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Cutaneous adverse reactions to hydroxyurea in patients with intermediate thalassemia.

Although the cutaneous effects of hydroxyurea have been described for patients with sickle cell anemia, myeloproliferative disorders, and psoriasis, there are no reports of cutaneous adverse effects from hydroxyurea when used for patients with intermediate thalassemia. Therefore 43 patients with intermediate thalassemia treated with hydroxyurea were examined by a dermatologist, and pertinent cutaneous findings were recorded. These patients had received hydroxyurea for a mean of 15.5 months. Nineteen had cutaneous hyperpigmentation, eight had xerosis, and three were found to have one cafe au lait macule each. Eleven patients had nail abnormalities, including nail ridging, partial leukonychia, and longitudinal melanonychia. There were no cases of leg ulceration. It was concluded that the risk of developing leg ulcers and pigmentary disorders appears to be related to the underlying disease being treated, as well as to a patient's age, gender, and pigmentation.

Adolescent↗

Postnatal confirmation of prenatally diagnosed trisomy 20 mosaicism in a patient with linear and whorled nevoid hypermelanosis.

Linear and whorled nevoid hypermelanosis (LWNH) is characterized by hyperpigmented reticulate macules in a Blaschko linear arrangement without atrophy or preceding inflammation. Underlying chromosomal mosaicism was often assumed, but has been verified in only a few published cases. We report a 7-year-old boy with LWNH associated with congenital ventricular septal defect and psychomotor retardation. Prenatal chromosomal analysis of amniocytes revealed trisomy 20 mosaicism, which was not confirmed in peripheral blood lymphocytes after birth. Histologic sections of skin biopsy specimens taken at age 6 years showed hyperpigmentation of the basal epidermal layer with prominent melanocytes and isolated melanophages in the upper dermis. Cytogenetic analysis of cultured skin fibroblasts revealed an extra chromosome 20 in 5 of the 30 metaphases studied (17%). Mosaic trisomy 20 is one of the most common autosomal mosaicisms identified in amniocytes and is, as a rule, compatible with normal pregnancy outcome. In postnatal analysis of peripheral blood lymphocytes, an extra chromosome 20 could never be detected. However, when confirmed in skin fibroblasts, trisomy 20 mosaicism may be associated with systemic anomalies. The present case shows for the first time an association of LWNH with trisomy 20 mosaicism and emphasizes the importance of analyzing skin fibroblasts in cases of prenatally diagnosed trisomy 20.

Abnormalities, Multiple↗

Odontotrichodysplasia: a case report with a review of conditions combining ectodermal dysplasia (subgroup 1-2) with skin manifestations.

We report a 5-year-old girl with partial anodontia, hypotrichosis, hyperpigmentation of the skin, absence of pilosebaceous structures, and long thin fingers. There has as yet been, to the best of our knowledge, no report of such a combination of features. A review of conditions combining ectodermal dysplasia (subgroup 1-2) with skin manifestations is presented.

Anodontia↗

A new intralesional therapy of cutaneous leishmaniasis with hypertonic sodium chloride solution.

One hundred and fifty-eight lesions of acute cutaneous leishmaniasis in 70 patients were treated with hypertonic sodium chloride solution "HSCS" (88 lesions) or sodium stibogluconate (50 lesions); 20 lesions were left untreated as controls. The injections were given at 7-10 day intervals, and patients were followed-up for 42 days. "HSCS" was shown to be a very effective local therapy (96.05% cure rate) and was as effective as local sodium stibogluconate (96.42% cure rate). With both types of therapy, most lesions needed only one injection. Mild improvement was noticed 7-10 days after the first injection, and the cure was complete within 2-6 weeks (mean 4 weeks) of follow-up. None of the control lesions showed a cure within the six weeks follow-up. The mechanisms of action of both "HSCS" and sodium stibogluconate probably involve interference with the osmotic pressure of the cell cytoplasm of the parasites and lesional tissues. Scarring was either absent or minimal following healing of the treated lesions with both types of treatment. Post-inflammatory hyperpigmentation was observed in all patients. We strongly recommend intralesional "HSCS" as a cheap, safe, and effective local method for treating cutaneous leishmaniasis.

Adolescent↗

A case of dermatopathia pigmentosa reticularis.

Dermatopathia pigmentosa reticularis (DPR) is a very rare disorder with the diagnostic triad of generalized reticulate hyperpigmentation, noncicatricial alopecia, and onychodystrophy. Many other dermatologic findings have been associated with this triad, including adermatoglyphia, hypohidrosis or hyperhidrosis, palmoplantar hyperkeratosis, and nonscarring blisters on the dorsa of the hands and feet. The mode of inheritance is unclear but may be autosomally dominant. To our knowledge, only 11 cases have been reported in the world, and none has previously been described in the Orient. We present a Korean patient with the typical features of the DPR triad, along with adermatoglyphia, hypohidrosis, and nonscarring blisters on the dorsa of the feet.

Adult↗

Microscopically controlled surgical excision combined with ultrapulse CO2 vaporization in the management of a patient with the nevoid basal cell carcinoma syndrome.

Nevoid basal cell carcinoma syndrome is an autosomal dominant condition characterized by multiple basal cell carcinomas, skeletal abnormalities and sometimes mental retardation. The large number of tumors, which are often disfiguring, presents extreme difficulties in the treatment of these patients. Microscopically controlled excision, compared to other modalities (radiation therapy, photodynamic therapy, intralesional interferon alpha-2b) offers the highest cure rate. However, because of the large size and involvement of wide areas of the skin, this approach is sometimes impractical. The ultrapulse CO2 laser with high energy and short pulses achieves char-free ablation of the tumors, bloodless surgical field, minimal nonspecific thermal damage, rapid healing and diminished postoperative pain. Also, a number of lesions can be removed in a single session. We present a 48-year-old man with a 6.5 x 4.5 cm large basal cell carcinoma involving the anterior abdomen and navel area. The central thick portion of the tumor was resected by microscopically controlled excision with 3 stages, and wide thinner peripheral crescentic plaque vaporized with ultrapulse CO2 laser. The laser settings were 300 mJ energy/pulse and 100 W average power, which corresponds to the fluence of 7.5 J/cm2. Computerized pattern generator (ultrascan handpiece) was adjusted to patterns of 3 (circle) and 1 (square) with sizes varying from 5 to 7, and density of 9 (60% overlapping). The tumor was vaporized with 6 passes, all the way to deep reticular dermis. A fifteen month-follow up disclosed no recurrent disease. Subsequent biopsies revealed only a scar with postinflammatory hyperpigmentation. Our experience indicates that combined treatment with microscopically controlled excision and ultrapulse CO2 laser ablation is a suitable modality for the large tumor plaques involving concave and convex areas of the skin respectively. Microscopically controlled excision of thicker, concave portions of basal cell carcinoma plaques, where CO2 laser surgery is less feasible, presents an effective addition that renders this combined modality a successful method for the treatment of nevoid basal cell carcinoma syndrome.

Abdominal Neoplasms↗

A case of Dowling-Degos disease suggesting an evolutional sequence.

We report a 47-year-old woman who presented with asymptomatic reticulate hyperpigmentations on the neck, lateral face, axillae, trunk, inguinal areas, and dorsa of both hands and feet. We thought it was an unusual case in the spectrum between the pole of Dowling-Degos disease (DDD) and that of reticulate acropigmentation of Kitamura (RAK). Another interesting point was that the biopsied specimens from the abdomen, neck, and axillary lesions showed somewhat different histopathologic features from typical DDD, suggesting an evolutional sequence. From these findings we suggest that a lichenoid inflammation may be responsible for the typical maculo-papular lesions of DDD.

Asian People↗

Laugier-Hunziker syndrome.

Laugier-Hunziker syndrome is a benign pigmentary disorder which manifests as macular hyperpigmentation of the lips and buccal mucosa. Some patients have longitudinal pigmented bands of nails. The syndrome has no systemic associations. Two patients of this rare syndrome are reported. Disorders producing similar pigmentary changes which must be differentiated are discussed.

Adolescent↗

Histological effects of tazarotene 0.1% cream vs. vehicle on photodamaged skin: a 6-month, multicentre, double-blind, randomized, vehicle-controlled study in patients with photodamaged facial skin.

BACKGROUND: Topical tazarotene has been shown to offer efficacy in ameliorating multiple effects of photodamage. OBJECTIVES: To evaluate the histological effects of tazarotene cream on photodamaged skin. METHODS: In this multicentre, double-blind, randomized, vehicle-controlled study, 50 patients with photodamaged facial skin (at least mild fine wrinkling and mottled hyperpigmentation, with at least one of these being moderate) were randomized to apply tazarotene 0.1% cream or vehicle cream to their face, once daily for 24 weeks. RESULTS: Blinded assessments showed that tazarotene was less likely than vehicle to be associated with an increase in keratinocytic and melanocytic atypia, and more likely than vehicle to be associated with a reduction in atypia. Between-group comparisons in distribution of change from baseline categories of severity were in favour of tazarotene (P = 0.055 for keratinocytic atypia, P = 0.034 for melanocytic atypia, and P < 0.001 for the number of granular cell layers). Compared with vehicle, tazarotene was associated with an increase in epidermal polarity (P = 0.008) and epidermal thickness (P = 0.012), and a tendency for stratum corneum compaction. Tazarotene was also associated with widened intercellular spaces (reported as epidermal oedema) relative to vehicle (P < 0.001). CONCLUSIONS: Treatment of photodamaged skin with tazarotene is associated with an amelioration of keratinocytic and melanocytic atypia, an improvement in epidermal polarity, and an increase in epidermal thickness.

Adult↗

Controlled study of excimer and pulsed dye lasers in the treatment of psoriasis.

BACKGROUND AND OBJECTIVES: The excimer laser delivers high energy monochromatic ultraviolet (UV) B at 308 nm. Advantages over conventional UV sources include targeting of lesional skin, reducing cumulative dose and inducing faster clearance. Studies of the pulsed dye laser (PDL) in psoriasis report between 57% and 82% response rates; remission may extend to 15 months. To our knowledge, this is the first study assessing both excimer and PDL in psoriasis. METHODS: We conducted a within-patient controlled prospective trial of treatment of localized plaque psoriasis. Twenty-two adult patients, mean Psoriasis Area and Severity Index 7.1, were recruited. Fifteen patients completed the full treatment, of which 13 were followed up to 1 year. Two selected plaques were treated with excimer twice weekly and V Beam PDL, pretreated with salicylic acid (SA), every 4 weeks, respectively. Two additional plaques, treated with SA alone or untreated, served as controls. The primary outcome measures were: (i) changes in plaque-modified Psoriasis Activity and Severity Index (PSI) scores from baseline to end of treatment; (ii) clinical response to treatment (CR(T)), assessed by serial photographs; (iii) percentage of plaques clear at the end of treatment; and (iv) percentage of plaques clear at 1-year follow-up. The secondary outcome measures were: (i) number of laser treatments to clearance; (ii) time to relapse; (iii) frequency of side-effects; and (iv) qualitative observations with SIAscope. RESULTS: The mean improvement in PSI was 4.7 (SD 2.1) with excimer and 2.7 (SD 2.4) with PDL. PSI improvement was significantly greater in excimer than PDL (P = 0.003) or both control plaques (P < 0.001). CR(T) indicated 13 patients responded best with excimer, two patients best with PDL, and in seven patients there was no difference between the two lasers. CR(T) was significantly greater for excimer than PDL (P = 0.003) or both controls (P < 0.001). CR(T) was also significantly greater for PDL than SA alone (P = 0.004) or untreated control (P =0.002). Nine (41%) patients cleared with excimer, after mean 8.7, median 10 weeks treatment. Seven of these nine patients were followed up to 1 year; four remained clear, two relapsed at 1 month, and one at 6 months. Six (27%) patients cleared with PDL, after mean 3.3, median four treatments. All six patients were followed up to 1 year; four remained clear, one relapsed at 4 months and one at 9 months. Despite common side-effects including blistering and hyperpigmentation, patient satisfaction was high. Serial images obtained with the SIAscope during treatment indicated different mechanisms of action of the two lasers. CONCLUSIONS: Excimer and V Beam PDL are useful treatments for plaque psoriasis. Although the excimer appears to be on average more efficacious, a subset of patients may respond better to PDL. Long-term remission is achievable with both lasers.

Adult↗

Skin manifestations in a case of trisomy 16 mosaicism.

We present a 48-year-old man with unilateral dermatological manifestations including hypertrichosis, telangiectasia, hyperkeratosis and hyperpigmentation. Additional findings included skeletal abnormalities and left-sided hearing loss. Skin biopsies showed changes characteristic of porokeratosis. Fibroblast karyotyping from affected skin demonstrated trisomy 16 mosaicism, in contrast to the normal karyotype in unaffected skin and blood lymphocytes. The possible role of trisomy 16 in porokeratosis is discussed.

Abnormalities, Multiple↗

The clinical appearance of clonal nevi (inverted type A nevi).

BACKGROUND: The term "clonal nevus" is used to describe a variant of melanocytic nevus that histologically exhibits a localized proliferation of pigmented epithelioid dermal melanocytes within an otherwise ordinary nevus (Ball NJ, Golitz LE. Melanocytic nevi with focal atypical epithelioid cell components: a review of seventy-three cases. J Am Acad Dermatol 1994; 30: 724-729). Reports to date have focused on the histologic appearance of these lesions. AIM: To characterize the clinical appearance of clonal nevi. METHODS: Clinical and histologic examinations were performed of a single clonal nevus from each of five patients (two men and three women; age range, 37-80 years). RESULTS: All nevi were round to oval in shape with smooth, well-defined borders. They were uniformly tan to light brown in color, except for a single blue-gray to blue-black focus of hyperpigmentation. The diameters of the nevi ranged from 2.5 to 10 mm. In individual nevi, the hyperpigmented focus was either centrally or eccentrically located and measured 1-2 mm in diameter. Histologically, these lesions showed banal melanocytes associated with a localized proliferation of melanocytes with abundant pigmented cytoplasm in the dermis, admixed with melanophages. CONCLUSIONS: The appearance of clonal nevi--tan with a focus of blue-gray to blue-black pigmentation--allows one to recognize the lesion clinically.

Adult↗

Hair removal using an 800-nm diode laser: comparison at different treatment intervals of 45, 60, and 90 days.

BACKGROUND: Some laser irradiation parameters such as wavelength, fluence, pulse duration, and spot size have been shown to influence the damage of any target inside the skin, however, the role of some patients' factors such as hair growth cycle is still under debate. OBJECTIVE: To determine the association of treatment interval and laser treatment outcome. METHODS: In a retrospective chart review of 176 patients undergoing laser-assisted hair removal with a diode laser 24 patients were selected. All accepted to cease the therapy, and be followed-up for 5 months. At the end of the study the patients were questioned about the efficacy of the treatment as well as the adverse effects. Hair counting was also performed. The adverse effects (pain, blister or erosion, hyperpigmentation, hypopigmentation, and folliculitis) were questioned during the follow-up period. RESULTS: The mean hair reduction was 78.1%, 45.8%, and 28.7% in 45, 60, and 90-day interval groups, respectively (P < 0.0001). CONCLUSION: The treatment interval was related to the treatment outcome in our study.

Adult↗

Treatment of keloids and hypertrophic scars with dermojet injections of bleomycin: a preliminary study.

BACKGROUND: Numerous treatment modalities have been used to treat keloids and hypertrophic scars, but the optimal treatment has not been established. OBJECTIVE: The aim of this study was to determine the efficacy and safety of intralesional jet injection of bleomycin as therapy for keloids and hypertrophic scars that are unresponsive to intralesional steroid injection. METHODS: The study included 14 patients with 15 keloids or hypertrophic scars that had not responded to a minimum of three intralesional injections of triamcinolone acetonide. Multiple jet injections of 0.1 ml of bleomycin (1.5 IU/ml) were administered to each lesion, with injection sites spaced 0.5 mm apart. Injections were repeated each month. Scar height was measured, and scar pliability and erythema were scored at baseline and then monthly during the treatment and follow-up periods. Patients' self-assessments of subjective symptoms (pruritus and pain) were also scored. Clinical improvement was defined primarily on the basis of scar height reduction (percentage reduction from baseline), and was classified using the following scale: complete flattening (100%), highly significant flattening (> 90%), significant flattening (75-90%), moderate flattening (50-75%), and minimal flattening (< 50%). Pre- and post-treatment mean values for scar height, scar pliability, erythema, pruritus and pain were statistically compared. RESULTS: The number of sessions required to successfully treat the lesions ranged from two to six. Eleven lesions (73.3%) showed complete flattening, one (6.7%) showed highly significant flattening, two (13.3%) showed significant flattening, and one scar (6.7%) showed moderate flattening. The mean scar height was significantly lower, and the mean scores for scar pliability and erythema were significantly better at the end of treatment (P < 0.001, P < 0.001 and P < 0.001, respectively). The mean scores for pruritus and pain also improved significantly (P < 0.001 and P = 0.01, respectively). The observed side-effects were hyperpigmentation (four lesions) and skin atrophy (three lesions). No recurrences were noted during follow up (mean duration of 19 months). CONCLUSIONS: Intralesional jet injection of bleomycin is an effective and safe method of treating keloids and hypertrophic scars that are unresponsive to intralesional steroid therapy.

Adolescent↗

Prurigo pigmentosa: not an uncommon disease in the Turkish population.

BACKGROUND: Prurigo pigmentosa (PP) is a rare inflammatory dermatosis of unknown etiology. It is characterized by erythematous papules of the trunk that resolve leaving a reticulate hyperpigmentation. The original report from Japan in 1971 was followed by new cases, mostly of Japanese origin. METHODS: We scrutinized PP cases diagnosed in our clinic and reviewed the English, German, French, and Turkish language literature. RESULTS: We report six additional Turkish patients. We found more than 40 cases reported from various countries outside Japan. The patients had different racial origins. The number of Turkish patients reported in recent years is higher than the number of other non-Japanese patients reported. CONCLUSIONS: The disease is not uncommon in the Turkish population. It may be underestimated in other countries with Caucasian populations. Therapeutic results with tetracycline and doxycycline was successful in our patients.

Adolescent↗

The clinical use of alpha hydroxy acids.

Alpha Hydroxy Acids are a group of organic acids which have recently become popular in the treatment of several skin conditions. This article explores some of the clinical uses of these products in the treatment of ichthyosis, acne, hyperpigmentation and photo-damage. Specific recommendations are given for the treatment of each condition.

Acne Vulgaris↗

Treatment of cutaneous leishmaniasis with 20% paromomycin ointment.

Cutaneous leishmaniasis is an infectious disease caused by flagellate protozoa of the genus Leishmania. In Mediterranean countries, the most common causative agents are Leishmania (L.) major, L. infantum and L. tropica. In Croatia, cutaneous leishmaniasis is a rare disease, the last case being reported in 1988. Our patient was a 5-year-old boy with a left cheek skin lesion in the form of papule with central exulceration, hyperkeratotic crust and erythema of a 6-month duration. The diagnosis of cutaneous leishmaniasis was based on history data (stay in the southernmost region of Croatia and multiple mosquito bites), light microscopic histology (dense infiltrates of large histiocytes with extracellular bodies), and positive Montenegro (leishmanin) test. A new therapy with aminosidine (paromomycin), an aminoglycoside antibiotic, in the form of ointment at a concentration of 20%, was for the first time used in Croatia. Four-week therapy resulted in complete regression of the skin lesions with residual hyperpigmentation. During therapy, no local or systemic side effects were observed. Thus, topical therapy with paromomycin could be considered an efficient therapeutic alternative in the management of cutaneous leishmaniasis.

Administration, Cutaneous↗

The safety of hydroquinone.

Hydroquinone is one of the most effective molecules for the treatment of hyperpigmentary disorders, with over 40 years of efficacy and safety data. Concerns over its safety have been raised because of the fact that it is a derivative of benzene and because of the long-term side-effects observed with cosmetic products containing high concentrations of hydroquinone. However, despite 40-50 years use of hydroquinone for medical conditions, there has not been a single documented case of either a cutaneous or internal malignancy associated with this drug. This article reviews the evidence for the safety of hydroquinone in the treatment of hyperpigmentation conditions.

Animals↗