Search

Patients with atopic dermatitis (AD) are susceptible to infectious and inflammatory cutaneous comorbidities.The aim of the study was to describe the prevalence of cutaneous comorbidities associated with AD, including their relationship with AD severity.A retrospective cross-sectional analysis was performed using the Israeli Maccabi Healthcare Services database. Prevalent AD cases on December 31, 2017, were diagnosed with AD at any time since 1998 and had 1 or more recent (2013-2017) AD diagnoses. Dispensed AD treatments within 5 or fewer years served as a surrogate for AD severity. Cutaneous comorbidities in AD cases were compared with non-AD controls matched 1:1 on age, sex, and residential area. Among adults, comorbidities were compared across AD severity using multinomial logistic regression.The eligible population included 94,483 patients with mild (57.7%), moderate (36.2%), or severe (6.1%) AD, and 94,483 matched non-AD controls. Skin infections, inflammatory skin conditions, cutaneous manifestations of AD, and sweat gland disorders were more prevalent ( P0.001) in patients with AD than in controls. Most cutaneous comorbidities that were more prevalent in adult patients with AD were also significantly ( P0.001) associated with AD severity.This study suggests that AD is associated with many infectious and inflammatory cutaneous comorbidities and highlights the relationship between AD severity and comorbidity prevalence.

Kerion celsi represents the inflammatory extreme of tinea capitis, as a delayed hypersensitivity reaction to the causative dermatophyte. Data regarding prevalence, trends in pathogens, and risk factors for scarring are limited.The main objective of the study is to assess clinical and epidemiologic features of children with kerion celsi and risk factors for scarring.We reviewed medical records of pediatric patients with kerion celsi treated between January 2006 and July 2020.Among 80 patients, the prevalence of permanent alopecia was 27.5%. Patients with remaining alopecia presented to our clinic at a mean 1.3 months earlier than those with complete response to treatment (2.2 ± 2.1 and 3.4 ± 4.8, respectively; p .05). Patients of Ethiopian ethnicity were more represented than in the general population; however, scarring was observed in only 11% (p = 0.08). Outcomes did not differ by pathogen, antifungal treatment prescribed, duration of treatment, or the use of prednisone or antibiotics.Scarring alopecia is a common complication of kerion celsi. Host innate immune response, pathogen virulence, and treatment timeline should be considered as possible variables affecting risk of scarring in the future studies.

Background Leishmania tropica (L. tropica) is associated with high morbidity and low response rate to therapy, especially in pediatric patients. Intravenous (IV) liposomal amphotericin B (LAmB) has been used off-label as a treatment for L. tropica CL for many years. However, data regarding its efficacy and safety in children are lacking. Objective To evaluate the efficacy and safety of IV LAmB for treating pediatric patients with L. tropica CL. Methods A retrospective single-center study of children diagnosed with L. tropica CL who were treated with IV LAmB during 2014-2020, at a tertiary medical center in Israel. Results Twenty-four patients were treated with IV LAmB. Fourteen (58%) completed the treatment protocol and 10 (42%) experienced an infusion-related adverse event (IRAE) leading to treatment termination. Complete response was noted in 6/14 (43%) patients, while 8/14 (57%) failed to respond. Lower response rate was noted in lesions involving the mid-facial area. Conclusion The relatively low response rate is speculated to result from a low dose of LAmB, short follow-up period, and difficult to treat anatomic locations. The observation of a lower response rate for mid-facial lesions should be validated in larger cohorts. The high risk of IRAE should be considered in physician decisions regarding this treatment. This article is protected by copyright. All rights reserved.

Real-world data on the epidemiology and economic burden of atopic dermatitis (AD) are limited. Here we describe the epidemiology and economic burden of AD using electronic healthcare data from Israel.A retrospective study was performed using the Maccabi Healthcare Services database. AD incidence in 2008-2017 and point prevalence (ADAD incidence was 7.0/1000 person-years; overall prevalence was 4.4% (female patients 4.5%, male patients 4.3%; age 0 to less than 6 months, 0.9%; 6 months to less than 12 years, 11.0%; 12 to less than 18 years, 5.8%; 18 years or older, 2.2%). Among ADAD epidemiology in Israel is comparable with other real-world database studies. AD imposes an economic burden that increases with disease severity.Occurrence and costs of atopic dermatitis in IsraelAtopic dermatitis is a disease that causes the skin to be inflamed and itchy. Atopic dermatitis is most common in children but can also occur in adolescents and adults. Using data from a large healthcare provider in Israel, this study aimed to describe how common atopic dermatitis is within the population. Costs related to the use of healthcare services (such as visits to dermatologists and creams to treat atopic dermatitis) in the year 2017 were compared between persons with versus without atopic dermatitis. For the years 2008 to 2017, approximately 7 out of 1000 people were newly diagnosed with atopic dermatitis each year (incidence). Among people alive on 31 December 2017, 4.4% had atopic dermatitis (prevalence), with 42.3% suggestive of moderate to severe disease. Patients with atopic dermatitis, particularly those with more severe disease, used healthcare services more frequently. Compared with people without atopic dermatitis, medical costs among patients with atopic dermatitis were 36% higher (corresponding to added costs of $126 per person per year). This study helps to better understand how many people have atopic dermatitis, and what healthcare resources are needed to manage this disease.

Juvenile psoriatic arthritis (JPsA) is a severe inflammatory arthritis, which is associated with psoriasis in most cases. While there are few validated screening tools for diagnosis of arthritis for adult patients with psoriasis, those screening tools were never evaluated in children. The aims of this study were to evaluate two screening tools among pediatric patients with psoriasis.Thirty-nine patients with the diagnosis of psoriasis completed two screening questionnaires: The Psoriasis Epidemiology Screening Tool (PEST) questionnaire and the new Early Arthritis for Psoriatic Patients (EARP) questionnaire. All patients were evaluated by a rheumatologist for the diagnosis of JPsA, and the accuracy of the two questionnaires was compared.The 4/39 (10.1%) patients diagnosed with JPsA had a PEST questionnaire score of ≥ 3, compared to a median PEST score of the patients without the diagnosis of JPsA of 0 (0-2). Thus, both the sensitivity and specificity of the PEST in diagnosing JPsA were 100%. For the EARP questionnaire, 8/39 patients had a screening questionnaire score of ≥ 3, suggestive of JPsA, four were true positive, and four false positive. Thus, the sensitivity and specificity of EARP in diagnosing JPsA were 100% and 89%, respectively.Both the PEST and EARP questionnaires were easy to use and had high sensitivity for the diagnosis of JPsA in the pediatric population with psoriasis. The PEST questionnaire had a higher specificity than the EARP.• EARP and PEST are good screening tools for diagnosis of arthritis in pediatric population with psoriasis.

Both atopic dermatitis and celiac disease are often accompanied by other immune-mediated disorders. The objective of this study was to evaluate the potential association between atopic dermatitis and celiac disease in a broad community-based population. A cross-sectional observational design was used. Demographic and clinical data were collected for patients enrolled in a large health management organization who were diagnosed with atopic dermatitis by a dermatologist in 2002–17. The presence of celiac disease/celiac disease-related morbidities was recorded for the whole group, for adults (age > 18 years), and for adults with moderate-to-severe atopic dermatitis. Findings were compared with a matched control group without atopic dermatitis. The study group included 116,816 patients of whom 45,157 were adults; 1909 adult patients had moderate-to-severe atopic dermatitis. Compared to the respective control subjects, the prevalence rate of celiac disease in the whole group was 0.6% vs. 0.4%; in the adults, 0.6% vs. 0.3%; and in the adults with moderate-to-severe atopic dermatitis, 0.8% vs. 0.3% (p

Traditionally, most children diagnosed with Henoch-Schonlein Purpura (HSP) were hospitalized. This policy gradually changed towards selective hospitalization, yet there are still no criteria for admitting pediatric patients with HSP.To examine the clinical features and criteria for hospitalization of pediatric patients with HSP.A retrospective analysis was conducted of medical records of pediatric patients with HSP examined in the emergency department (ED) of Schneider Children's Medical Center, during 2005-2015. We compared children who were admitted with those not admitted to the hospital.During the study period, 116 children diagnosed with HSP were examined in the ED of Schneider Children's Medical Center: 14(12%) were admitted at first referral, and 22 (19%) of the children were hospitalized subsequently. The average age of all the children with HSP was 7.4 years, 57% were boys, and approximately 10% of the children had additional diseases, most of them auto-inflammatory. The main indications for hospitalization were gastrointestinal involvement (abdominal pain or bleeding), renal involvement (hematuria or proteinuria), and inability to walk. Most of these symptoms were also present in the children who were not admitted.Approximately 30% of the children were admitted to the hospital, most of them not at first referral. No clear criteria for admission were found, except for gastrointestinal bleeding.

Background: Psoriasis is a systemic disease with associated comorbidities. An association between renal diseases and psoriasis has previously been reported in adult patients, but little is known about renal diseases in pediatric patients. Objective: To determine whether there is an association between psoriasis and renal comorbidities in adult and pediatric patients. Methods: This cross-sectional study analyzed the database of the largest health care maintenance organization in Israel. Logistic regression was used to calculate odds ratios to compare 68,836 psoriatic patients and 68,836 controls with respect to renal comorbidities. Results: In adults, an inverse association emerged between psoriasis and dialysis (OR, 0.69; 95% CI, 0.58–0.83) and kidney transplantation (OR, 0.60; 95% CI, 0.43–0.83), a positive association with other kidney diseases (OR, 1.09; 95% CI, 1.05–1.13), and no association between psoriasis and chronic kidney disease (OR, 1.03; 95% CI, 0.98–1.09). Comparing 9,127 pediatric patients and 9,478 controls, no association was found between psoriasis and renal comorbidities, chronic kidney disease (OR, 0.90; 95% CI, 0.33–2.48), dialysis (OR, 2.06; 95% CI, 0.19–22.69), kidney transplantation (OR, 0.34; 95% CI, 0.04–3.29), or other kidney diseases (OR, 0.98; 95% CI, 0.79–1.23), even after a multivariate analysis adjusting for putative confounders. Conclusion: As opposed to adult patients, pediatric patients with psoriasis were not shown at risk of kidney diseases.

OBJECTIVE: To describe the cutaneous adverse effects (AE) to MAPK Extracellular Signal-Regulated Kinase (MEK) Inhibitor Trametinib in the pediatric population. METHODS: This was a retrospective single-center study. Included were all pediatric patients, treated with trametinib, for an oncologic indication. All patients were evaluated by a pediatric dermatologist, prior to, and during treatment, with documentation of cutaneous findings. RESULT: Twenty patients were enrolled in the study. All patients received treatment with trametinib, of which 6 received a combination of trametinib and dabrafenib (BRAF inhibitor). Out of twenty patients, 18 patients (90%) presented with at least one cutaneous AE. Xerosis and pruritic eczematous changes were the most common (15 patients, 75%), which, in most cases, were tolerable and responded well to the use of emollients and topical corticosteroids. Eleven patients (55%) presented with paronychia which was treated with topical combined corticosteroids antifungals and antibiotics, all with good response. Six patients (30%) presented with acneiform eruption, treated with topical antibiotic, benzoyl peroxide and tretinoin, mostly with good response. Six patients (30%) presented with irreversible hair heterochromia. Reaction grades were reported for cutaneous reactions, most of them were Grade I or II. Only 2 patients reported to have grade III and IV cutaneous reactions: exfoliative dermatitis and erythema multiforme, respectively. Out of 6 patients that received combined treatment of trametinib and dabrafenib one patient had no cutaneous adverse reaction, and one had panniculitis (which was related to dabrafenib). The rest presented relatively mild AE. DISCUSSION: Cutaneous AEs are very common in children and adolescents treated with trametinib, and in most cases are classified as mild. Nevertheless, as this treatment is usually chronic, it is important to inform the patients and their guardians of the potential cutaneous toxicities prior to treatment initiation, and to refer them to a dermatologist for proper management.

Segmental pigmentation disorder (SPD) is characterized by hypo- or hyper-pigmented patches segmentally distributed, present in infancy, more prominently in darker-skinned children. The aim of this study was to define the demographic and clinical characteristics of SPD in a large series of patients. This was a retrospective case-control study at 2 paediatric dermatology centres in Israel. Data were collected through a telephone questionnaire and medical records. The study group consisted of 144 individuals with SPD and 144 individuals visiting the same institutions matched for age and sex. Median age of onset of SPD was near birth; 51% of patients were Sephardic Jews, and patients were followed up for a median period of 27 years. The patches were located on the torso (43%), mostly hypopigmented (52%), and remained of the same intensity and size in 55% and 41% of cases, accordingly. No differences in extracutaneous morbidities were found between SPD and control patients. This study delineates the demographic and clinical characteristics of SPD, confirms that cutaneous findings in SPD are more prominent in darker skin, tends not to expand in size or accentuate throughout the years, nor to be associated with extracutaneous morbidities.

Background: Primary cutaneous lymphoproliferative disorders in solid organ recipients are rare. Reports of mycosis fungoides (MF) diagnosed in these patients are limited to less than 30 cases, with a heterogeneous disease course. Furthermore, management in terms of type and modulation of the preceding immunosuppressive treatments after MF diagnosis was not uniform. Objective: To report the characteristics of MF, treatment response, disease course, and status of immunosuppressive therapy in patients who are solid organ recipients, or under similar immunosuppression. Methods: The medical files were retrospectively reviewed for all solid organ transplant patients/patients who are under similar immunosuppression for other indications, who were also diagnosed with MF, and were followed at the cutaneous lymphoma outpatient clinics of 2 tertiary medical centers from 1/2010 to 12/2020. Results: The cohort included 11 patients (8 male; median age at organ transplantation/immunosuppressive state, 33 years). Transplanted organs involved the liver (n=5), kidney (n=3), liver and kidney (n=1), or heart (n=1). Patients were treated with mycophenolic acid, tacrolimus, and prednisone (n=5) or tacrolimus (n=5). One patient with nephrotic syndrome received cyclosporine. Median age at diagnosis of MF was 48 years, 45% were diagnosed before the age of 18 years, median time from organ transplantation/immunosuppressive state to diagnosis of MF was 8 years (range 0.5–22 years). Ten patients had early stage, (IA, 5; IB, 5), 50% of whom had early folliculotropic MF (FMF), (in some combined with classic/hypopigmented MF). One patient had advanced MF (IIIA) with folliculotropic erythroderma. Early-stage patients were treated mainly with skin-directed therapies, achieving either partial (50%) or complete response (50%). The erythrodermic patient achieved partial response after being treated with ultraviolet A and narrow-band ultraviolet B with acitretin. Immunosuppressive treatment was modulated in 4/11 patients, (cyclosporine switched to mycophenolic acid, 1; mycophenolic acid stopped and tacrolimus continued, 1; tacrolimus continued at a lower dose, combined with mycophenolic acid and prednisone, 1; tacrolimus stopped, 1). Median time of follow-up was 4 years (range 1–8 years), with no stage progression noted. At the last visit, 6 had no evidence of disease, and 5 had active disease (IA, 4; III, 1). Conclusions: Most solid organ recipient MF patients present with early-stage disease, similar to MF in the general population, with over representation of FMF, and over representation among children. Treatment is challenging due to the notorious higher risk of non-melanoma skin cancer in organ transplant recipients. Furthermore, immunosuppressive therapy modifications to improve MF should be balanced against the risk of solid organ rejection. Response to treatment and course do not seem to differ from MF in the general population, although longer follow-up is needed.

Background/objectives Lichen sclerosus is a rare, pruritic, mucocutaneous disease affecting mostly the anogenital area. Reports have occasionally associated lichen sclerosus with overlapping vascular lesions. This study explores this association in children. Methods A retrospective study was conducted in the dermatology unit of a pediatric tertiary care medical center. Electronic medical records were searched for patients diagnosed with lichen sclerosus from 2006 to 2019. Review of the cases was performed to identify overlapping vascular lesions and review the clinical course of overlap cases. Results Of 74 children diagnosed with lichen sclerosus during the study period, five (6.75%) had overlapping vascular lesions and genital lichen sclerosus. Four patients presented with reticular telangiectatic macules and patches (n = 4, 5.4%) that appeared at or shortly after disease onset; resolution occurred a few months after treatment initiation. The fifth patient presented with telangiectases that appeared more than 2 years after the onset of the first symptoms of lichen sclerosus (n = 1, 1.3%). Conclusion Vascular lesions in children with genital lichen sclerosus are common and have variable clinical manifestations. Early appearance of reticular macules, patches, and papules is a variant of the disease and is followed by prompt resolution of these lesions. Pathogenesis is attributed to structural changes and repositioning of the papillary vascular plexus. These changes may be alarming to parents and therefore must be recognized by physicians to prevent unnecessary concern and investigations.

Atopic dermatitis is a chronic inflammatory skin disease. Methotrexate is an off-label systemic agent for treating uncontrolled atopic dermatitis.This study assessed the safety and efficacy of methotrexate in pediatric patients with atopic dermatitis.This multicenter, retrospective study assessed pediatric patients with atopic dermatitis who were treated with methotrexate.Medical records of 26 pediatric patients with atopic dermatitis were reviewed. All the patients had Investigator Global Assessment (IGA) scores of 3-4 and had received systemic therapy before receiving methotrexate. In all, 53.85% patients showed clinical improvement at 3 and 6 months after methotrexate initiation. The IGA scores of the patients decreased significantly at 3 months after methotrexate initiation compared with those at baseline; moreover, the IGA scores of the patients at 6 months after methotrexate initiation were lower than those at 3 months after methotrexate initiation. In all, 54% study patients are still undergoing methotrexate treatment. Median satisfaction rate with methotrexate was 7.5/10. However, 10.35% patients developed adverse events, of which only one patient discontinued the treatment.Our results indicate that methotrexate is a tolerable and effective agent for treating refractory childhood atopic dermatitis.

The verrucous epidermal nevus (VEN) is the most common type of epidermal nevi. As lesions can be disfiguring, treatment is often sought. Many therapeutic approaches have been reported, with variable efficacy and safety. Picosecond (PS) lasers are novel laser devices designated to target small chromophores. A side effect of these lasers is blistering due to epidermal-dermal separation. We aimed to harness this side effect of the PS lasers to treat patients with VEN. The purpose of this study was to report our experience treating VEN using a PS 532-nm laser. We present a retrospective case series of six patients with large VEN who were treated using a PS 532-nm laser (2–6 treatments, 8–10 weeks apart). Response in clinical photographs was assessed by two independent dermatologists and graded on a scale of 0 (exacerbation) to 4 (76–100% improvement). Patient satisfaction was recorded on a scale of 1–5. All patients demonstrated significant improvement. Average improvement was 3.7 on the quartile scale of improvement. Patient satisfaction rate averaged 4.7. The PS 532-nm laser is a promising novel modality for the treatment of large VEN.

Background and objectives The understanding that adipocytes are greatly influenced by thermal changes combined with the advancement of non-invasive ultrasound technologies have led to the application of ultrasound as an energy source to induce thermal fat destruction. While application of high intensity focused, ultrasound energy have been widely explored, there is far less information regarding the effects of non-focused ultrasound on adipose tissue. The purpose of this study was to characterize the effects of a novel transcutaneous, multi-elements, non-focused ultrasound energy regimen in an animal model, as a proof-of-concept of its potential to treat non-invasive subcutaneous benign tumors. Materials and methods The non-invasive transcutaneous ultrasound system prototype (LUMENIS, Ltd., Yoqneam, Israel) was applied to thermally induce adipocytes' death. During treatment, the ultrasound energy was transmitted into the subcutaneous adipose tissue (SAT) of 12 domestic adult female pigs. Two modes of operation (long and short), which differ in both the acoustic energy applied to the tissue and in their time durations (i.e., differ in their power settings), were used in this study. Efficacy and safety assessments included: Temperature measurement of skin and subcutaneous adipose tissue (SAT) visual inspection and ultrasound imaging of the thermally affected areas, histopathological assessment of tissue samples using hematoxylin & eosin, and Masson's trichrome stains and in situ cell death detection kit for apoptosis assessment. Results The long and short treatment modes led to a 13.2°C and 17.8°C rise from baseline, respectively, in the SAT, whereas skin surface temperature was practically unaffected. Visual, ultrasonographic, and histopathological evaluation of the treated area showed SAT ablation. No treatment-related changes were observed in the epidermis, dermis subcutaneous muscle and nerves, or in livers and kidneys of treated animals. Additionally, no significant changes from baseline in blood- and urine-borne analytes were detected post-treatment. Conclusions The novel transcutaneous, multi-elements, non-focused ultrasound energy regimen used in this study, proved effective in non-invasively ablating SAT in an animal model. The usage of low energy settings such as in the current study might reduce unwanted side effects related to high energy application. Lasers Surg. Med. 49:110-121, 2017. © 2016 Wiley Periodicals, Inc.

Aberrant nucleic acids generated during viral replication are the main trigger for antiviral immunity, and mutations that disrupt nucleic acid metabolism can lead to autoinflammatory disorders. Here we investigated the etiology of X-linked reticulate pigmentary disorder (XLPDR), a primary immunodeficiency with autoinflammatory features. We discovered that XLPDR is caused by an intronic mutation that disrupts the expression of POLA1, which encodes the catalytic subunit of DNA polymerase-α. Unexpectedly, POLA1 deficiency resulted in increased production of type I interferons. This enzyme is necessary for the synthesis of RNA:DNA primers during DNA replication and, strikingly, we found that POLA1 is also required for the synthesis of cytosolic RNA:DNA, which directly modulates interferon activation. Together this work identifies POLA1 as a critical regulator of the type I interferon response.

Background Disorders of the umbilicus are commonly seen in infancy, including hernias, infections, anomalies, granulomas, and malignancies. Meticulous inspection of the umbilicus at birth might reveal a persisting embryonic remnant, such as an omphalomesenteric duct (OMD), manifested by a variety of cutaneous signs, such as an umbilical mass, granulation tissue, or discharge. Objective To systematically review the available data regarding the presence and management of OMD remnant with cutaneous involvement to suggest a practical approach for diagnosis and treatment. Methods A systematic review of the literature evaluating OMD anomalies presenting with cutaneous symptoms was performed. In addition, an index case of an 11-month-old patient is presented. Results We included 59 publications reporting 536 cases; 97% of the patients whose age was noted were infants (mean age 11 months). In 7.5% of the cases, diagnosis was established only after treatment failure. In 6.4% of patients, nonlethal complications were reported, and in 10.3%, the outcome was death, partly due to delayed diagnosis or mismanagement. Limitations Limited quality of the collected data, reporting bias. Conclusion OMD is relatively rare; however, the clinician must consider this remnant while examining patients with umbilical abnormalities because mismanagement could cause severe morbidity and mortality.

There is a paucity of data on the use of biologic therapy in recalcitrant pediatric psoriasis. The current study presents pediatric psoriasis cases treated with biologic agents in a tertiary referral center.In this retrospective case series, data were collected on all patients ≤18 years old with severe psoriasis treated with biological therapy from 2010 through 2016 in a tertiary children's hospital. We included demographic data, previous systemic treatments, reason for discontinuation or switch to other systemic treatments, efficacy and side effects.There were 10 patients, mean age 5.75 (±3.3) years treated with biologic agents in our center; Etanercept was the most frequent biological treatment prescribed (n = 9) followed by adalimumab (n = 5) ustekinumab (n = 3) and infliximab (n = 2). Additional systemic therapy was added to the biological therapy in seven cases: Methotreaxate (n = 5), phototherapy (n = 4), cyclosporine A and colchicine (1 case each). The most common reason for discontinuation was secondary failure (5 for etanercept, 3 for adalimumab). Six patients failed one biological treatment and three patients failed two biological treatments. Four patients are still being treated with a first line biologic (Etanercept in all). Adverse events were rare.Biologic therapy is effective and safe in recalcitrant pediatric psoriasis. Larger series are needed to confirm our observation.

Amelanotic melanomas (AMMs) account for a small proportion of all melanomas. They pose a risk of delayed diagnosis and, consequently, poor prognosis. AMMs may atypically present as a pyogenic granuloma-like lesion. This study sought to investigate the prevalence and clinical and histological features of AMM masquerading as pyogenic granuloma. The database of a tertiary medical center was screened for all patients pathologically diagnosed with melanoma in 2005-2016. Those with a suspected primary (i.e. pre-excision) clinical diagnosis of pyogenic granuloma were identified, and their demographic, clinical, histologic, and outcome data were collected from the medical files. Of 2038 patients diagnosed with melanoma, 10 (∼0.5%) had a pyogenic granuloma-like AMM. The mean±SD age at lesion presentation was 56±18.9 years and the mean time from lesion appearance to diagnosis was 91.5±117.1 months. Nine tumors were located on the skin surface, and one on the oral mucosa. The mean lesion size was 19.6±14.1 mm and the mean Breslow's depth was 6.47±3.1 mm; all tumors presented in the vertical growth phase. Seven (70%) patients had lymph node involvement or metastasis at diagnosis. Two patients died of the disease within 1 year of diagnosis. Given the potential lethality of AMM and the benign nature of pyogenic granuloma, clinician recognition of pyogenic granuloma-like AMMs is crucial. In the presence of a pyogenic granuloma-like lesion, findings of older patient age and large tumor size should raise the index of suspicion and prompt a biopsy study, thereby ensuring early and accurate treatment.

Background/objectives Childhood allergic contact dermatitis is recognized as a significant clinical problem. The objective was to evaluate the rate of positive patch tests in Israeli children with clinically suspected allergic contact dermatitis, identify possible sex and age differences, compare results with those in Israeli adults, and review pediatric studies in the literature. Methods The study sample included 343 children and adolescents (197 female, 146 male; 1-18 years of age, mean age 11.8 years) with clinically suspected allergic contact dermatitis who underwent patch testing with a standard pediatric series of 23 allergens at a tertiary medical center from 1999 to 2012. Data on clinical characteristics and test results were collected retrospectively from the medical files. Results Ninety-eight subjects (28.6%) (75 girls [38.1%], 23 boys [15.8%]) had at least one positive reaction. The most frequent reactions were to nickel sulfate, followed by potassium dichromate and cobalt chloride. Nickel sulfate sensitivity was more common in girls, especially those younger than 3 years and older than 12 years. The prevalence of contact sensitization was similar in subjects with and without atopic dermatitis (50% and 51%, respectively). Conclusion Nickel is the most common allergen in Israeli children, especially girls. Patch testing should be performed in children with clinically suspected allergic contact dermatitis regardless of atopic background.

Background: Multifocal (≥5) infantile hemangiomas (IHs) are known as a risk factor for extracutaneous involvement. Liver is the most commonly involved organ, but involvement of other systems has also been reported. This study aims to describe the characteristic findings in a group of infants with multiple cutaneous hemangiomas, with emphasis on intracranial involvement. Methods: A retrospective case series study was carried out in a pediatric dermatology unit of a tertiary pediatric medical center. Patients diagnosed with multiple cutaneous IHs from 2006 to 2015 were identified by a computerized search. Clinical data were retrieved from the medical charts. Results: A total of 60 infants (37 females and 23 males) were identified for analysis. Forty-four brain ultrasounds were recorded and reported as normal. One patient out of the 44 was later diagnosed with a small asymptomatic hemangioma seen on a brain MRI/MRA done for another indication. Conclusion: Brain hemangiomas may present as an asymptomatic incidental finding in infants presenting with multifocal cutaneous and liver IHs. The single case reported in our study emphasizes the low prevalence and the benign course expected. Therefore, routine ultrasound screening for brain involvement is probably unnecessary for this population.

The verrucous epidermal nevus (VEN) is the most common type of epidermal nevi. As lesions can be disfiguring, treatment is often sought. Many therapeutic approaches have been reported, with variable efficacy and safety. Picosecond (PS) lasers are novel laser devices designated to target small chromophores. A side effect of these lasers is blistering due to epidermal-dermal separation. We aimed to harness this side effect of the PS lasers to treat patients with VEN. The purpose of this study was to report our experience treating VEN using a PS 532-nm laser. We present a retrospective case series of six patients with large VEN who were treated using a PS 532-nm laser (2-6 treatments, 8-10 weeks apart). Response in clinical photographs was assessed by two independent dermatologists and graded on a scale of 0 (exacerbation) to 4 (76-100% improvement). Patient satisfaction was recorded on a scale of 1-5. All patients demonstrated significant improvement. Average improvement was 3.7 on the quartile scale of improvement. Patient satisfaction rate averaged 4.7. The PS 532-nm laser is a promising novel modality for the treatment of large VEN.

Pediatric mycosis fungoides (MF) is a rare disease characterized by over-representation of atypical clinical variants, with a different prognosis from adult MF. Several human leukocyte antigen (HLA) alleles have been associated with MF in certain adult populations, including Israeli Jews. However, HLA data on pediatric MF as a group are lacking. To evaluate the possible association of the HLA system with pediatric MF, 59 Israeli Jewish patients diagnosed with MF at age ≤ 18 years underwent high- and intermediate-resolution genotyping for HLA class I (HLA-A*, HLA-B*) and class II (HLA-DRB1*, DQB1*) loci. The results were compared with data on 4169 umbilical cord blood units retrieved from a public cord blood bank in Jerusalem and samples from 252 healthy, unrelated Israeli Jewish volunteers. No statistically significant associations were found between pediatric MF and any of the alleles examined except HLA-B*73. However, given the extremely low frequency of B*73 in both the control group (0.1%) and the study group (2%), the biological significance of this finding is questionable. Further subgroup analyses by ethnicity (Ashkenazi and non-Ashkenazi) and clinicopathologic variant (follicular and non-follicular) yielded no significant between-group differences. These results suggest that the associations with the HLA system, reported previously in adult MF, do not hold true for pediatric MF. Thus, pediatric MF differs from its adult counterpart not only in clinical manifestations and course, but apparently also in the underlying immuno-pathogenetic mechanism.

Background Unilesional folliculotropic mycosis fungoides (UFMF) has been rarely reported. Objective The aim of this study was to describe our experience with UFMF. Methods Data were collected on all patients with clinicopathological UFMF who attended the Department of Dermatology of a tertiary university-affiliated medical centre in 1996–2013 and were followed prospectively. Results The sample included seven patients (five male, two female) of mean age 38 years at diagnosis; two were aged

Background The literature on mycosis fungoides (MF) in children/adolescents is sparse. Objective We sought to evaluate the characteristics of juvenile MF in a large cohort. Methods Data were collected on all patients with MF, aged 18 years or younger at the time of clinicopathologic diagnosis, who attended the Rabin Medical Center Dermatology Department, Petach Tikva, Israel, between 1994 and 2012 and were followed up prospectively. Results There were 50 patients (30 male; mean age 11.4 years at diagnosis); 18 (36%) had Fitzpatrick skin type IV or higher. All were given a diagnosis of early-stage disease (IA-IIA) except 1 (tumor stage, IIB). Eight had classic MF lesions only and 42 had other variants, alone or in combination; these were mainly hypopigmented MF (n = 29) and cases with subtle but clear clinicopathologic features of folliculotropic MF (FMF) (n = 18). Among the various skin-targeted therapies, psoralen plus ultraviolet A (systemic/bath) proved beneficial for FMF. During a follow-up period of 0.25 to 15 years (mean 4.5), 2 patients progressed from stage IA to IB or IIA. Limitations Relatively short follow-up is a limitation. Conclusions This case series shows that FMF is not uncommon in children and adolescents. It is characterized by more superficial clinical features and less heavy perifollicular lymphocytic infiltrates than adult FMF, and responds well to psoralen plus ultraviolet A. The prognosis of childhood FMF remains unclear.

Telangiectasia are cardinal features of systemic sclerosis (SS) and calcinosis, Raynaud's syndrome, esophageal motility, sclerodactyly, telangiectasias (CREST) syndrome. The etiology of telangiectasia in these syndromes is unknown, but vascular dysfunction has been proposed. However, the telangiectasia of CREST have anecdotally been considered relatively resistant to pulse dye laser (PDL), the treatment of choice for classic telangiectasia. The study was designed to test whether SS/CREST telangiectasia require more treatments than sporadic telangiectasia and to identify clinical and histological features that could explain such an effect. Nineteen skin biopsies from patients with SS or CREST and 10 control biopsies were examined and compared for features that may predict a differential response to PDL. Sixteen cases of SS or CREST treated with PDL between 1997 and 2007 were evaluated and response to treatment was compared with 20 patients with sporadic telangiectasis. Relative to normal skin, CREST/scleroderma telangiectasia exhibited thickened vessels in 17 out of 19 sections and thickened collagen fibers in the reticular or deep dermis in all sections. The number of treatments required to clear SS/CREST telangiectasia was approximately twofold higher. SS/CREST telangiectasia are more resistant to PDL but can be effectively cleared with more treatments.

Objective Propranolol is the treatment of choice for infantile hemangiomas requiring medical intervention. Although contraindicated in asthma, its bronchoconstrictive effect in infants and children has not been extensively studied. We aimed to assess the incidence of wheezing episodes in infants and children treated with propranolol for infantile hemangiomas. Study Design A retrospective case-control study. Setting: a tertiary pediatric hospital. Patients: All Children followed for infantile hemangioma between 2009 and 2014. Children followed conservatively served as control group and were matched 1:1 for gender and month of birth by random matching to children treated with propranolol. Interventions: All respiratory episodes (asthma, wheezing, stridor, and pneumonia) and respiratory associated hospitalizations were recorded from hospital records, from the primary care physician visits records and pharmacy prescriptions. The main outcome measure was the incidence of respiratory episodes in the treatment and the control groups. Results A total of 1828 clinic visits were reviewed for 683 children. In addition, primary care physician visits records were available in 80% of them. Two hundred and sixteen children were treated with propranolol. Incidence of respiratory episodes and recurrent respiratory episodes was similar in the propranolol and control groups (8.3% vs 12%, P = 0.265; 3.7% vs 6.5%, P = 0.274, respectively). Time to first episode was similar in the treatment and control groups (5.03 ± 3.32 vs 4.45 ± 3.21 months, respectively, P = 0.09). Respiratory hospital admission rate was similar in both groups. Conclusions Propranolol treatment does not exacerbate wheezing episodes in infants and children.

Background/Objective: Alopecia areata may occur at any age, though usually before the age of 20 years. Treatment often consists of systemic steroids administered as high-dose bolus infusions. This study sought to investigate the effectiveness and side effects of intravenous high-dose pulse corticosteroids in children with alopecia areata and to identify prognostic factors for successful treatment. Methods: Patients treated with pulse corticosteroids for alopecia areata in 2001-2008 at the day care unit of a tertiary pediatric medical center were identified by computerized file search and clinical treatment and outcome data were collected. Results: The sample included 24 children (16 female, 8 male) with a mean age of 8.5 ± 4.6 years at diagnosis; 8 (33%) had multifocal disease,10 (42%) multifocal disease with ophiasis, 4 (17%) alopecia totalis and 2 (8%) alopecia universalis. Nail involvement was noted in 9 patients (38%). Mean duration of disease was 22 ± 27 months. Patients were treated with 8 mg/kg body weight intravenous methylprednisolone on 3 consecutive days at 1-month intervals. After a mean of 5.65 ± 1.95 courses, 9 patients (38%) had a complete response, 7 (29%) a partial response and 8 (33%) no response. Of the 16 responders, 13 (81%) relapsed at 9.5 ± 12 months after the last course; 3 patients had side effects, none of which were severe. Three positive prognostic factors were identified: short disease duration (≤6 months), younger age at disease onset (Conclusions: Careful patient selection is necessary to achieve maximal benefit from pulse corticosteroid treatment for alopecia areata in children.

The understanding that adipocytes are greatly influenced by thermal changes combined with the advancement of non-invasive ultrasound technologies have led to the application of ultrasound as an energy source to induce thermal fat destruction. While application of high intensity focused, ultrasound energy have been widely explored, there is far less information regarding the effects of non-focused ultrasound on adipose tissue. The purpose of this study was to characterize the effects of a novel transcutaneous, multi-elements, non-focused ultrasound energy regimen in an animal model, as a proof-of-concept of its potential to treat non-invasive subcutaneous benign tumors.The non-invasive transcutaneous ultrasound system prototype (LUMENIS, Ltd., Yoqneam, Israel) was applied to thermally induce adipocytes' death. During treatment, the ultrasound energy was transmitted into the subcutaneous adipose tissue (SAT) of 12 domestic adult female pigs. Two modes of operation (long and short), which differ in both the acoustic energy applied to the tissue and in their time durations (i.e., differ in their power settings), were used in this study. Efficacy and safety assessments included: Temperature measurement of skin and subcutaneous adipose tissue (SAT) visual inspection and ultrasound imaging of the thermally affected areas, histopathological assessment of tissue samples using hematoxylineosin, and Masson's trichrome stains and in situ cell death detection kit for apoptosis assessment.The long and short treatment modes led to a 13.2°C and 17.8°C rise from baseline, respectively, in the SAT, whereas skin surface temperature was practically unaffected. Visual, ultrasonographic, and histopathological evaluation of the treated area showed SAT ablation. No treatment-related changes were observed in the epidermis, dermis subcutaneous muscle and nerves, or in livers and kidneys of treated animals. Additionally, no significant changes from baseline in blood- and urine-borne analytes were detected post-treatment.The novel transcutaneous, multi-elements, non-focused ultrasound energy regimen used in this study, proved effective in non-invasively ablating SAT in an animal model. The usage of low energy settings such as in the current study might reduce unwanted side effects related to high energy application. Lasers Surg. Med. 49:110-121, 2017. © 2016 Wiley Periodicals, Inc.

Psoriasis is a multifactorial chronic inflammatory disease. Monogenic psoriasis has been described recently, including dominantly inherited plaque and generalized pustular types, related to activating mutations in the CARD14 gene. We describe here a family with CARD14-related psoriasis, exhibiting an extreme variability of clinical presentation (from mild plaque-type to generalized pustular psoriasis) and early disease onset. The affected family members harboured the c.349G>A [p.Gly117Ser] mutation in CARD14, which has not previously been linked to pustular psoriatic phenotype. Furthermore, most severely affected individuals carried 3 additional CARD14 coding region polymorphisms (rs2066964, rs34367357 and rs11652075), suggesting their possible effect on disease expression. Early-onset psoriasis co-segregated with the HLA-C*0602, indicating that HLA-C*0602 could potentially modulate the time of disease onset. In summary, this paper describes a family with CARD14-related psoriasis and discusses the possible influence of the specific haplotypes on intra-familial variation in the clinical phenotype of the disease.

Background Some authorities consider alopecia mucinosa (AM)/follicular mucinosis (FM) to invariably represent mycosis fungoides (MF). This understanding of AM/FM derives from observations in adults. Objectives We sought to explore the clinicopathologic features and natural history of pediatric AM/FM. Methods Medical records were searched for children given the diagnosis of AM/FM from 1998 through 2009. Diagnosis of AM/FM was defined as the presence of well-demarcated hairless plaques with follicular prominence plus an abundance of mucin on histopathologic examination. Results Forty children with a clinical diagnosis of AM/FM were identified. Nine did not meet the inclusion criteria. In the 31 remaining cases (16 boys, 15 girls) the mean age at onset was 9 ± 3.5 years. Histopathologic examination showed folliculotropism in 28 patients (90%) and epidermotropism in 15 (48%). Twelve cases fulfilled the International Society of Cutaneous Lymphomas (ISCL) diagnostic criteria for early MF. The histopathologic findings were typical of MF in only in two of these cases. T-cell receptor gene rearrangement was positive in 3 of 6 (50%) of tested samples, one in a patient who fulfilled the ISCL criteria for early MF. Mean duration of follow-up was 6.2 ± 3.7 years. All skin lesions resolved and none persisted or recurred. Hodgkin lymphoma was diagnosed 6 months after diagnosis of AM/FM in one patient. Limitations This was a retrospective study. Conclusions Although some pediatric cases meet the diagnostic criteria for MF, AM/FM cannot be regarded unequivocally as early follicular MF in this age group. We suggest the current diagnostic criteria for early MF should exclude children with AM/FM. Long-term follow-up of children with AM/FM is nevertheless warranted.

Background: Infantile hemangioma is the most common tumor of infancy. There are recent reports of the efficacy of propranolol in the treatment of these hemangiomas. Nasal tip hemangiomas pose a particularly sensitive concern aesthetically and functionally. The treatment of nasal tip hemangiomas is controversial. We assessed the effect of propranolol therapy in hemangiomas of the nasal tip. Objectives: To evaluate the response of nasal tip hemangiomas to systemic propranolol. Methods: During 2008-2010 ten infant with nasal tip hemangiomas presented to our tertiary care center. All underwent comprehensive evaluation by a multidisciplinary team and were then treated with oral propranolol at 2 mg/kg/day, with continuous clinical follow-up until age 14-16 months, or in older infants until the proliferative phase resolved. Results: Eight patients demonstrated good clinical improvement. Two patients had partial improvement. One patient discontinued treatment due to wheezing. Three patients had mild sleep disturbance which did not warrant discontinuation of treatment. No rebound was noticed after cessation of treatment. Limitations: Children presented by referral at variable ages. It is possible that routine initiation of propranolol in neonates at the first sign of nasal hemangioma may reduce the required treatment duration or dose. Conclusions: Early treatment of hemangiomas of the nasal tip with propranolol prevents lesion proliferation, reduces lesion volume, and prevents nasal and facial deformation. Propranolol appears to be a safe and effective treatment. Its efficacy and safety profiles, relative to other accepted therapies, suggest that it should be considered as the first-line treatment when intervention is required.

Background Human herpesvirus (HHV) 8, an essential etiologic agent of Kaposi sarcoma, is also associated with several lymphoproliferative disorders. The involvement of HHV 8 in mycosis fungoides (MF) and large plaque parapsoriasis (LPP) is controversial, with contradictory reports from various countries worldwide. Objective We sought to investigate the presence of the HHV 8 genome in skin lesions of LPP and early-stage sporadic, familial, and juvenile MF in patients in Israel. Methods Archival paraffin-embedded and frozen samples from skin biopsies of untreated patients with LPP and early-stage MF performed in 1990 through 2006 were randomly collected from the department of dermatology of a tertiary medical center in central Israel. DNA was extracted, and a TaqMan-based real-time polymerase chain reaction assay specific for the K6 gene region was used to detect the HHV 8 genome. Results A total of 46 biopsies were sampled from 11 patients with LPP and 35 with early-stage MF (17 adults with sporadic MF, 10 children, and 8 patients with familial MF). In all, 44 samples were negative for HHV 8 DNA; two samples from adults with sporadic MF were positive. Limitations The presence of HHV 8 antibodies or virus sequences was not assessed in peripheral blood. Conclusion The results of this study, conducted in a region relatively endemic for HHV 8, support most earlier studies showing a lack of association of HHV 8 infection with LPP and sporadic adult-type MF. To our knowledge, the lack of association of HHV 8 infection with juvenile and familial MF has not been previously reported.

Pharmacological therapies for infantile hemangiomas were considered effective only during the proliferative phases. Recently reported beneficial effects of propranolol may extend beyond the proliferative phase of infantile hemangiomas. The purpose of the study was to assess the effect of oral propranolol therapy for infantile hemangiomas beyond the proliferative phase of these lesions. Members of the Society for Pediatric Dermatology were invited to participate in a multicenter retrospective study. Only children with infantile hemangiomas with documented cessation of lesions' growth or those older than 12 months of age were eligible for the study. Clinical and demographic information and digital photographs before, at the start, and following the treatment were collected. Scaled panels of photographs were distributed among preselected experienced pediatric dermatologists. Visual analog scale was used to assess photographs for each case. Paired t-test was used for statistical analyses. Data on 49 eligible patients from eight pediatric dermatology centers was collected. Seven cases were excluded because of insufficient photographic documentation. The age of the patients at the start of propranolol therapy ranged 7 to 120 months (mean 28 mos, median 22 mos). The duration of propranolol therapy ranged 1 to 8 months (mean 3.6 mos). The mean visual analog scale score before the treatment was 6.8 ± 2.15, and mean reduction in the visual analog scale score at the assessment was 2.6 ± 1.74 (p < 0.001). The rate of visual analog scale reduction was 0.4 per month before the start of the therapy, while this rate was accelerated to 0.9 per months following the therapy (p < 0.001). No significant side effects were reported. We conclude that propranolol is effective in infantile hemangiomas, including post-proliferative phase, and should be considered as the first-line therapy in that setting.

Dead Sea (DS) mud and water are known for their unique composition of minerals, and for their therapeutic properties on inflammatory skin diseases. The objective of the study was to evaluate the efficacy of an emollient cream enriched with DS water in children with atopic dermatitis (AD). Eighty six AD children were randomized in a double-blind controlled study to receive twice-daily topical treatment with a body cream enriched with DS minerals (TP) compared to two types of control: 1) DM, DS minerals with lower DS water concentrations than TP, and 2) an emollient (E) with no DS minerals. Efficacy was assessed by a change in clinical skin severity scores: SCORing Atopic Dermatitis (SCORAD), investigator’s global assessment (IGA) and patient global assessment (PGA) as well as by objective physiological parameters: transepidermal water loss (TEWL), stratum corneum hydration (SCH), affected body surface area (BSA) and Objective Severity Assessment of Atopic Dermatitis (OSAAD). The total length of the trial was 12 weeks divided to 6 visits at weeks 0, 2, 4, 6, 8, 12. The study showed that both TP and DM creams improved OSAAD scores. Only TP improved TEWL and SCH. TP was the most effective regarding TEWL, SCH and OSAAD compared to DM and E. Treatment with E decreased more significantly IGA score compared to TP. Although within each treatment group significant improvements in SCH, BSA, SCORAD, IGA and PGA were observed, the reduction in BSA, SCORAD and PGA was not significantly different among the groups. Our results clearly show the benefits of TP as a leave on-skin emulsion enrich with DS water in terms of skin barrier function. Thus, TP can serve as an effective adjuvant treatment for AD skin as well as for its maintenance.

Summary Background Cutaneous lymphomas rarely occur in children and adolescents, and are mostly of the T-cell lineage. Low-grade primary cutaneous B-cell lymphoma (CBCL) is extremely rare in individuals under 18 years old. Only 11 patients under 20 years old have been reported in the literature. Objectives To evaluate the number of patients younger than 18 years with primary CBCL diagnosed at our centre and to investigate its clinicopathological features, treatment and course in this age group. Methods We reviewed the files of all 90 patients with primary CBCL who attended the Department of Dermatology of our tertiary care university-affiliated centre from 1992 to 2007. Results Four patients who met study criteria were identified: three girls and one boy. Mean age at diagnosis was 16·6 years (range 16–17). Three patients had cutaneous marginal zone lymphoma (CMZL), and one had a spindle-cell (sarcomatoid) lymphoma, most probably follicular centre cell type. All were treated with the standard regimen used in adults. The mean duration of follow up was 45 months. No extracutaneous progression was noted. At present two of the four patients are in complete clinical remission. Conclusions In Israel, primary CBCL apparently occurs more often in young patients than reported in the literature. CMZL is the most frequent type. Long follow up is mandatory to assess the biological behaviour of CBCL in the paediatric/adolescent age group.

The immunomodulator, AS101, inhibits production of IL-10, IL-4 and expression of p38 MAPK, while increasing production of IFNgamma and IL-2. Together with its excellent clinical safety profile in humans, suggests a potential therapeutic use for AD.

In this article, we describe a novel autosomal recessive ichthyosis with hypotrichosis syndrome, characterized by congenital ichthyosis associated with abnormal hair. Using homozygosity mapping, we mapped the disease locus to 11q24.3-q25. We screened the ST14 gene, which encodes matriptase, since transplantation of skin from matriptase(-/-)-knockout mice onto adult athymic nude mice has been shown elsewhere to result in an ichthyosislike phenotype associated with almost complete absence of erupted pelage hairs. Mutation analysis revealed a missense mutation, G827R, in the highly conserved peptidase S1-S6 domain. Marked skin hyperkeratosis due to impaired degradation of the stratum corneum corneodesmosomes was observed in the affected individuals, which suggests that matriptase plays a significant role in epidermal desquamation.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon dermatosis of unknown etiology that manifests as characteristic red nodules and papules with a predilection for the scalp and periauricular region. Treatment is required for both esthetic and functional reasons, as lesions may ulcerate and bleed. Many treatment approaches have been reported, including excision, systemic medical approaches, topical or intralesional therapies, and non-invasive modalities including cryotherapy, electrosurgery, and laser. Treatments have exhibited variable efficacy, and the recurrence rate is 100 %. We report the combination of pulsed dye laser and CO2 laser in the treatment of ALHE in 14 patients. All patients exhibited clinical response after a mean of 2.4 ± 0.4 treatment sessions. The clinical efficacy of the combined treatment, together with its well-tolerated nature, render the use of pulsed dye laser in combination with CO2 laser, a viable treatment for debulking ALHE lesions. Ongoing maintenance treatments are needed to due to the high degree of relapse.

BACKGROUND Laser treatment of lymphangioma circumscriptum is a major challenge because of the persistent nature of the disease and the large variations in lesion size, depth, and anatomic location. OBJECTIVE To determine the safety and effectiveness of electro-optical synergy, a new technology that combines a 900 nm diode laser with radiofrequency current, for the treatment of lymphangioma circumscriptum. METHODS Six patients with Fitzpatrick skin types II to IV were treated for lymphangioma circumscriptum with 1 to 3 sessions of simultaneous radiofrequency energy (60–80 J/cm3) and optical diode laser energy (fluence 90–100 J/cm2). Lesion clearance was evaluated by three specialists on the basis of digital photographs taken before the first treatment and 1 and 2 months after the last treatment. RESULTS Results were rated as “excellent” in four patients and “good” in two patients. Transient swelling, erythema, and pain were present in all patients, and ulcers and scarring in two patients. CONCLUSION The combination of laser light and radiofrequency energy is effective and relatively safe for the treatment of lymphangioma circumscriptum. It provides additional heating of the blood vesicles without increasing the laser intensity and allows the clinician to treat the clear lymphatic component, which lacks a specific optical chromophore. Side effects are tolerable.

Monilethrix is a structural defect of the hair shaft usually inherited in an autosomal dominant fashion and caused by mutations in the hHb1, hHb3, and hHb6 keratin genes. Autosomal recessive inheritance in this disease has been sporadically reported. We encountered 12 Jewish families from Iraq, Iran, and Morocco with microscopic findings of monilethrix, but with no evidence of vertical transmission. Since no mutations were found in these three hair keratin genes, we examined nine chromosomal regions containing gene clusters encoding skin and hair genes. On chromosome 18q, a common haplotype in the homozygous state was found among all seven Iraqi patients, but not in 20 controls (P