Educational intervention and atopic dermatitis: impact on quality of life and treatment.

BACKGROUND: Educational intervention (EI) could improve understanding of atopic dermatitis (AD) and adherence to treatment, decreasing severity, and improving quality of life (QoL). OBJECTIVE: This study aims to evaluate the influence of an EI on the severity of the disease and on the QoL in children with AD. METHODS: A controlled clinical trial was performed, including children up to 14 years of age with AD. Patients were allocated into control group (CG), which received usual guidelines on AD during the outpatient visit, and a study group (SG) that, in addition to the usual guidelines, participated in the EI. The severity of AD was assessed by Scoring Atopic Dermatitis (SCORAD) and Eczema Area and Severity Index (EASI). QoL was assessed by the Children's Dermatology Life Quality Index and the Dermatitis Family Impact Questionnaire. RESULTS: Twenty-seven participants were included in the CG and 21 in the SG. There was a decrease in the median value for the QoL of children in the SG after the intervention (p = 0.04), as well as in the caregiver's (p = 0.04). In the CG, the median QoL of children and caregivers remained unaltered, for caregivers the median value for the QoL was equal throughout first and second evaluation (p = 0.32). In the SG, EASI values decreased after the intervention (p = 0.04), as well as SCORAD (p = 0.04). The CG did not show any decrease in the values of EASI (p = 0.88) scores nor of SCORAD scores (p = 0.82). CONCLUSION: The EI rendered a decrease in severity of the disease and improvement in the QoL of patients and their caregivers.

Parry Romberg disease with En Coup de Sabre Scleroderma: Effect of tocilizumab on periodontal bone inflammation.

Parry Romberg disease and En Coupe de Sabre Scleroderma are frequently associated disorders that affect the face and can cause severe aesthetic and functional impairment. Systemic immunosuppression is the gold standard of first-line treatment in the pediatric rheumatology standpoint although it is often delayed in the pediatric dermatology clinics and more often used in cases of refractory neurological impairment. We report on a case with dental root resorption and severe periodontal bone inflammation detected on magnetic resonance imaging, which was successfully treated with the anti-IL-6 agent tocilizumab.

The musculoskeletal impairment negatively impacts the quality of life of children and adolescents with psoriasis.

BACKGROUND: The severity of nail disease, the presence of arthralgia and fatigue are predictors of development of psoriatic arthritis (PsA) in patients with psoriasis (Pso). In children, little is known about the musculoskeletal (MSK) impairment in patients with Pso and its effect on health-related quality of life (HRQoL). OBJECTIVES: To determine the frequencies of pain and MSK inflammation (i.e., arthritis, enthesitis, and sacroiliitis) among children and adolescents with Pso and its relationship to HRQoL and fatigue. METHODS: Pediatric patients with Pso underwent a rheumatologic physical examination to evaluate synovitis, enthesalgia, sacroiliac joint (SIJ) pain and tender points of fibromyalgia. The core set of domains recommended by the GRAPPA - OMERACT to be measured in PsA studies was assessed. Ultrasound (US) was performed in clinical cases of enthesitis, and magnetic resonance imaging (MRI) was performed in cases of SIJ pain. RESULTS: Forty-three participants (10 ± 2.9 years old) were evaluated. Pain on palpation of the entheses was observed in 10 (23.2%) patients and pain on SIJ palpation was observed in 3 (7%). No patient presented with synovitis; one presented with enthesitis on US, but MRI did not confirm sacroiliitis in any case. Patients with MSK pain had greater skin disease severity (PASI 5.4 vs. 2, p <  0.01), worse fatigue, and lower HRQoL scores on all instruments used. The estimated risk of HRQoL impairment was eight times higher in the presence of MSK pain, which was an independent predictive factor. With a NAPSI greater than 30, the probability of pain was greater than 80%. CONCLUSION: MSK pain is frequent among children with Pso, related to the severity of skin and nail disease, and negatively affects HRQoL. The typically used complementary exams might not detect the inflammatory process caused by Pso.

Colorimetric analysis of images in the follow-up of infantile hemangiomas.

BACKGROUND/OBJECTIVES: Infantile hemangiomas (IH) are common vascular tumors that appear early in life, have a rapid proliferative phase and slowly involute. There are no standardized ways to evaluate the regression of these lesions. We propose a colorimetric analysis of photographs to allow a more precise determination of IH treatment response and involution. METHODS: Patients 1-10 months of age with superficial or mixed IH were included. The lesions were managed with 0.5% topical timolol ophthalmic solution. Patients were followed for 16 weeks with 6 evaluations each. Photographic images were taken with a red and green circle placed beside each hemangioma. The photographs were treated as to equalize the size, color, and brightness among them based on the colors of the two circles. A grading scale was established based on the color of the patient skin (0) and the color of the hemangioma at the beginning of treatment (100) by pixel analysis using Adobe Photoshop® software. RESULTS: A total of 17 patients 1 to 10 months of age were included, of whom 16 were girls (94%). Fourteen lesions were superficial, and 3 were mixed IH. The median time prior to initiation of treatment was 105 days (44-232). All lesions showed some degree of clearing. The mean of lightening of color intensity observed was of 45% (17%-74%) over the period of follow-up. CONCLUSIONS: The colorimetric analysis of the digital images allowed an accurate and objective evaluation of IH clearing.

Cutaneous manifestations in patients with Wiskott-Aldrich syndrome submitted to haematopoietic stem cell transplantation.

INTRODUCTION: Wiskott-Aldrich syndrome (WAS) is an X-linked primary immunodeficiency caused by a mutation of the WAS protein gene. This protein actively participates in important cellular processes, and its presence is related to diverse clinical manifestations, including cutaneous alterations. The classical triad of WAS consists of recurrent infections, thrombocytopaenia with small platelets and atopic dermatitis (AD)-like lesions. OBJECTIVE: To evaluate the frequencies of cutaneous manifestations in patients with WAS prior to haematopoietic stem cell transplantation (HSCT). RESULTS: Twenty-four boys diagnosed with WAS and treated with HSCT between 1992 and 2007 were included. The characteristic triad of WAS occurred in 46% of patients. Before HSCT, the most frequent cutaneous manifestations included eczema similar to AD (71%), followed by petechiae and/or ecchymosis (58%) and cutaneous infections (17%). CONCLUSIONS: Cutaneous manifestations in patients with WAS are frequent, especially those similar to the eczema found in AD.

An inflammatory reaction surrounding molluscum contagiosum as possible manifestation of immune reconstitution inflammatory syndrome in HIV infection.

Highly active antiretroviral therapy can restore specific immune responses and control of microorganism infections in human immunodeficiency virus-positive patients. This immune recovery may cause an inflammatory reaction to microbial and autoimmune antigens known as immune reconstitution inflammatory syndrome. We describe a clinical case with an intense inflammatory response surrounding molluscum contagiosum after highly active antiretroviral therapy. The clinical and laboratory findings suggested that the reaction was due to immune reconstitution inflammatory syndrome occurring during a period of immune recovery in a child with acquired immune deficiency syndrome.

Behçet disease in a child--emphasis on cutaneous manifestations.

The diagnosis of Behçet disease is based upon clinical criteria because of the lack of pathognomonic laboratory findings. Recurrent episodes of oral and genital ulcerations, skin lesions, and ocular manifestations are seen. The disease may also involve the central nervous system, gastrointestinal tract and, less frequently, the large vessels. In general, manifestations occur in the third or fourth decade of life and are not common in children. Therefore few data concerning this age group have been found in the literature. In this study we report a child with Behçet disease beginning at 1 year of age whose cutaneous manifestations were exuberant acne-like and folliculitis-like lesions, which were crucial for diagnostic confirmation.