Percutaneous ethanol sclerotherapy is a promising treatment for recalcitrant angiolymphoid hyperplasia with eosinophilia.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare benign vascular proliferation, which manifests as characteristic red nodules and papules, mostly located on the scalp and periauricular regions. Patients seek treatment for both aesthetic and functional reasons, as lesions may ulcerate, bleed and itch. Many therapeutic approaches have been reported, with variable success, and relapse remains a troublesome issue. The aim of this study was to report our experience treating ALHE using percutaneous ethanol sclerotherapy (PES). We present a retrospective case series of three patients treated with PES (1-2 treatment sessions each). All patients had tried and failed other treatments prior to this intervention, but following PES treatment, all patients demonstrated significant improvement, which was sustained at follow-up (range 8-17 months after first treatment). Adverse effects were tolerable and transient. This case series demonstrates PES as a promising treatment for recalcitrant ALHE.

Epidemiology of cutaneous porphyria in Israel: a nationwide cohort study.

BACKGROUND: From a dermatologist's perspective, there are four major types of cutaneous porphyrias (CPs): porphyria cutanea tarda (PCT), erythropoietic protoporphyria (EPP), variegate porphyria (VP) and hereditary coproporphyria (HCP). Scarce data are available regarding the epidemiology of CPs. OBJECTIVES: To describe the epidemiology of CPs in Israel, including distribution, incidence and prevalence rates of major types. METHODS: This retrospective study includes all patients who were diagnosed with CPs between the years 1988-2018. It is based on data from Israel's National Service for the Biochemical Diagnoses of Porphyrias, and Israeli patients' nationwide electronic medical charts. Incidence and prevalence rates were calculated. RESULTS: Of 173 patients with CPs diagnosed during a 30-year period, 65 (38%) had VP, 62 (36%) had PCT, 31 (18%) had HCP and 15 (9%) had EPP; with incidence rates of 0.29, 0.30, 0.17, 0.07, and prevalence rates of 6.3, 4.8, 2.9, 1.6, respectively, per million population. Characteristics of patients with PCT differed from those with other CPs with regard to lack of family history, older mean age at diagnosis [51 vs. 36 (VP), 35 (HCP) and 25 (EPP) years] and male predominance (81% vs. similar distribution). All patients with PCT were diagnosed at adulthood, while 20%, 19% and 15% of patients with VP, HCP and EPP, respectively, were diagnosed during childhood or adolescence. CONCLUSIONS: Variegate porphyria and PCT were found to be the most prevalent in Israel; however, CPs might be underdiagnosed, thus dermatologists' awareness of these rare disorders is highly important.

Real-life experience in the treatment of solar urticaria: retrospective cohort study.

BACKGROUND: Solar urticaria (SU) is a rare photodermatosis causing a significant impact on patients' quality of life (QoL), and treatment is often challenging. AIM: To analyse clinical experience with a tailored stepwise therapeutic approach. METHODS: A retrospective cohort design was used. Patients with suspected SU underwent laboratory investigations and photoprovocation. Those with a high minimal urticaria dose (MUD) were treated with a single antihistamine (protocol 1), and those with a lower MUD received three types of antihistamines (protocol 2); both protocols included a leucotriene receptor antagonist (LRA). In cases of failure, treatment was switched to omalizumab at doses of < 300 mg/month with incremental dosage increases as necessary (monthly dose range, 150-600 mg/month). Symptom relief and photoprovocation under treatment were evaluated. RESULTS: In total, 30 patients (10 men, 20 women) were enrolled. Most (87%) were sensitive to visible light (1-70 J/cm2 ) with or without extension to ultraviolet A. Of the 30 patients, 23 opted for our stepwise approach: 22 achieved complete remission on protocols 1 or 2 (n = 17) or after switching to omalizumab (n = 5), and another patient achieved partial remission under omalizumab. There were no treatment-related severe adverse effects. CONCLUSIONS: Symptoms of SU can be well controlled by treatment with antihistamines and an LRA tailored to the degree of photosensitivity, followed by omalizumab in refractory cases. This has important implications for patient QoL.

Safety profile during initiation of propranolol for treatment of infantile haemangiomas in an ambulatory day-care hospitalization setting.

BACKGROUND: Propranolol is the mainstay of treatment for infantile haemangioma. Despite its good safety profile, it is not risk-free. Guidelines for propranolol initiation and monitoring have been suggested, but protocols vary among practitioners. OBJECTIVE: This study sought to assess the prevalence of adverse events and clinically significant fluctuations in haemodynamic parameters in children with infantile haemangioma during initiation of treatment with propranolol in a day-hospitalization setting. METHODS: Children with infantile haemangioma treated with propranolol in a day-hospitalization department of a tertiary paediatric medical centre in 2008-2014 were identified retrospectively. The pretreatment evaluation included clinical examination by a paediatric dermatologist and electrocardiography, echocardiography and clinical examination by a paediatric cardiologist. The propranolol dosage was escalated from 0.5 mg/kg/day to 2 mg/kg/day, divided into three doses/day, over 3 days. Heart rate, blood pressure and blood glucose level were measured before treatment onset and 60 min after the first two doses each day. The third dose was given at home. RESULTS: The cohort included 220 children aged 1 month to 5 years. No severe treatment-related adverse events were documented; 27 patients had minor side-effects. There was a significant decrease in heart rate each day after the first two doses (P < 0.001), and in systolic blood pressure, on day 2 (1 mg/kg/day) after the first dose (P = 0.01). Blood glucose level remained stable. The haemodynamic changes were clinically asymptomatic and did not require intervention. CONCLUSIONS: Propranolol treatment (2 mg/kg/day in three doses) for infantile haemangioma is well tolerated and safe and may be administered and monitored in an ambulatory setting.

Integrated cooling-vacuum-assisted 1540-nm erbium:glass laser is effective in treating mild-to-moderate acne vulgaris.

Acne treatment by a mid-infrared laser may be unsatisfactory due to deeply situated acne-affected sebaceous glands which serve as its target. Skin manipulation by vacuum and contact cooling may improve laser-skin interaction, reduce pain sensation, and increase overall safety and efficacy. To evaluate the safety and efficacy of acne treatment using an integrated cooling-vacuum-assisted 1540-nm erbium:glass laser, a prospective interventional study was conducted. It included 12 patients (seven men and five women) suffering from mild-to-moderate acne vulgaris. The device utilizes a mid-infrared 1540-nm laser (Alma Lasers Ltd. Caesarea, Israel), which is integrated with combined cooling-vacuum-assisted technology. An acne lesion is initially manipulated upon contact by a vacuum-cooling-assisted tip, followed by three to four stacked laser pulses (500-600 mJ, 4 mm spot size, and frequency of 2 Hz). Patients underwent four to six treatment sessions with a 2-week interval and were followed-up 1 and 3 months after the last treatment. Clinical photographs were taken by high-resolution digital camera before and after treatment. Clinical evaluation was performed by two independent dermatologists, and results were graded on a scale of 0 (exacerbation) to 4 (76-100 % improvement). Patients' and physicians' satisfaction was also recorded. Pain perception and adverse effects were evaluated as well. All patients demonstrated a moderate to significant improvement (average score of 3.6 and 2.0 within 1 and 3 months, respectively, following last treatment session). No side effects, besides a transient erythema, were observed. Cooling-vacuum-assisted 1540-nm laser is safe and effective for the treatment of acne vulgaris.

Noninvasive in vivo confocal laser scanning microscopy is effective in differentiating allergic from nonallergic equivocal patch test reactions.

Patch testing is the gold standard for the validation of contact dermatitis. It relies on the subjective scoring by an evaluator of the inflammatory reaction induced by an allergen applied to the skin. Equivocal reactions imply faint erythema and could represent allergic, irritant, or negative reactions. They constitute approximately 1 % of the positive reactions encountered in patch test practice. Histological evaluation of the equivocal reaction has proven helpful for the correct interpretation but is however time consuming, and its invasive nature is often unacceptable to the patient. In vivo confocal laser scanning microscopy (CLSM) is a novel, noninvasive imaging technique which permits real-time visualization of skin structures and lesions at a resolution close to that obtained by conventional histology. CLSM has been successfully applied for the differentiation between clinically clear-cut allergic and irritant patch test reactions. The objective of this study is to determine the relevance of CLSM in differentiating between allergic, irritant, and negative equivocal patch test reactions. Fifteen patients who underwent patch testing in our clinic were observed as having 20 equivocal reactions. All 20 reactions were evaluated using in vivo CLSM and compared with adjacent normal skin. In vivo CLSM evaluation revealed that 8 of the 20 equivocal reactions (40 %) showed confocal patterns consistent with the patterns encountered in positive allergic reactions. Anamnestic exposure, i.e., detailed assessment of previous related contact with these allergens, confirmed high relevance rates. In vivo CLSM is useful in differentiating between allergic, irritant, and negative equivocal patch test reactions, a differentiation that cannot be made by conventional clinical patch test reading.

Hypertrichosis in Becker's nevus: effective low-fluence laser hair removal.

Becker's nevus is cosmetically bothersome both due to the hyperpigmentation and due to the hypertrichosis which can accompany it, particularly in males. Laser hair removal can be considered, but the pigmented background of the Becker's nevus makes the treatment more challenging. Fifteen patients with Becker's nevus underwent eight sessions of hair removal with low-fluence high-repetition-rate diode lasers (808-810 nm). All participants experienced significant hair reduction at 6 and 12 months. No adverse events were reported. The study supports the use of low fluence with high-repetition-rate diode laser hair removal as a safe and effective method for the management of hypertrichosis in Becker's nevus.

Treatment of verrucous epidermal nevus: experience with 71 cases.

OBJECTIVE: The aim of this study was to evaluate the clinical results of 71 cases with verrucous epidermal nevus (VEN) treated by cryotherapy or CO2 laser. METHODS: The files and photographs of patients with VEN who were treated in the laser unit of a tertiary hospital between January 2005 and December 2011 were evaluated. Pretreatment and 12-month follow-up photographs were evaluated using a 5-point scale: 'excellent' (75-100% clearance), 'good' (50-75% clearance), 'fair' (25-50% clearance), 'poor' (<25% clearance) or 'worse'. RESULTS: Of 71 patients, 62 responded well to cryotherapy alone, and 9 facial VEN required CO2 laser treatment. Small VEN required relatively few treatments (mean 3.4) with 90% scoring 'excellent' and 10% scoring 'good'. Larger VEN required more treatments (mean 7.4) and did not respond as well, with 71% scoring 'excellent', 14% scoring 'good' and 14% scoring 'fair'. CONCLUSION: Our experience suggests that cryotherapy is the ideal treatment for small facial VEN, while ablation by CO2 laser should be considered an option for resistant facial lesions.

Effects of 420-nm intense pulsed light in an acne animal model.

BACKGROUND: Blue light in the 400-420 nm range has been shown to reduce the levels of Propionibacterium acnes (P. acnes) in the skin. P. acnes has been postulated to be a critical trigger for inflammatory acne. Thus, treatment with 420 nm-intense pulsed light should reduce inflammatory activity in acne. AIM: To evaluate the clinical and histological effects of 420 nm-intense pulsed light treatment on acne in animal model. METHOD: Inflammation acne animal model was constructed by intradermal injection of P. acnes of rat auricular. Levels of tumour necrosis factor alpha (TNF-α) and matrix metalloproteinase 2 (MMP-2), markers of inflammation implicated in acne, were assessed in treated and untreated animals by immunohistochemistry and quantitative polymerase chain reaction (PCR). RESULT: Treatment with 420 nm intense pulsed light led to marked improvement after 6 biweekly treatments. Immunohistochemistry and PCR showed that TNF-α and MMP-2 levels correlated with the extent of acneiform activity and were reduced by treatment with 420 nm light. CONCLUSION: A 420-nm intense pulsed light may exert its beneficial effects on inflammatory acne by reducing the levels of P. acnes and secondarily reducing inflammation induced by the bacteria.

Are port wines stains a feature of tuberous sclerosis?

BACKGROUND: Tuberous sclerosis complex is a multisystem inherited disorder characterized by the development of tumour-like growths in brain, skin and other organs. Although cutaneous vascular anomalies are not considered a common manifestation, we have encountered co-occurrence of port wine stains and tuberous sclerosis. OBJECTIVE: To assess the prevalence of port wine stain in patients with previously diagnosed tuberous sclerosis. METHODS: All cases diagnosed with tuberous sclerosis at two tertiary care centres from 2000 to 2009 were reviewed. Cases with clinically documented port wine stains were included for evaluation. RESULTS: Of 24 patients diagnosed with tuberous sclerosis, three (12.5%) had clinically evident port wine stains. The prevalence of port wine stains in this series of tuberous sclerosis patients was significantly higher than the 0.3% prevalence of port wine stain in the general population. CONCLUSION: Port wine stain rate in this population was significantly greater than the expected rate. Further studies are needed to assess the frequency of port wine stains in tuberous sclerosis and to clarify whether the finding should be added to the list of cutaneous features of tuberous sclerosis.

Best practice options for hair removal in patients with unwanted facial hair using combination therapy with laser: guidelines drawn up by an expert working group.

Hirsutism is a common disorder affecting between 5 and 15% of the population. One of the most devastating consequences of hirsutism is the presence of unwanted facial hair. Treatment of hirsutism involves a two-pronged approach: treating the underlying cause and reduction of visible hair. Laser hair removal is one of the most effective options for reducing visible hair, however, it may not be wholly effective in all patients and combination therapy may need to be considered. Pharmacological therapy is often used in combination with mechanical hair removal due to the time needed for the drug treatment to demonstrate visible results. Clinical data investigating the use of laser treatment in combination with other treatments has focused on laser with topical eflornithine. The expert working group reviews existing data and provides guidance on the use of eflornithine in combination with laser for resistant hirsutism.

Targeted UVB phototherapy for psoriasis: a preliminary study.

Excimer laser treatment for psoriasis has been associated with good results at a lower cumulative dose than narrowband ultraviolet (UV)B protocols. To examine the clinical performance of a new targeted UVB lamp (290-320 nm; BClear) in the treatment of plaque-type psoriasis, 28 consecutive patients attending a dermatology service were treated twice weekly with the UVB lamp for 6-18 sessions (median 10). UV doses were based on multiples of a predetermined minimal erythema dose (MED). MEDs ranged from 150 to 350 mJ/cm2; maximal dose was 8 MED. Mean cumulative fluence until remission was 12.63 J/cm2. The Psoriasis Severity Index (PSI) was measured every 2 weeks for 16 weeks. Mean PSI improvement during treatment peaked at 73% after 6 weeks, and declined to 63% at 16 weeks. At that point, 36% of the patients had a > 75% improvement in PSI, and 21% showed complete clearance. Targeted radiation with the UVB lamp is effective for the treatment of plaque-type psoriasis, requiring as few as six sessions and achieving moderately long remission. As treatment is selectively directed toward lesioned skin, normal surrounding skin is spared unnecessary radiation exposure.

Basal cell carcinoma arising over facial port wine stain: a single-centre experience.

BACKGROUND: A growing number of reports point to a possible connection between basal cell carcinoma (BCC) and port wine stain (PWS). Researchers suggest that either the elevated temperature induced by the increased dermal vasculature or an oncogenic factor produced by the ectatic vessels makes the overlying epidermis more susceptible to ultraviolet or ionizing radiation. OBJECTIVE: To check the prevalence of BCC in patients with PWS at a large national vascular amomalies centre. METHODS: The study group included 68 patients, 44 women and 24 men, with facial PWSs. Detailed data were collected on background features, past treatments and past exposure to radiation, and a comprehensive physical examination was performed. Biopsy samples were taken from suspect lesions for histological study. RESULTS: Four patients (5.9%) were found to have histologically proven BCC, 3 nodular and one multifocal. All had been exposed to radiation during childhood. Treatment consisted of excision; there was one recurrence. CONCLUSION: Although the co-occurrence of BCC and PWS is probably related to radiation treatment in childhood, these findings should alert physicians to regularly examine PWSs for cancerous changes, especially in patients at risk.

Use of in vitro release of interferon-gamma in the diagnosis of contact allergy to potassium dichromate - a controlled study.

The use of in vitro release of interferon-gamma (IFN-gamma) in the diagnosis of contact allergy to potassium dichromate was studied in 20 patients who had positive patch tests to chromate and in 30 control subjects (10 patients with contact dermatitis, allergic to other allergens, 10 patients with other dermatologic diseases and 10 healthy subjects). The release of IFN-gamma in the supernatants of the peripheral blood lymphocytes was significantly higher in the patients with proven allergy to chromate (P = 0.001). Further studies are needed to determine if IFN-gamma release may serve as an additional diagnostic tool in contact dermatitis.

Changing distribution of Candida species isolated from sterile and nonsterile sites in Israel.

To determine if the prevalence of non- albicans Candida spp. has increased at two institutions in Israel, the distribution of 6,954 Candida isolates obtained from sterile and nonsterile sites during the periods 1995-1996 and 1999-2000 were compared. In the latter period, a slight decrease was observed in the prevalence of non- albicans Candida spp. isolated from sterile sites (from 39% to 37%) and nonsterile sites (from 38% to 35%). Specifically, the prevalence of Candida glabrata increased significantly in sterile sites, from 26% to 35% (P=0.0095), and in nonsterile sites, from 18% to 27% (P<0.0001), and the prevalence of Candida krusei increased significantly in sterile sites, from 2% to 7% (P=0.0072). The prevalence of Candida parapsilosis decreased significantly in nonsterile sites, from 31% to 23% (P=0.0002). Continuous surveys of the distribution of Candida spp. and analysis of the clinical significance of changes are warranted.

Mycosis fungoides: HLA class II associations among Ashkenazi and non-Ashkenazi Jewish patients.

BACKGROUND: An immunogenetic mechanism has been suggested to play a role in the pathogenesis of mycosis fungoides (MF). While results of studies on HLA class I associations haveproved inconsistent, two previous studies showed that certain HLA class II alleles were significantly increased among North American caucasian patients with MF: HLA-DRB1*11 and DQB1*03. OBJECTIVES: To investigate the possible HLA class I and class II associations with MF among Jewish patients. METHODS: The patient group comprised 68 Jewish patients with MF: 38 Ashkenazi and 30 non-Ashkenazi. The control group comprised 252 healthy Jewish volunteers: 132 Ashkenazi and 120 non-Ashkenazi. Tissue typing for HLA class I (A and B) was performed using the National Institutes of Health microlymphocytotoxicity technique. DNA-based low-medium resolution analysis for DRB1* and DQB1* alleles was performed using polymerase chain reaction (PCR) amplification with sequence-specific primers. For those alleles found to have significantly increased frequency, high-resolution analysis was done by means of PCR sequence-specific oligotyping. RESULTS: The allele frequency of HLA-DRB1*11 was found to be significantly increased but only among Ashkenazi patients with MF (30% vs. 19% in the controls; P = 0.034). High-resolution analysis for DRB1*11, not previously performed, suggested that its greater frequency is due to the increased number of Ashkenazi MF patients with the DRB1*1104 allele (P corrected = 0.036). Analysed together, DQB1*03 alleles (DQB1*0301-0304) had a significantly greater frequency in MF as a group as compared with controls (47% vs. 33%, P = 0.003). DQB1*0301 was demonstrated to be the specific allele associated with MF in Jewish patients (allele frequency of 36% vs. 23% in controls; P corrected = 0.0068), which was not the case for North American caucasian patients with MF. No greater frequencies of any of the HLA class I A or B antigens were found. CONCLUSIONS: Our findings further demonstrate the 'universality' of MF HLA class II susceptibility alleles, i.e. HLA-DRB1*11 and HLA-DQB1*03, suggesting that HLA polymorphism is likely to be important in the pathogenesis of MF in Jewish patients, as it is in North American caucasian patients. Not previously reported is our finding that HLA-DRB1*1104 is the specific allele more prevalent among patients with MF. Our study also underscores some differences in HLA profiles between non-Jewish and Jewish patients with MF and between Ashkenazi and non-Ashkenazi Jewish patients, indicating the possibility of diverse HLA disease associations in populations with different genetic backgrounds. Our study provides further evidence for the lack of association between HLA class I and MF.

Cutis marmorata telangiectatica congenita: clinical findings in 85 patients.

Cutis marmorata telangiectatica congenita (CMTC) is an uncommon, sporadic, congenital cutaneous condition presenting with persistent cutis marmorata, telangiectasia, phlebectasia and possible ulceration of the involved skin, skin atrophy, and undergrowth of the involved extremity. To further the current understanding of this disorder, we analyzed the clinical features of a large series of patients with CMTC. The files of 85 patients with CMTC who were examined in our center over the last 20 years were reviewed. The data recorded included patient sex, age at onset, nature and distribution of the lesions, associated abnormalities, and course of disease. CMTC was characterized by early recognition of the lesions (94% at birth) and equal sex distribution. The lesion was unilateral in 65% of patients and most commonly involved the limbs (69%). Additional capillary lesions, noted in 20% of patients, may well have been an extension of the CMTC itself. Associated anomalies were found in 18.8% of patients. Almost half of the patients showed a definite improvement in the reticular vascular pattern on follow-up. No familial cases of CMTC were noted. CMTC is more prevalent than previously recognized, affects both sexes equally, and has a mostly localized distribution; additional vascular lesions are associated with the disorder, but other anomalies occur less often. Prognosis is generally good.

Follicular cutaneous T-cell lymphoma: a clinicopathological study of nine cases.

Nine patients with follicular cutaneous T-cell lymphoma (CTCL), a recently described variant of lymphoma, are presented. On the basis of clinical manifestations and disease course, three groups of patients were distinguished: (i) two patients with follicular CTCL not associated with conventional lesions of mycosis fungoides (MF) and showing no evolution towards MF in follow-up periods of 3 and 6 years; (ii) one patient with follicular CTCL that evolved into conventional MF within 3 years; (iii) six patients showing conventional MF lesions either before or concurrently with the follicular lesions and thus representing follicular CTCL of the true MF type. The follicular lesions included hair-devoid patches or plaques with spiky hyperkeratotic papules (four patients), keratosis pilaris-like lesions (four), comedo-like lesions (four), follicular papules with alopecia (three) and milia-like lesions (three). Histopathological examination showed perifollicular and intrafollicular lymphocytes, without mucin deposition and with minimal or no involvement of the overlying epidermis. Significant syringotropism was also observed in three cases. Immunohistochemical analysis showed the predominance of CD4 + T cells, deletion of CD7 in some cases, Ki-67 + lymphocytes confined mainly to the follicular epithelium, and expression of keratinocyte intercellular adhesion molecule-1 exclusively in the hair follicle. T-cell receptor gamma gene rearrangement was positive in the one case studied from each group. Different treatment modalities were employed, the most commonly used as monotherapy being phototherapy: psoralen ultraviolet A in four patients, two of whom showed a complete clinical and histopathological remission, and ultraviolet B in one patient, who showed a complete remission (both clinical and histopathological). This study indicates that follicular CTCL is more common than reflected in the literature, has heterogeneous clinical manifestations, and is either an expression of or closely related to MF. The influence of the follicular involvement on the therapeutic response remains to be clarified. However, our therapeutic experience clearly suggests that some patients with follicular CTCL can benefit from phototherapy.