Danielle Marcoux

Eosinophilic Pustular Folliculitis (Ofuji Disease) in Childhood: A Review of Four Cases

Abstract: Four children under 2 years of age were treated at our hospital in the last three years with a recurrent vesiculopustular eruption of the scalp, variably extending to the face and limbs. All cases followed a cyclical pattern. Three of the children had a moderate response to topical steroids, and one went into remission after a two‐month course of erythromycin. Ethnic origin may be an important predisposing factor. Histologically, all patients showed a moderate mixed inflammatory infiltrate with numerous eosinophils centered around hair follicles. Peripheral white blood cell count showing leukocytosis with eosinophilia was observed in those cases measured, but no consistent immunologic abnormalities could be identified.

Therapeutic Patient Education in Atopic Dermatitis: Worldwide Experiences

Therapeutic patient education (TPE) has proven effective in increasing treatment adherence and improving quality of life (QoL) for patients with numerous chronic diseases, especially atopic dermatitis (AD). This study was undertaken to identify worldwide TPE experiences in AD treatment. Experts from 23 hospitals, located in 11 countries, responded to a questionnaire on 10 major items. Patients in TPE programs were mainly children and adolescents with moderate to severe AD or markedly affected QoL. Individual and collective approaches were used. Depending on the center, the number of sessions varied from one to six (corresponding to 2 to 12 hours of education), and 20 to 200 patients were followed each year. Each centers education team comprised multidisciplinary professionals (e.g., doctors, nurses, psychologists). Evaluations were based on clinical assessment, QoL, a satisfaction index, or some combination of the three. When funding was obtained, it came from regional health authorities (France), insurance companies (Germany), donations (United States), or pharmaceutical firms (Japan, Italy). The role of patient associations was always highlighted, but their involvement in the TPE process varied from one country to another. Despite the nonexhaustive approach, our findings demonstrate the increasing interest in TPE for managing individuals with AD. In spite of the cultural and financial differences between countries, there is a consensus among experts to integrate education into the treatment of eczema.

Trichothiodystrophy associated with photosensitivity, gonadal failure, and striking osteosclerosis

Three new unrelated cases of PIBIDS (Photosensitivity, Ichthyosis, Brittle, sulfur-deficient hair [trichothiodystrophy], Impaired intelligence, Decreased fertility, and Short stature) are reported. Decreased survival of skin fibroblast lines after UVB exposure was found. All three male patients had hypogonadism and primary end-organ gonadal failure. Striking osteosclerosis was present in all three patients. To the best of our knowledge the third patient is the first reported case of a black man with PIBIDS.

Large congenital melanocytic nevi and neurocutaneous melanocytosis: One pediatric center's experience

BACKGROUND Large congenital melanocytic nevi (LCMN) predispose to neurocutaneous melanocytosis (NCM), which is associated with significant morbidity and mortality. OBJECTIVE To identify risk factors for NCM in patients with LCMN and suggest guidelines for their management. METHODS Medical records of patients with LCMN were reviewed at Sainte-Justine Hospital between 1980 and 2006. Presence of multiple satellite nevi and posterior midline location were evaluated as risk factors for NCM using chi-square test. Magnetic resonance imaging scans were reviewed by a neuroradiologist. RESULTS Twenty-six of 52 patients underwent radiologic investigation. Six of 26 (23%) had NCM. Patients with this condition are more likely to have multiple satellite nevi (100% vs 50%, P = .03) and have a trend to posterior midline location of their LCMN (100% vs 60%, P = .08). Patients with NCM are more likely to have both multiple satellite nevi and posterior midline location (100% vs 25%, P = .002). Radiologic findings are also presented. LIMITATIONS This was a retrospective case series with imprecise chart data in 38% of cases. CONCLUSION The presence of multiple satellite nevi alone or with associated posterior midline location of LCMN is associated with a higher risk of NCM. We recommend magnetic resonance imaging testing before 4 months of age in patients with these features.

Therapeutic patient education in children with atopic dermatitis: position paper on objectives and recommendations

Poor adherence is frequent in patients with atopic dermatitis (AD), leading to therapeutic failure. Therapeutic patient education (TPE) helps patients with chronic disease to acquire or maintain the skills they need to manage their chronic disease. After a review of the literature, a group of multispecialty physicians, nurses, psychologists, and patients worked together during two international workshops to develop common recommendations for TPE in AD. These recommendations were structured as answers to nine frequently asked questions about TPE in AD: What is TPE and what are its underlying principles? Why use TPE in the management of AD? Who should benefit from TPE in AD? How can TPE be organized for AD? What is the assessment process for TPE in AD? What is the evidence of the benefit of TPE in AD? Who are the people involved in TPE? How should TPE be funded in dermatology? What are the limits of the TPE process?

Deep cutaneous fungal infections in immunocompromised children

BACKGROUND Life-threatening infections from ubiquitous fungi are becoming more prevalent in adults and children because of the increased use of immunosuppressive agents and broad-spectrum anti-infective drugs. Extremely low birth weight premature neonates and patients with a disrupted epidermal barrier are also at increased risk. Lethality is high, particularly with delayed diagnosis. As cutaneous lesions are often the first manifestation of such infections, early recognition of suspicious lesions is crucial to decrease associated morbidity and mortality. The clinical features of deep cutaneous fungal infection (DCFI) in immunocompromised children deserve special attention. OBJECTIVES This study aimed to characterize our pediatric patients with DCFI, the causative fungi, and the associated risk factors. METHODS A medical record review was conducted of pediatric patients with DCFI treated at out institution using data retrieved from the hospitals pathology and microbiology database (1980-2008). RESULTS In all, 26 patients with DCFI were identified (9 girls and 17 boys) ranging in age from 1 day to 18 years (mean age: 8 years), the majority of whom had a hematologic disorder. All patients were immunocompromised, 90% were receiving broad-spectrum antibiotics, and 50% had severe neutropenia (absolute neutrophilic count < or = 500 x 10(6)/L). Necrotic ulcers (42%) and papules (34%) represented the most frequent lesion morphology. Fungal species were identified by culture in 20 (87%) of 23 patients tested and were observed histopathologically in 20 of 23 patients tested. Aspergillus was identified in 12 (44%) patients and Candida in 9 (33%). The other species included Fusarium (one), Exserohilum rostratum (one), Alternaria (one), Zygomycetes (two), and Blatomycetes (one). All but two patients received systemic antifungal therapy; wide surgical excision was performed in 13. Infection resolved in 20 (77%), whereas 6 (23%) died of disseminated infection. LIMITATIONS This study was limited by the small number of cases and the retrospective nature of the collected data. DISCUSSION DCFI should rank high in the differential diagnosis of any suspicious skin lesions in immunocompromised children. Early biopsies should be performed for histopathology and microbiological analysis, as lethality is high if appropriate treatment is delayed.

Short- and long-term outcome of linear morphoea in children

Linear morphoea (LM) is a rare fibrosing disorder of the limbs or the face that may cause functional disability and severe aesthetic sequelae. Despite a wide range of therapeutics reported for LM, there is currently a lack of consensus on the optimal therapy. Little is known about the long‐term outcome of this disease.

Connective tissue nevi in children: Institutional experience and review

BACKGROUND Connective tissue nevi (CTN) are circumscribed hamartomas of the skin in which there is an abnormal mixture of normal components of the dermis that may be sporadic or associated with syndromes such as Buschke-Ollendorff, tuberous sclerosis, and Proteus. OBJECTIVE We sought to specify the clinical and histologic features of CTN in childhood and to propose a diagnostic approach and updated classification. METHODS This was a retrospective study in a tertiary pediatric outpatient population, accessing clinical and histopathological records. RESULTS We classified 114 cases of CTN from 1980 to 2008. LIMITATIONS The majority of cases were confirmed by histopathological examination. Therefore, our series excludes many CTN that were not biopsied. In addition, follow-up was variable. CONCLUSION Our series demonstrates the usefulness of a modified classification for CTN. Biopsy should be done when clinical diagnosis is uncertain, or in multiple lesions. When biopsy is performed it should include normal-appearing skin for comparison and, in Buschke-Ollendorff syndrome, limited anterior-posterior x-rays of the hands, wrists, feet, ankles, knees, and pelvis instead of a full skeletal survey.

Canadian Practical Guide for the Treatment and Management of Atopic Dermatitis

BackgroundAtopic 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.ObjectiveTo provide primary-care physicians, pediatricians and dermatologists with the first practical and comprehensive set of Canadian recommendations for the management of atopic dermatitis.MethodsA 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.ResultsIn 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 anti-inflammatory 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.

Therapy of ulcerated hemangiomas.

Background: Cutaneous ulceration is the most common complication of infantile hemangiomas (IHs) seen in a pediatric dermatology practice. Objective: The most effective treatments in our experience are compared to those in the current literature. Methods: The study was a retrospective chart review of therapy of 169 ulcerated IHs at a tertiary care pediatric hospital and a literature review. Results: Combination therapy was the rule. Local wound care was required in all, pain management in 72%, pulsed dye laser in 42%, infection control in 38%, diminution of the hemangioma through systemic therapy in 36%, and suppression of bleeding in 2%. Limitations: A retrospective review compared to a case-control study has inherent bias. In addition, our cases were all at a tertiary referral center. Conclusion: All ulcerated IHs benefit from local barrier creams or dressings. Pulsed dye laser, antibiotics, topical morphine 0.1% in hydrogel, topical becaplermin, and, most importantly, systemic therapy (especially propranolol) to reduce the hemangioma may be useful.