp-pumped pulsed dye laser. The underlying hemangioma responded well to laser therapy while the porokeratosis remained unchanged. The implications of this for the pathogenesis of porokeratosis and the specificity of the pulsed dye laser are discussed

EMLA cream (Eutectic Mixture of Local Anesthetics) is a new topical anesthetic composed of 25 mg lidocaine and 25 mg prilocaine in an oil-in-water emulsion cream. It has been found to be very effective for local anesthesia prior to venepuncture, minor surgical procedures, and pulsed dye laser (PDL) therapy for port-wine stains (PWS) in children. However, since EMLA may cause vasoconstriction of cutaneous vessels, we tried to determine whether pretreatment with EMLA decreases the efficacy of subsequent PDL treatment. We report eight patients between the ages of 4 and 32 years with PWS who received two test site treatments prior to PDL treatment. One site was pretreated with EMLA cream under occlusion for 60 minutes and then left unoccluded for 15 minutes prior to PDL test treatment. The other site, in the same area of the PWS and patient's body, was not pretreated with EMLA. The test sites were compared 6-8 weeks later to determine whether EMLA decreased the degree of lightening of the PWS compared to the non-EMLA-treated site. We conclude that EMLA is an effective topical anesthetic for PDL treatment of PWS and does not adversely affect the efficacy of the treatment