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Significance of extensive Mongolian spots in Hunter's syndrome.

BACKGROUND: The importance of early diagnosis in infants with a mild form of Hunter's syndrome should be emphasized. If applied sufficiently early, haematopoietic stem cell transplantation (HSCT) or recombinant enzyme therapy may improve the prognosis. At present, however, diagnosis of the mild form of Hunter's syndrome tends to be delayed, especially in infants with relatively normal intelligence. OBJECTIVES: To investigate the occurrence of Mongolian spots in infants with Hunter's syndrome, and to clarify the relationship between the Mongolian spots and Hunter's syndrome clinically and histopathologically. METHODS: Seven Japanese boys with Hunter's syndrome who had received HSCT at ages 4-11 years were observed. The cutaneous manifestations of Mongolian spots before HSCT were evaluated, and compared with those after HSCT. In two patients, the hyperpigmentation from the Mongolian spots was examined by light and electron microscopy. RESULTS: Pre-HSCT observation revealed that all the patients had an extensive Mongolian spot. These were present at birth and have shown no signs of resolution during the post-HSCT period. Electron microscopic findings showed that pigment-bearing dermal melanocytes contained many free melanosomes in stage IV. These were surrounded by extracellular sheaths and encircled by elastic fibres. CONCLUSIONS: Our results indicate a strong clinical correlation between the extensive Mongolian spots and Hunter's syndrome. Ultrastructural findings also clearly suggest that the hyperpigmentation is a long-lasting symptom. The recognition of the extensive Mongolian spots is essential as it may lead to early diagnosis in patients with a mild form of Hunter's syndrome.

Child↗

Efficacy of pulsed dye laser treatment of port wine stain malformations of the lower limb.

BACKGROUND: There is relatively little information about treatment of port wine stains (PWS) of the lower limb. Few studies have specifically addressed the efficacy of pulsed dye laser (PDL) treatment of a PWS on the lower limb and there is no information on the relative efficacy at different sites on the lower limb. It has been suggested that treatment is not as successful when compared with the face and postinflammatory hyperpigmentation has been reported to be a significant problem. OBJECTIVES: To review retrospectively patients who attended for PDL treatment of PWS affecting the lower limb and assess the efficacy and adverse effects with particular reference to skin site. METHODS: A retrospective review was carried out of patients attending the Leeds Dermatology Laser Centre for treatment of a PWS on the lower limb with a 585-nm PDL. RESULTS: A total of 52 sites in 39 patients were treated: 17 on the thigh, 31 on the lower leg and four on the foot. The mean number of sessions per patient was 14, with an excellent outcome in seven treatment sites (13.5%), good in 13 (25%), moderate in 21 (40.4%) and poor outcome in 11 (21.1%). Patients were generally pleased with their results with a mean improvement of 7 on a scale of 0-10. Perifollicular persistence of the PWS was observed in six sites (11.5%). Adverse effects occurred in 36 patients (92.3%), most commonly hyperpigmentation (87%). Six patients (15.4%) developed atrophic scarring and four (10.3%) hypopigmentation. Atrophie blanche-like changes were seen in four patients on the lower leg. Hypertrophic scarring was not seen. CONCLUSIONS: Although physician-assessed good or excellent responses of 38.5% are lower than frequently reported for other skin sites and adverse effects may be more frequent, patient satisfaction with treatment was generally high. Patients with PWS on the lower limb merit a trial of PDL treatment.

Adolescent↗

Nitric oxide and endothelin-1,2 in actinic keratosis and basal cell carcinoma: changes in nitric oxide/endothelin ratio.

BACKGROUND: Nitric oxide (NO) is an inorganic free radical gas which has cytostatic/cytotoxic actions in tumoral tissues, including gynecologic, breast, and colon cancer. Nitric oxide is also a multifunctional signaling molecule active in many cells of the body, including endothelial cells, macrophages, monocytes, hepatocytes, mast cells, osteoblasts, and astrocytes. Endothelin-1 (ET-1) is a 21-amino acid peptide that stimulates the proliferation of vascular smooth muscle cells, fibroblasts, and keratinocytes, and plays a role in the expression of proto-oncogenes (c-myc, c-fos), which precedes cell proliferation. Similar to NO, ET is secreted by different cell types, including macrophages, monocytes, hepatocytes, endothelial cells, vascular smooth muscle cells, and various tumor cells. Elevated ET-1 levels are observed in pulmonary, hepatocellular, and prostate cancers. Actinic keratosis (AK) and basal cell carcinoma (BCC) are common skin tumors with accentuated hyperkeratinization, hyperpigmentation, and keratinocyte proliferation. AIM: To investigate plasma NOx (nitrite/nitrate -- the end products of NO metabolism), ET, and the NOx/ET ratio in patients with AK and BCC in comparison with healthy controls. METHODS: NOx, ET, and the NOx/ET ratio were measured in 13 patients with AK, 12 patients with BCC, and in 16 healthy controls. RESULTS: Data analysis indicated a significant increase in plasma NOx, ET, and NOx/ET values in BCC patients in comparison with controls (P < 0.001, P < 0.05 and P < 0.001, respectively). Plasma ET levels in AK were also increased in comparison with controls (P < 0.001). When the two study groups (AK and BCC) were compared, a significant increase (P < 0.001) in the NOx/ET ratio in BCC was found. CONCLUSIONS: The increased plasma ET and NOx levels in AK and, especially, BCC are probably the result of and/or reason for the accentuated hyperkeratinization, hyperpigmentation, and keratinocyte proliferation. The increased production of ET and NO by keratinocytes may function as growth and cytotoxic factors and potential mitogens, and may accelerate further proliferation of these skin tumors. In addition, the increased NOx/ET ratio probably reflects a disturbed equilibrium between these two substances, leading to cell damage and tumor promotion and proliferation.

Adult↗

Allergic contact dermatitis to temporary tattoos with positive para-phenylenediamine reactions: report of four cases.

BACKGROUND: In recent years, temporary tattoos instead of permanent tattoos have become popular worldwide. Although contact allergy to temporary henna tattoos appears to be rare in the past, it is progressively more commonly reported. PATIENTS: Four Taiwanese patients of allergic contact dermatitis following application of temporary tattoos were patch tested and they were followed up for 1 year after treatment. RESULTS: All four of the tested patients were positive to paraphenylenediamine. At 1-year follow-up, all four patients still showed various degrees of remnant hyperpigmentation on their previous tattooed areas. CONCLUSION: Temporary tattoos may pose similar risks of allergic reactions associated with permanent tattoos. A high risk of prolonged post-inflammatory hyperpigmentation after allergic contact dermatitis from temporary tattoos should also be alerted, especially in Asian skin type.

Adult↗

Treatment of presumed iris melanoma in dogs by diode laser photocoagulation: 23 cases.

A semiconductor diode laser was used to cause remission of isolated presumed iris melanoma in 23 dogs. All cases presented as unilateral areas of raised iris hyperpigmentation, ranging in size from 2x 3 mm to 4x12 mm. Cases were treated using a diode laser delivery system through either an operating microscope adapter (OMA) or a laser indirect ophthalmoscope (LIO) with a 20D lens. Laser treatment was delivered 'to effect' using power ranging from 80 to 1000 mW and cumulative durations up to 14 min, 31 s (14:31). Immediate shrinkage of the mass was noted following treatment. Five cases required more than one laser treatment with three eyes receiving two treatments and two eyes receiving three treatments. Follow-up from the last laser treatment ranged from 6 months to 4.5 years during which time the lesions exhibited no enlargement. Minor complications related to laser treatment were seen, including: dyscoria, iris hyperpigmentation, and corneal edema due to collateral hyperthermia. Glaucoma and cataract formation were not observed. Non-invasive diode laser photocoagulation appears to be a safe and effective method of treatment for isolated, pigmented iris masses in dogs.

Journal Article↗

Post-sclerotherapy compression: controlled comparative study of duration of compression and its effects on clinical outcome.

BACKGROUND: Although much has been published on the effects of compression on the venous system, relatively few studies address the duration of compression following sclerotherapy of telangiectatic webs associated with reticular veins. OBJECTIVE: To perform a controlled study comparing the effects of different durations of compression following sclerotherapy of reticular veins and telangiectasias in similar locations. METHOD: This study consisted of a total of 40 patients, 30 patients who received compression therapy and 10 control patients who did not receive compression therapy. The compression group consisted of 10 in each of three duration groups: 3 days, 1 week, and 3 weeks. Patients were evaluated at 1 week, 2 weeks, 6 weeks, 12 weeks, and 24 weeks for degree of improvement and side effects. RESULTS: The three compression groups showed significantly greater improvement at 6 weeks (p = .004). There was a strong correlation between the length of time compression was applied and degree of improvement at 6 weeks, 12 weeks, and 24 weeks of clinical follow-up; r = .74, p = .0006, r = .59, p = .006, r = .66, p = .0001, respectively. The patients treated with compression for 3 days and 1 week had more improvement than the control patients while the patients treated for 3 weeks of continuous compression had the most improvement. In terms of side effects, the 1 week and 3 week compression groups experienced the least amount of post-sclerotherapy hyperpigmentation. CONCLUSIONS: Compression enhances the results following sclerotherapy in a statistically significant way and is directly correlated with duration of compression. Three weeks of continuous compression leads to the best results, although even 3 days of compression results in greater improvement than no compression. Compression leads to a statistically significant reduction of post-sclerotherapy hyperpigmentation.

Adult↗

Tumescent anesthesia in ambulatory phlebectomy: addition of epinephrine.

BACKGROUND: The advantages of using tumescent anesthesia for ambulatory phlebectomy have recently been described. Previously, tumescent solutions have avoided epinephrine for concerns of toxicity given the large volume of anesthetic sometimes used. OBJECTIVE: To evaluate the efficacy and safety of using epinephrine in the tumescent anesthesia solution during ambulatory phlebectomy. METHODS: Over the course of 1 year, epinephrine in the concentration of 1:100,000 was added to the tumescent solution of patients undergoing ambulatory phlebectomy. A retrospective review of 94 sequential patients was performed to determine the rate of complications associated with the procedure. RESULTS: The complication rate was considerably improved using tumescent lidocaine with epinephrine compared to a previously performed study of tumescent lidocaine without epinephrine. The rate of hematoma was decreased to nil while the rate of hyperpigmentation decreased from 3.6% to 0%. Overall, the rate of complications was improved when epinephrine was added to the tumescent lidocaine solution. Blood pressure measured every 5 minutes and heart rate measured continuously did not significantly change before, during or after infiltration of the anesthetic solution. CONCLUSIONS: Epinephrine in appropriate concentrations is clearly safe when used in the tumescent anesthetic solution during ambulatory phlebectomy and should be used to reduce the incidence of hematoma and hyperpigmentation.

Ambulatory Surgical Procedures↗

Cryogen spray cooling and higher fluence pulsed dye laser treatment improve port-wine stain clearance while minimizing epidermal damage.

BACKGROUND: When a cryogen spurt is applied to the skin surface for an appropriately short period of time (on the order of tens of milliseconds), the cooling remains localized in the epidermis, while leaving the temperature of the deeper port-wine stain (PWS) blood vessels unchanged. OBJECTIVE: The objective of this study was to compare the efficacy and safety of noncooled laser treatment (NC-LT), and cryogen spray cooled laser treatment (CSC-LT) of PWS birthmarks in a large series of patients. METHODS: A retrospective review was conducted of 196 patients with head or neck PWS birthmarks treated with the pulsed dye laser (lambda = 585 nm; taup = 450 microseconds) over a 7-year period. Subjects' ages ranged between 2 months and 62 years; there were 109 females and 87 males, all of whom were Asian. Ninety-eight patients received NC-LT using light dosages of 5-7 J/cm2. Subsequently, 98 patients received CSC-LT using light dosages of 8-10 J/cm2. The primary efficacy measure was the quantitative assessment of the blanching response scores of NC-LT PWSs as compared, on a blinded basis, to CSC-LT PWSs. RESULTS: Based on chi-squared analysis, there were clinical, and statistically significant, differences in the blanching response scores favoring PWS receiving CSC-LT as compared to the NC-LT group (P <.001). Permanent scarring was noted in 3.1% (n = 3) of the patients in the NC-LT group. Permanent scarring was not observed in the CSC-LT treatment group. Transient hyperpigmentation was noted in 57% (n = 56) and 48% (n = 47) of the patients in the NC-LT and CSC-LT groups, respectively. In both groups, the transient hyperpigmentation resolved in all patients within 1 year. Two patients in the NC-LT group developed delayed permanent hypopigmentation. Permanent hypopigmentation was not observed in the CSC-LT group. CONCLUSION: CSC permitted the use of higher incident light dosages leading to improved PWS clearance without producing complications such as permanent scarring or dyspigmentation.

Adolescent↗

Medium-depth chemical peels in the treatment of acne scars in dark-skinned individuals.

BACKGROUND: Multiple modalities are available for treating acne scars, one of which is chemical peeling. OBJECTIVE: To evaluate the efficacy of medium-depth peels in the treatment of acne scars. METHODS: A total of 15 patients (14 women and 1 man) were seen between November 1998 and March 2000. A medium-depth chemical peel was performed. The peel was performed using a combination of Jessner's solution followed by the application of 35% trichloroacetic acid (TCA). The mean age of patients who entered the study was 28 years. A total of 42 peeling sessions were performed: 13 patients had the full three-session regiment, 1 patient had two sessions, and 1 had only one session. RESULTS: Patients in our study had two forms of acne scars, the atrophic saucer or crater-like form and the pitted (ice-pick) form. Improvement occurred in all except one of our patients. Significant improvement (greater than 75% clearance of lesions) occurred in 1 patient (6.6%), moderate improvement (51-75% clearance) in 8 patients (53.3%), mild improvement (26-50% clearance) in 4 patients (26.6%), minimal improvement (1-25% clearance) in 1 patient (6.6%), and no response in 1 patient (6.6%). All patients had different combinations of the above two forms. Four patients (26.6%) had mainly pitted scars and deep atrophic scars. The clinical response in those patients was moderate, mild, minimal, and no response, respectively. The remainder of our patients had mainly atrophic scars of moderate depth. Nine patients (73.4%) suffered from transient postinflammatory hyperpigmentation. In two of them it was preceded by erythema that lasted for more than 1 month. All patients were free of noticeable pigmentation 3 months after the final peel. Patients in whom hyperpigmentation did not develop were of light brown complexion. CONCLUSION: We conclude that medium-depth chemical peel is a safe and effective method of treating acne scars even in patients with dark complexion.

Acne Vulgaris↗

Clinical application of lasers in Asians.

BACKGROUND: Laser surgery for Asians differs from that for Caucasians in several important respects. In Asians, some conditions such as nevus of Ota are frequently seen and certain adverse reactions, especially postinflammatory hyperpigmentation, tend to be more common. OBJECTIVE: This article reviews the use of different types of lasers and intense pulsed light (IPL) sources for the treatment of Asian patients. METHODS: Various cutaneous conditions amenable to laser treatment, including lentigines, nevus of Ota, acquired bilateral nevus of Ota-like macules, port-wine stains, and acne scarring, are discussed. Strategies for the management of postinflammatory hyperpigmentation are offered. RESULTS: Appropriate selection and careful planning of the treatment can lead to excellent clinical outcome. CONCLUSION: Lasers and intense pulsed light sources are important tools for the treatment of a wide range of cutaneous conditions in Asians.

Asian People↗

Sensitization to para-phenylenediamine from a streetside temporary tattoo.

"Temporary" henna tattoos (skin painting or pseudotattooing) are in vogue among American and European youngsters, particularly when vacationing. A 17-year-old girl presented with a severe contact dermatitis of her scalp and face after having dyed her hair with a permanent oxidative hair dye. She denied previous use of oxidative hair dye. Eight months earlier she had a "temporary" henna tattoo applied on her shoulder by a transient artist in downtown Montreal and developed an acute, erythematous, edematous eruption that resolved with residual, prolonged hyperpigmentation. As henna tattooing is a lengthy and tedious procedure, para-phenylenediamine (PPD) may be added to the mixture to accelerate the process, to darken, and to give more precision to the design. This short-lived fad can have longer-term sequelae then expected, ranging from postinflammatory hyperpigmentation of the tattoo site to permanent sensitization to PPD and related compounds.

Adolescent↗

[Bilateral nevus of the caruncle].

BACKGROUND: Pathologic processes of the caruncle are relatively rare with a predominance of papillomas, pyogenic granulomas and nevi. PATIENT: A 22 year old Caucasian male patient presented with bilateral hyperpigmentations of the caruncle. They had first been observed at an age of seven and showed slight progression in size and colour with time. Examination revealed hyperpigmentation with clear pseudocystic spaces. The tarsal or bulbar conjunctiva was not involved. An excision of both processes was performed. HISTOLOGY: Light microscopy revealed nests of nevus cells with melanin granules and round basophilic nuclei as well as gland ducts and sebaceous glands, beneath and within the multilayered keratinizing and nonkeratinizing squamous epithelium. The sections revealed a bilateral melanocytic compound nevus of the caruncle. DISCUSSION: To our knowledge, there is no report on bilateral nevi of the caruncle among the Caucasian population. The possibility of a common precursor cell during the embryologic development causing the bilaterality is discussed.

Adult↗

[Incontinentia pigmenti (Block-Sulzberger syndrome) associated with congenital retinal fold--a long-term 18-year observation].

Report on a case of incontinentia pigmenti presenting with the main characteristics features: patchy hyperpigmentation of the skin, alopecia and dental anomalies. In addition to the rare hyperpigmentation of the lower eyelids, hemiatrophy of the right face and horizontal nystagmus were found. Eye involvement consisted of a congenital retinal fold with retinal vessels draen into and bordered by pigment clumps.--When the patient with 14 total retinal detachment associated with a totally liquefied vitreous occurred and surgical reattachment failed. As a rarity in this case, the full picture of retrolental fibroplasia was not present; it was possible to follow developments over a period of 18 years.

Alopecia↗

[Pigmented exanthema after spironolactone allergy in progressive systemic scleroderma].

A 37-year-old man developed generalized medium-brown hyperpigmentation in the course of progressive systemic scleroderma. Histologically there were numerous Melanin granules in the basal epidermis, but also in the upper Malpighian layer and in corneocytes. An allergic rash developed on administration of spironolactone given for the scleroderma. After healing there remained slate-grey spots. As a consequence of the drug rash the already present hyperpigmentation was aggravated by marked pigment deposition in the upper corium. At the same time there were numerous melanophages as sign of an absorptive removal of the melanin granules. The drug allergy was thus the occasion for the persisting pigment deposition with circumscribed spotty pigmentation of the skin.

Adult↗

Purification and characterization of an allergy-induced melanogenic stimulating factor in brownish guinea pig skin.

We have demonstrated recently that phenylazonaphthol (PAN) allergy-induced hyperpigmentation in brownish guinea pig skin is associated with the concomitant appearance of a melanogenic soluble factor(s) that activates the intracellular signal transduction system, including phosphatidylinositol turnover subsequent to ligand-receptor binding in cultured guinea pig melanocytes. In this study we have purified and characterized the PAN-induced melanogenic stimulating factor (PIMSF) that occurs in allergy-associated hyperpigmented skin. By successive column chromatography on TSK 2000SW, Mono Q, and octadecyl-NPR, the PIMSF was purified to homogeneity with a single band of apparent molecular mass of 7.9 kDa by sodium dodecyl sulfate-polyacrylamide gel electrophoresis. The specific bioactivity of PIMSF increased by 5,195-fold over the original skin homogenate. In cultured guinea pig melanocytes, this purified PIMSF had the potential of activating an intracellular signal transduction system such as inositol 1,4,5-trisphosphate formation and intracellular calcium levels through a pertussis toxin-sensitive G protein-coupled receptor. PIMSF consistently caused a rapid translocation of cytosolic protein kinase C (PKC) to membrane-bound PKC within 5 min of treatment with a return to the basal level after 120 min. The stimulating effects of PIMSF on proliferation and melanization of cultured guinea pig melanocytes were abolished completely by a PKC down-regulating agent (phorbol 12,13-dibutyrate). PIMSF was similar in molecular mass to rat growth-related oncogene alpha (GRO-alpha; molecular mass of 7.9 kDa) on sodium dodecyl sulfate-polyacrylamide gel electrophoresis and had immunocross-reactivity with GRO-alpha upon Western immune blotting analysis. Further, the stimulatory effect of purified PIMSF on DNA synthesis of cultured guinea pig melanocytes was suppressed markedly by the addition of anti-rat GRO-alpha antibody, implying that the PIMSF is apparently identical to GRO-alpha. These findings suggest that PAN allergy provides a new mechanism of hyperpigmentation in which biological factors such as the GRO-alpha superfamily generated within allergy-induced skin stimulate melanocytes through activation of the PKC-related signal transduction pathway.

Allergens↗

The VECAT study: methodology and statistical power for measurement of age-related macular features. Vitamin E, Cataract, and Age-related Maculopathy Study.

PURPOSES: (1) To develop the methodology for the grading of macular one-frame stereoslides and to assess the reliability of the system. (2) To determine the prevalence of soft drusen (> 63 microm) and pigment abnormalities synonymous with age-related maculopathy (ARM) at baseline, in a clinical trial of volunteers aged between 55 and 80 years of age. (3) To ascertain the power of the study to detect the 4-year incidence and progression of ARM in vitamin E versus placebo treated participants, given the baseline prevalence. METHODS: The 1204 participants enrolled in the Vitamin E, Cataract, and Age-related Maculopathy Study (VECAT) had colour stereoslides of their fundus taken using the Nidek 3-DX mydriatic fundus camera. The stereoslides were graded by two masked graders according to the "International Classification System for ARM and AMD". Assessment of inter- and intra-observer reliability was carried out on a regular basis on 15% of randomly selected slides. Anticipated rates of incidence and progression were based on results reported by the Beaver Dam Eye Study and the Chesapeake Bay Waterman Study. Power estimations were determined using the "nQuery Advisor" software program. Analyses were carried out on the worse affected eye. RESULTS: Inter-observer reliability was moderate to substantial (Kappa 0.5-0.88) whilst intra-observer agreement was high (0.6-1.0). The prevalence of any soft drusen was 32%. Significant associations were found between soft large indistinct drusen, hypopigmentation, hyperpigmentation and age (p = 0.0001, 0.024 and 0.0001, respectively). The study has at least 87% power to detect an odds ratio equal to two for the progression of soft distinct, soft indistinct, hyperpigmentation and hypopigmentation. CONCLUSIONS: The VECAT study methodology appears to be highly reliable and to have sufficient power to detect the differences in the four-year progression of soft distinct and indistinct drusen and pigment abnormalities between the treatment groups.

Aged↗

Follow-up of a girl with cleft lip and palate and multiple malformations: trisomy 20 mosaicism.

We describe a 16(1/2) -year-old girl with multiple anomalies including cleft lip and palate and a normal karyotype in blood lymphocytes. Because of hyperpigmention along the lines of Blaschko, her karyotype in skin fibroblasts was examined, which showed mosaicism for trisomy 20. This is the first report to our knowledge of this karyotype where hyperpigmentation and facial clefting occurred together.

Abnormalities, Multiple↗

Evaluation of three different strengths of trichloroacetic acid in xanthelasma palpebrarum.

BACKGROUND: Xanthelasma is a common cutaneous condition that presents in the periocular region. Essentially benign, treatment is of cosmetic importance. OBJECTIVE: Evaluation of varying strengths of trichloroacetic acid (TCA) in xanthelasma palpebrarum. METHODS: Three strengths of TCA were used on 51 patients randomly after categorizing their xanthelasma into papulo-nodular, flat plaques and macular lesions. The average number of sittings was calculated in each category and patients were reviewed fortnightly. RESULTS: Papulo-nodular lesions required an average of two applications with 100%, 2.67 with 70% and 4.16 with 50% TCA. Flat plaques responded to an average of 1.43, 1.50 and 3.55 sittings with 100%, 70% and 50% TCA, respectively. Macular lesions responded to only one application of all strengths of TCA applied. Eleven patients developed hypopigmentation, five had hyperpigmentation and one developed mild scarring. CONCLUSION: 100% TCA gives the best results in papulo-nodular lesions, 100% or 70% TCA give similar results in flat plaque xanthelasma and in macular lesions 50% is sufficient. Hypopigmentation is the commonest side effect, followed by hyperpigmentation. Scarring is a minor problem.

Adult↗