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The article presents information on a case study of a 5 year old girl child for an exacerbation of her reactive airways disease. Medical history revealed that the child had been seen at age 2-5 years by the orthopedics service for a swollen right hand that was asymptomatic. Two months before that visit she had experienced a single left-sided focal seizure attributed to viral encephalitis. Examination at that time revealed a right hand that was larger than the left, and hypertrophy of the right third and fourth fingers. A diagnosis was made up films performed 2 years later showed that the lesions had grown proportionately to bony growth.

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Abstract Background There are neither sensitive nor specific laboratory tests for measuring disease activity in localized scleroderma (LS). Monitoring is done almost exclusively by clinical assessment. Our aim was to determine whether serum concentrations of TGFβ1 are a good biomarker of disease activity in children with LS. Methods 55 pediatric patients with LS were divided into sub-types according to their main lesion; morphea, generalized morphea, linear scleoderma affecting a limb or the face. The lesions were further categorized by overall clinical assessment into active, inactive, and indeterminate groups according to disease activity. Serum TGFβ1 concentration levels were measured by enzyme linked immunosorbent assay (ELISA), analyzed and correlated with disease subtypes and disease activity. Results The mean TGFβ1 concentration were significantly higher in the patient group (51393 ± 33953 pg/ml) than in the control group (9825 ± 5287 pg/ml) (P < 0.001). The mean concentration were elevated in all the disease subtypes, and did not correlate with disease duration or activity. Conclusion Serum concentration of TGFβ1 were elevated in patients with all subtypes of LS irrespective of clinical disease activity. Although TGFβ1 may play an important role in the pathogenesis of local skin fibrosis, circulating blood levels of molecules known to act locally may not be useful biomarkers of disease activity.

Purpose: Neonatal lupus erythematosus is caused by the transplacental passage of maternal autoantibodies. The aim of this study was to determine the risk of connective tissue disorders in mothers of children with cutaneous neonatal lupus erythematosus, as compared with the risk in mothers of children with congenital heart block, which is also often caused by maternal autoantibodies.Subjects and Methods: We prospectively studied all mothers of children with cutaneous neonatal lupus erythematosus during a 14-year period at the Hospital for Sick Children, Toronto, Ontario, Canada. We identified 28 mothers, of whom 24 were eligible for study. The health and antibody status of the mothers were determined at the birth of the child and at followup.Results: All mothers had anti-Ro antibodies at the time of birth. Initially 10 mothers were healthy and 14 mothers had either a defined (n = 9) or an undifferentiated (n = 5) autoimmune disorder. At a mean follow-up of 7 years, 13 (1 of whom had died) had a defined connective tissue disease, and 5 had an undifferentiated autoimmune disorder. Only 6 (25%) remained asymptomatic. By comparison, 36 (56%) of 64 mothers of children with congenital heart block were asymptomatic at follow-up (P <0.005).Conclusions: The majority of mothers of children with cutaneous neonatal lupus erythematosus had a defined or undifferentiated autoimmune disorder at the time of the child's birth, and others developed these conditions during follow-up. The health of these mothers appears to differ from that of mothers of children with congenital heart block. [ABSTRACT FROM AUTHOR]

For centuries, diapering has been a global practice. Most cultures around the world have historically implemented mechanisms to cover the genital area, both for privacy and to contain waste. The latest and most important innovation was the advent of the disposable diaper in the mid-twentieth century. Modern disposable diapers have considerably decreased the incidence of irritant diaper dermatitis through their design and construction. Disposable diaper use continues to grow globally. This article reviews the history of diaper practices worldwide., (© 2016 The International Society of Dermatology.)

Importance: Alopecia areata is an idiopathic cause of hair loss with limited therapeutic repertoire., Objective: To compare the efficacy and safety of a high- vs low-potency topical corticosteroid in pediatric patients., Design, Setting, and Participants: This single-center, randomized, blind, 2-arm, parallel-group, superiority trial was carried out over a 24-week period at a tertiary referral academic dermatology clinic at The Hospital for Sick Children in Toronto, Ontario, Canada. Forty-two children attending the outpatients clinic, 2 to 16 years of age with alopecia areata affecting at least 10% of scalp surface area, were eligible; 1 declined to participate. There were no withdrawals from the study. INTERVENTIONS FOR CLINICAL TRIALS: Patients were randomly assigned to receive clobetasol propionate, 0.05% cream, or hydrocortisone, 1%, cream. Patients applied a thin layer of the assigned cream twice daily to the areas of hair loss for 2 cycles of 6 weeks on, 6 weeks off, for a total of 24 weeks., Main Outcomes and Measures: The primary outcome was the change in scalp surface area with hair loss over 24 weeks following enrollment. RESULTS All participants were assessed at 6, 12, 18, and 24 weeks (except 1 participant who missed the 6-week visit). After adjusting for baseline hair loss, the clobetasol group had a statistically significant (P < .001) greater decrease in the surface area with hair loss, compared with the hydrocortisone group at all time points except at 6 weeks. One patient with extensive alopecia areata experienced skin atrophy that resolved spontaneously in 6 weeks. There was no difference observed in the number of patients with abnormal urinary cortisol at the beginning and the end of the study., Conclusions and Relevance: Topical clobetasol propionate, 0.05%, cream is efficacious and safe as a first-line agent for limited patchy childhood alopecia areata. TRIAL REGISTRATION clinicaltrials.gov Identifier: NCT01453686.

Background: Cutaneous disorders can be associated with a wide variety of dental manifestations that should be familiar to dermatologists., Objective: We sought to describe the development of the teeth, explain current dental terms, and review the dental manifestations of some dermatologic conditions., Methods: A MEDLINE search (1966-May 2007) was performed to find relevant articles pertaining to dental manifestations of dermatologic conditions., Results: Dental manifestations are associated with a wide variety of skin diseases that include genetic, infectious, inflammatory, and immune disorders., Limitations: The review is broad and focuses on commonly described manifestations., Conclusions: An appreciation and understanding of dental signs can aid in the diagnosis and treatment of many skin conditions.

Atopic is the most common of the dermatitides seen in infancy and childhood, but there are numerous other diseases that can mimic the skin findings. These include seborrheic dermatitis, immunodeficiency, and psoriasis in infancy; scabies, tinea corporis infection, perioral, nummular, contact, and molluscum dermatitis in childhood. It is sometimes extremely difficult to differentiate between ichthyosis and AD, and it is also important to differentiate AD from erythrodermic conditions including acrodermatitis enteropathica, biotin deficiency, and Netherton syndrome. A rare condition in children that may mimic AD is mycosis fungoides.

Background: To evaluate the clinical course and outcome of infants with Langerhans cell histiocytosis (LCH) involving skin and to estimate the incidence of progression to multi-system (M-S) disease in those with isolated skin involvement., Methods: A retrospective review was conducted on 22 LCH patients who were younger than 12 months at the onset of their skin eruption., Results: Twelve patients had isolated skin involvement at diagnosis and 10 were evaluable for progression. Four of the 10 (40%) evaluable patients progressed to multi-system (M-S) disease. Of the 10 patients with M-S disease at diagnosis, 5 had a history of a preceding skin eruption 2 to 13 months prior to diagnosis. Eleven of the 14 (79%) patients with M-S disease had risk organ involvement. The mortality rate of M-S disease was 50%., Conclusions: It is important for primary caregivers to recognize that isolated cutaneous LCH in infants is not always a benign disorder. The diagnosis of self-healing cutaneous LCH should only be made in retrospect. Careful, albeit non-invasive, follow-up is recommended to monitor for disease progression and development of long-term complications.

Alopecia areata, the alleged autoimmune process leading to nonscarring hair loss, is not uncommon. It has been classified as an acquired cause of alopecia; however, recently it has been reported in the neonatal period. We report 4 cases of congenital alopecia areata with follow-up from 3 to 5 years. The diagnosis was made clinically in all cases. All patients had prolonged periods of quiescence of hair loss ranging from 6 to 24 months. Treatments used included minoxidil 2% and a range of topical steroids including hydrocortisone 1%, betamethasone valerate 0.05%, fluocinonide 0.05%, and clobetasol propionate 0.05%. The best regrowth observed resulted from the use of clobetasol propionate 0.05%, giving full regrowth in 50% of those treated. Alopecia areata can occur at all ages and, thus, can be classified as both an acquired and a congenital disorder resulting in hair loss.

Background: Atopic dermatitis is a common condition, with a lifetime prevalence of approximately 10% to 20% among the Canadian population. A clear, practical, Canadian guideline for the management of these patients has been lacking., Objective: To provide primary-care physicians, pediatricians and dermatologists with the first practical and comprehensive set of Canadian recommendations for the management of atopic dermatitis., Methods: A group of Canadian dermatologists convened to review the current issues of diagnosis, treatment and international guidelines and adapt them to the Canadian context. The reviewers used the latest clinical trial data on atopic dermatitis, complemented by clinical experience, to develop the consensus recommendations found in this review., Results: In the present report, following a brief review of the epidemiology of and clinical diagnosis criteria for atopic dermatitis, the recommendations for treatment and management are detailed. These recommendations, which are intended to provide clinicians with a useful and valuable tool to help manage their patients with atopic dermatitis, are divided into the following sections: epidemiology, diagnosis, general measures/skin care, acute management of atopic dermatitis, long-term management/disease control, adjunct therapies, and considerations for switching between antiinflammatory therapies/handling treatment failure. General measures discussed include hydration with bathing and the use of moisturizers. Management strategies discussed include topical corticosteroids, topical calcineurin inhibitors, antihistamines and anti-infectives. A management algorithm is also presented.

A3-year-old boy presented to the Hospital for Sick Children with systemic symptoms and oropharyngeal and peripheral extremity changes suggestive of Kawasaki disease. He was found to have severe hypertension. Investigation for a catecholamine-secreting tumor was negative. Toxins were considered when the patient's 20-month-old brother presented with similar symptoms, and the boys were subsequently diagnosed with elemental mercury poisoning. We review the literature on mercury intoxication and discuss the historical context, clinical syndrome (acrodynia), treatment, and radiologic findings of this unusual diagnosis.

Acute febrile neutrophilic dermatosis (Sweet syndrome) has been reported in a few adults receiving all-trans retinoic acid for acute promyelocytic leukemia. The authors report a case of Sweet syndrome associated with the administration of all-trans retinoic acid for acute promyelocytic leukemia in a pediatric patient.

The autosomal recessive disorder lipoid proteinosis results from mutations in extracellular matrix protein 1 (ECM1), a glycoprotein expressed in several tissues (including skin) and composed of two alternatively spliced isoforms, ECM1a and ECM1b, the latter lacking exon 7 of this 10-exon gene (ECM1). To date, mutations that either affect ECM1a alone or perturb both ECM1 transcripts have been demonstrated in six cases. However, lipoid proteinosis is clinically heterogeneous with affected individuals displaying differing degrees of skin scarring and infiltration, variable signs of hoarseness and respiratory distress, and in some cases neurological abnormalities such as temporal lobe epilepsy. In this study, we sequenced ECM1 in 10 further unrelated patients with lipoid proteinosis to extend genotype-phenotype correlation and to add to the mutation database. We identified seven new homozygous nonsense or frameshift mutations: R53X (exon 3); 243delG (exon 4); 507delT (exon 6); 735delTG (exon 7); 785delA (exon 7); 892delC (exon 7) and 1190insC (exon 8), as well as two new compound heterozygous mutations: W160X/F167I (exon 6) and 542insAA/R243X (exons 6/7), none of which were found in controls. The mutation 507delT occurred in two unrelated subjects on different ECM1 haplotypes and may therefore represent a recurrent mutation in lipoid proteinosis. Taken with the previously documented mutations in ECM1, this study supports the view that exons 6 and 7 are the most common sites for ECM1 mutations in lipoid proteinosis. Clinically, it appears that mutations outside exon 7 are usually associated with a slightly more severe mucocutaneous lipoid proteinosis phenotype, but neurological features do not show any specific genotype-phenotype correlation.

Objectives: Terbinafine has been shown to be effective in tinea capitis, using different treatment durations. However, no direct comparison of treatment duration has previously been investigated. This randomized, double-blind, parallel-group, multicenter study was designed to assess the effect of terbinafine treatment duration on the outcome of Trichophyton tinea capitis in a North American population., Methods: A total of 176 patients with a clinical diagnosis of tinea capitis were enrolled in this study and treated with oral terbinafine (3-6 mg/kg/d) for 1, 2, or 4 weeks. All patients were to be followed until week 12. A total of 159 patients had culture-confirmed tinea capitis attributable to Trichophyton species and constituted the intent-to-treat population used for efficacy analysis (50, 55, and 54 patients in the 1-, 2-, and 4-week arms, respectively)., Results: At the end of study, effective treatment, defined as negative culture and low scores on signs and symptoms, was achieved in 56%, 69%, and 65% of patients who were treated with terbinafine for 1, 2, and 4 weeks, respectively. A negative culture was achieved in 60%, 76%, and 72%, respectively. Overall, the efficacy data showed that both the 2- and 4-week treatment regimens are clinically superior to the 1-week regimen. Terbinafine was well tolerated, and the incidence of adverse events showed no relationship to the duration of therapy., Conclusion: When efficacy, cost, and compliance are taken into consideration, 2 weeks of terbinafine therapy appears to be the optimal treatment duration for patients with Trichophyton tonsurans tinea capitis.

Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare, chronic skin condition that begins in early childhood. We present two children with ILVEN and arthritis, a previously undescribed association. We discuss the relevance of this association and suggest appropriate management for this arthritis.

We evaluated the self-esteem and quality of life of 47 children with morphea with the use of the Harter self-perception profile for children and Visual Analog Scale. Most children with morphea have normal self-worth and a high quality of life. Morphea, like some other childhood chronic illnesses, does not impair self-esteem.

Vulvitis circumscripta plasmacellularis (VCP) is a rare, benign vulvar disorder that is typically described in adult women. Our case occurred in an 8-year-old girl. The primary diagnostic concern was sexual abuse. VCP may also mimic lichen sclerosus, extramammary Paget's disease, pemphigus vulgaris, fixed drug eruption, squamous cell carcinoma, candidiasis, allergic contact dermatitis, and herpes simplex infection. Evaluation should include a biopsy because the histopathologic features of VCP are distinctive.

Introduction: Localized scleroderma (LS) can cause permanent disability, and there is no universally accepted effective treatment. Methotrexate (MTX) has been shown to be effective in the treatment of systemic sclerosis., Objectives: To determine the efficacy and tolerability of MTX and corticosteroid therapy in patients with LS., Methods: MTX, 0.3 to 0.6 mg/kg per week, was given to 10 patients (6 girls, 4 boys; mean age, 6.8 years; mean disease duration before starting treatment, 4 years) with active LS. In addition, pulse intravenous methylprednisolone, 30 mg/kg for 3 days monthly for 3 months, was given to 9 patients at the initiation of therapy., Results: One patient discontinued taking MTX after a month; the remaining 9 patients responded. The median time to response was 3 months (95% CI, 1.15-4.85). One responder discontinued taking MTX after a year because of leukopenia; the LS worsened within 2 months. In another patient LS flared up after 10 months and responded to an increased dose of MTX and intravenous methylprednisolone. At the last follow-up visit, all patients who continued to receive MTX therapy had inactive skin lesions., Conclusion: Treatment with MTX and corticosteroids appears to be effective in the treatment of LS and is generally well tolerated. A placebo-controlled study is necessary to confirm the efficacy of MTX therapy in LS.

Objective: Serum autoantibodies to fibrillin 1, the major component of microfibrils in the extracellular matrix, recently have been reported to occur in the tight skin mouse and in patients with systemic sclerosis, but not in patients with other connective tissue diseases. This study was undertaken to determine whether antifibrillin 1 antibodies could be detected in patients with localized forms of scleroderma., Methods: Sera from 50 patients with localized scleroderma (27 with linear scleroderma and 23 with morphea) and 51 normal controls were tested for IgG and IgM antifibrillin 1 autoantibodies, using a radioimmunoassay (RIA) and a human recombinant fibrillin 1 protein (rFbn-1)., Results: Both in patients with linear scleroderma and in those with morphea, mean levels of IgM and IgG binding to rFbn-1 were significantly higher than in controls. Eight patients with linear scleroderma (30%) and 6 patients with morphea (26%) had IgG autoantibodies to fibrillin 1 (rFbn-1) by RIA, compared with 3 controls (6%) (P = 0.006 and P = 0.022, respectively). No correlations between antifibrillin 1 antibodies and active skin disease or antinuclear antibody positivity were found., Conclusion: Autoantibodies to fibrillin 1 occur in patients with both forms of localized scleroderma (linear scleroderma and morphea). The clinical and pathogenetic significance of this autoimmune response remains to be determined.