Paediatric mycosis fungoides - characteristics, management and outcomes with particular focus on the folliculotropic variant.

BACKGROUND: The literature on paediatric mycosis fungoides (MF) and especially its folliculotropic variant (FMF) is sparse. OBJECTIVES: To describe the clinical manifestations, treatments, outcomes and long-term course of paediatric MF, including FMF. METHODS: A retrospective analysis was conducted of all consecutive MF patients diagnosed at ≤18 years attending two medical centres in 1995-2015. RESULTS: The cohort included 71 patients, all but two of whom had early-stage disease: hypopigmented (55%), folliculotropic (42%) and classical MF (39%), alone or in combination. The head and neck area were involved in 43% of patients with early-stage FMF compared to 12% of the non-FMF group (P = 0.004). There was no difference in the involvement of other body areas between the groups. Pruritus, although mild, was more often recorded among patients with early-stage FMF compared to non-FMF (58% vs. 29%, respectively, P = 0.02). Complete response (CR) was achieved in 60 of the 69 patients with early-stage MF (87%) after an average of 1.8 treatment modalities. NBUVB was the most administered treatment to non-FMF patients with CR rates of 63% vs. 29% of FMF patients (P = 0.04). Systemic/bath PUVA and UVA+NBUVB were the most administered treatments to FMF patients with CR rates of 60% vs. 81% for non-FMF patients (P = 0.17). During a mean follow-up of 9.2 years (range 1-24), stage progression was observed in four (6%) of the patients with early-stage disease, two of whom (all FMF) to advanced stage. CONCLUSIONS: Paediatric MF presents as an early-stage disease with over-representation of hypopigmented and FMF variants. NBUVB and UVA-based therapies yield good response rates in non-FMF and FMF patients, respectively. Disease course is indolent, and even on relatively long follow-up, it has a very low progression rate from early to advanced-stage disease, occurring in patients with FMF. We propose a treatment algorithm for paediatric MF.

Clinical clues and trends in epidemiology and pathogens in paediatric tinea capitis: a retrospective cohort study.

BACKGROUND: Tinea capitis is the most common dermatophyte infection in children. Pathogens differ between populations. During the past few decades pathogen shifts have been reported in many countries around the world. This study aimed to characterize the pathogens involved in tinea capitis in a large tertiary paediatric medical centre in Israel. METHODS: The electronic medical records of the dermatology unit were searched for patients diagnosed with tinea capitis from 2010 to 2019, and demographic, clinical and mycological data were retrieved. A stepwise logistic regression was performed to determine the variables most significantly associated with positive cultures. RESULTS: The prevalence of Trichophyton tonsurans isolates was found to be significantly increased during 2019. In the logistic regression analysis, alopecia was most significantly associated with a positive culture (OR = 8.72, 95% CI 4.29-17.7, P < 0.001) while positive culture was also associated with age (OR = 1.11 per year of age, 95% CI 1.02-1.22, P = 0.01) and Ethiopian ethnicity (OR = 3.67, 95% CI 1.42-9.45, P = 0.01). The presentation of alopecia was significantly related to having a positive culture. This symptom was more prevalent in boys than in girls, and the diagnosis of tinea capitis was delayed by an average of 2 months in girls. CONCLUSION: The results of this study may herald a pathogen shift in Israel. Physicians should be aware that rates of alopecia are lower in girls and this can result in delayed diagnosis and the development of complications, including chronicity, scarring alopecia and kerion formation.

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.

Unilesional folliculotropic mycosis fungoides: a unique variant of cutaneous lymphoma.

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 <18 years. The lesion presented as a solitary patch/plaque with follicular accentuation in five patients, an infiltrated plaque devoid of hair in one and with follicular nodules in one. Four patients had alopecia, and one, secondary anetoderma. The lesion was located on a limb in four patients, the trunk in two, and the face in one. In all cases, the atypical folliculotropic lymphocytes expressed mainly surface CD4(+). Monoclonality was detected in three of the six patients analysed. Treatment consisted of localized electron beam in five patients, all of whom had a complete response (CR), and excision in one patient. The remaining patient, a 9-year-old boy, was treated with topical psoralen and UVA with CR. The duration of follow-up was 0.5-10 years (mean 4). There were no recurrences in six patients and local recurrence in one. CONCLUSION: UFMF presents at a young age, usually with early disease clinical morphology. The treatment goal should be cure. Our experience indicates an excellent prognosis of early UFMF with no multifocal/internal spread.

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.

Barrier-restoring therapies in atopic dermatitis: current approaches and future perspectives.

Atopic dermatitis is a multifactorial, chronic relapsing, inflammatory disease, characterized by xerosis, eczematous lesions, and pruritus. The latter usually leads to an "itch-scratch" cycle that may compromise the epidermal barrier. Skin barrier abnormalities in atopic dermatitis may result from mutations in the gene encoding for filaggrin, which plays an important role in the formation of cornified cytosol. Barrier abnormalities render the skin more permeable to irritants, allergens, and microorganisms. Treatment of atopic dermatitis must be directed to control the itching, suppress the inflammation, and restore the skin barrier. Emollients, both creams and ointments, improve the barrier function of stratum corneum by providing it with water and lipids. Studies on atopic dermatitis and barrier repair treatment show that adequate lipid replacement therapy reduces the inflammation and restores epidermal function. Efforts directed to develop immunomodulators that interfere with cytokine-induced skin barrier dysfunction, provide a promising strategy for treatment of atopic dermatitis. Moreover, an impressive proliferation of more than 80 clinical studies focusing on topical treatments in atopic dermatitis led to growing expectations for better therapies.

Effect of insulin-like growth factor-1 deficiency or administration on the occurrence of acne.

BACKGROUND: The role of growth hormone, insulin, and insulin-like growth factor-1 (IGF-1) in the development of acne is incompletely understood. OBJECTIVE: To study the effect of the absence of IGF-1 and its pharmacologic replacement on the occurrence of acne vulgaris. PATIENTS AND METHODS: Laron syndrome (LS) is characterized by congenital IGF-1 deficiency. The study group consisted of 21 patients with classical LS, who underwent puberty: 13 (8 male, 5 female) untreated and under regular follow-up until age 20?48 years; and 8 (2 male, 6 female) treated with IGF-1 (70-200 µg/kg/day), including 6 adults (2 male, treated at age 14.5-29 years and 4 female, treated at age 30-37 years) and 2 adolescents (2 female, treated at age 3.5-16 years). The medical files were reviewed for occurrence of acne and the corresponding sex hormone levels, and the findings were compared between the treated and untreated patients. RESULTS: Puberty was delayed in all untreated patients. Only one patient had slight acne at age 22 years, when he reached full puberty. Among the 2 IGF-1 treated male patients, none acquired acne. Among the 6 treated female patients, 3 had signs of hyperandrogenism (oligo-amenorrhea) and acne during IGF-1 over-dosage. On reduction of the IGF-1 dose (to 50 µg/kg/day) or cessation of treatment, the acne disappeared in all 3 patients. CONCLUSION: This study demonstrates for the first time that serum IGF-1 deficiency prevents the occurrence of acne. The findings suggest that an interaction between IGF-1 and androgens is necessary for the development of acne.

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.

Juvenile onset of primary low-grade cutaneous B-cell lymphoma.

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.

Intralesional and sub-Tenon's infusion of corticosteroids for treatment of refractory periorbital and orbital capillary haemangioma.

PURPOSE: To evaluate the effect of combined intralesional and sub-Tenon's administration of corticosteroids for the treatment of refractory periocular and orbital capillary haemangioma in a retrospective, non-comparative study. METHODS: Seven infants with resistant periorbital and orbital capillary haemangioma who attended our tertiary centre from 2000 to 2005 were treated with an intralesional injection of a mixture of betamethasone 6 mg/cc and triamcinolone 10 mg/cc, by body weight, together with sub-Tenon's infusion of betamethasone 6 mg/cc and triamcinolone 40 mg/cc of a volume of 1 cc, close to the orbital lesion, in the same session. Visual axis, proptosis, pre- and post-treatment changes in four refractive parameters, and parental satisfaction were evaluated. RESULTS: None of the babies had proptosis or visual axis obstruction after treatment. Mean spherical power decreased by 22.7% (P=0.06). Mean spherical equivalent decreased in 34%, but the difference was not statistically significant (P=0.09). No early or late ocular side effects were observed. Mean intraocular pressure results were normal before and immediately after treatment, and during the follow-up period. Parental satisfaction score during follow-up was 9/10. Mean time to improvement was 96.43+/-58.3 days. CONCLUSIONS: Combined local corticosteroid administration by posterior sub-Tenon's infusion and intralesional injection in babies with extensive capillary haemangioma is associated with a satisfactory anatomical and functional outcome and there were no ophthalmic side effects.

In vitro cytokine profile in childhood alopecia areata and the immunomodulatory effects of AS-101.

The aim of the present study was to determine the effect of AS-101, a known immunomodulator, on the pattern of cytokine production in children with patchy alopecia areata (PAA). Ten previously untreated children with PAA were compared to 10 healthy age- and sex-matched controls. Peripheral blood mononuclear cells (PBMC) were isolated from all participants. Unstimulated and phytohaemagglutinin (PHA)-stimulated PBMC were tested with and without the addition of AS-101. The production of interferon gamma (IFNgamma), soluble interleukin (IL)-2 receptor (IL-2R), IL-10, IL-5 and IL-6 was determined. The levels of soluble IL-2R, IL-5 and IL-6 were significantly higher in the PAA patients than the controls. AS-101 inhibited the production of IL-10, IFNgamma, IL-2R and IL-5 in both PAA patients and controls, but there was a greater inhibitory effect in children with PAA.

Eyelash length in children and adolescents with allergic diseases.

Long eyelashes may be congenital, acquired in association with certain systemic diseases, or drug induced. In the past, long eyelashes were considered an external sign found in children with allergic diseases. However, this claim has never been examined in a controlled study. We compared the eyelash lengths of allergic children and adolescents with perennial allergic rhinitis, with or without bronchial asthma and atopic dermatitis (n = 60) to those of age- and sex-matched nonallergic controls (n = 80). The eyelashes of the allergic patients were found to be significantly longer than those of the controls: 9.43 +/- 1.39 mm versus 8.45 +/- 1.30 mm (p < 0.001). Eyelash length did not differ between patients with allergic rhinitis only (n = 31; 9.65 +/- 1.43 mm) and patients with allergic rhinitis and other allergic diseases (n = 29; 9.19 +/- 1.31 mm) (p = 0.196). These results indicate that children and adolescents with allergic diseases have longer eyelashes compared to nonatopic controls and that long eyelashes may be a part of the phenotype of the allergic patient.

Laron syndrome (primary growth hormone insensitivity): a unique model to explore the effect of insulin-like growth factor 1 deficiency on human hair.

BACKGROUND: Classical Laron syndrome is a recessive disease of primary insulin-like growth factor 1 (IGF-1) deficiency and primary growth hormone insensitivity. Affected children have, among other defects, sparse hair growth and frontal recessions. The hair is thin and easy to pluck. Young adults have various degrees of alopecia, more pronounced in males. OBJECTIVE: The aim of the present study was to investigate the effect of primary IGF-1 deficiency on hair structure. The study sample included 11 patients with Laron syndrome--5 children (2 untreated) and 6 adults (5 untreated). Hairs were examined by light and electron microscopy. RESULTS: The most significant structured defect, pili torti et canaliculi, was found in 2 young, untreated patients. Grooving, tapered hair and trichorrhexis nodosa were found in the remainder. IGF-1-treated patients had either none or significantly fewer pathological changes compared to the untreated patients. CONCLUSION: This is the first documentation of the role of primary IGF-1 deficiency on hair structure in human beings.

Handwashing patterns in two dermatology clinics.

BACKGROUND: The hands of health care workers are a major source of nosocomial infection. Studies conducted mainly in intensive-care units and emergency departments have shown low compliance with hand hygiene recommendation. OBJECTIVE: To study hand hygiene practices in outpatient dermatology clinics in Israel. METHODS: The fingers of 13 dermatologist physicians were sampled for bacterial cultures and their hand hygiene practices were monitored by two observers. In addition, 51 dermatologists attending a professional conference completed a questionnaire on hand hygiene practice. RESULTS: All the physicians' hands were found to be contaminated. Staphylococcus aureus was isolated in 9 cases (69.2%), a methicillin-resistant S. aureus in 1 (7.7%). Monitoring revealed 555 opportunities for handwashing; the average compliance was 31.4%. In the questionnaire, the main reasons given for poor hand hygiene were excessive work schedule (58%), lack of awareness (35.3%), reaction to disinfectants (17.7%) and lack of readily available facilities (15.7%). CONCLUSION: The hands of dermatologists are frequently colonized with microbial pathogens, but compliance with hand hygiene practice recommendations is low, despite a fairly high awareness of the importance. An active educational infection control program should be introduced in dermatology clinics.

[The current approach to hemangiomas and vascular malformations of the head and neck].

Though most hemangiomas do not need treatment, a significant minority are associated with complications and external deformities that demand intervention. Steroids play an important role in therapy, but not infrequently afford only partial and temporary benefit. Thanks to improvements in the surgical approach and equipment, hemostasis control devices and laser techniques, we can now treat patients who would otherwise go untreated. Moreover, in certain cases, we can now recommend earlier intervention, saving patients from years of living with deformities and the concomitant psychosocial problems. Vascular anomalies of the head and neck include venular, venous and arteriovenous malformations. These lesions are slow growing vascular ectasia that never involute spontaneously and almost always require intervention. Treatment includes laser therapy, injection of sclerosing agents, embolization through angiography and surgery, which in many cases is the only definitive treatment. We present the current treatment approach and describe our experience in the treatment of 16 patients.

Cutis marmorata telangiectatica congenita and hypospadias: report of 4 cases.

Cutis marmorata telangiectatica congenita (CMTC) is an uncommon sporadic congenital vascular anomaly. Of the 111 patients with CMTC examined in our clinic during the past 25 years, 4 were found to have hypospadias. All cases were sporadic. Although CMTC has been associated with various abnormalities, to our knowledge there are no reports in the literature of concurrent hypospadias.