Pediatric Dermatology Department Research Articles

Association Between Increased Body Mass Index (BMI) and Atopic Dermatitis in Children Attending a Tertiary Referral Center: A Case-Control Study.

Background Atopic dermatitis (AD) and obesity represent chronic diseases, with growing worldwideprevalence, that rely on a common pathophysiological background: perpetual inflammation. Moreover, AD is considered more and more to be a beyond-the-skin disease with various associated comorbidities. This study aimed to investigate a potential link between overweight/obese status and AD in children. Methods A case-control study was performed on 130 AD patients and 130 exact age and sex match controls that attended the Pediatric Dermatology Department of Colentina Clinical Hospital. Based on the weight (in kilograms) and height (in centimeters), the body mass index (BMI), and the corresponding age and gender percentiles were assessed in both groups; study participants were divided as normal-weight, underweight, overweight, or obese. AD severity was evaluated using the Scoring Atopic Dermatitis Index (SCORAD), and quality of life impairment was assessed with the Dermatology Life Quality Index (DLQI).Descriptive statistics, t-tests, and logistic regression with odds ratios (OR) and associated 95% confidence intervals (CI) were used for data analysis. Results A statistically significantly higher BMI was identified in the AD group compared to controls (p=0.027). The relative risk for overweight/obese status in the AD group compared to controls was three times more frequent (OR 3.61, 95% CI 1.45-10.3, p<0.01). Additionally, the increased BMI in the AD group correlated significantly with disease severity as determined by SCORAD (p<0.05), with a relative risk for overweight/obese status in the moderate-severe AD subgroup being 20 times more frequent as compared to mild AD (OR 20.4, 95% CI 6.53-90.7, p<0.001). Conclusions To our knowledge, this is the first study to evaluate the correlation between AD and BMI in Romanian children. Statistically significant correlations between increased BMI, AD development, and AD severity in children were identified in our study population. This study's small sample size and single-center study design represent possible limitations. Additional, larger, multicentric studies are required to establish a more precise correlation between AD and obesity. Physicians should be aware of this potential association in order to perform obesity screening in AD children for more appropriate multidisciplinary management of such patients.

O03 Langerhans cell histiocytosis: case series of presentations to dermatology in the BRAF era

Abstract Langerhans cell histiocytosis (LCH) is a rare disease driven by MAPK pathway mutations. This condition is on a spectrum, from single-system to life-threatening multisystem disease. Identification of the BRAF mutation is leading to improved understanding and targeted therapies. However, there is limited information on genetic status, phenotype and dermatological features. Fourteen patients were seen in the Paediatric Dermatology department in period 2015–2022: 11 males and 3 females; 9 patients had multisystem LCH; 8 patients had cutaneous manifestations of LCH; and 4 had limited cutaneous LCH. Six patients were diagnosed in the department and typically had multiple incorrect diagnoses and treatments prior to review. Clinical features varied from widespread skin involvement, scalp/groin intertrigo, flexural ulcerations and telangiectatic papules. Eight had LCH diagnosed in other tissues by oncology and referral advice on cutaneous symptoms were sought. Most patients have required input from dermatology throughout treatment. Biopsies were taken when unclear diagnosis and/or pathology would influence treatment. Several consultations revealed benign pathologies including keratosis pilaris and dermatitis requiring topical treatment advice only. All five patients with BRAF v600 mutation had multisystem disease including diabetes insipidus, adrenal insufficiency, gut and bone lesions. Two patients with severe LCH and BRAF mutations are being treated successfully with dabrafenib. Our series demonstrates the important role of dermatology in the diagnosis and management of LCH. This series confirms clinical heterogeneity and an association of BRAF V600E mutation with multiorgan disease. Ongoing work should expand our knowledge of the relationship of genetic alterations with clinical phenotypes and comorbidities.

P09 A case series of anetodermic pilomatrixomas

Abstract Pilomatrixoma is a benign tumour that arises from the base of the hair follicle matrix. Anetodermic changes overlying the tumour occur in only 2% of the cases. The aim of this study is to evaluate a case series of paediatric patients who presented with anetodermic pilomatrixoma to our Paediatric Dermatology department. A retrospective analysis of all our paediatric cases with a clinical diagnosis of anetodermic pilomatrixoma within the last 4 years was made. Data were collected according to age at time of presentation, suspected clinical diagnosis, histopathological features, treatment and postoperative outcomes. Five cases were identified. All were referred as a rapidly growing tumour. The median age of onset was 15 years. The male : female ratio was 3 : 2; one presented on the neck, two on the upper limbs and two on the back. Two out of three patients reported that the lesions were painful. Clinical suspicion of anetodermic pilomatrixoma (large, atrophic, wrinkled lesion overlying a firm subcutaneous mass) was made prior to surgical removal. Four were confirmed histopathologically. Simple excision was carried out without any postoperative complications. There have been no recurrences. Anetodermic pilomatrixoma, also referred to as pseudobullous pilomatrixoma, is a rare variant of pilomatrixomas, occurring more frequently in the upper body. It presents with identifiable features but should be differentiated from other skin tumours. Pain can be a symptom due to the size of the lesion. Surgical removal is the gold standard treatment with a low recurrence rate.

P25 Becker naevus is associated with neurocognitive abnormalities in a substantial proportion of children

Abstract Becker naevus and Becker naevus syndrome are mosaic disorders caused in 60% of cases by postzygotic variants in the gene ACTB which encodes β-actin. We observed several patients with Becker naevus and coexistent neurocognitive abnormalities and began to study this potential association. All 51 patients seen in the last 5 years with a differential diagnosis of Becker naevus in electronic and/or photographic records were identified retrospectively. Clinical notes, photographs, radiology and histopathology were reviewed. Twenty-one patients were removed due to a different or doubtful final cutaneous diagnosis. Neurocognitive diagnoses were included only when made by a clinician, all external to the Paediatric Dermatology department. All but three patients had had their neurodevelopment assessed. Nine of the 27 patients assessed (33.3%) had a neurocognitive diagnosis, and all nine were male. Two children had autistic spectrum disorder (ASD), four had attention deficit hyperactivity disorder (ADHD) and two children had both diagnoses. MRI scans had been performed in six of the nine patients, and two had abnormal findings. A very substantial proportion of this cohort of patients with a definite diagnosis of Becker naevus have an independent diagnosis of ASD or ADHD, which in line with all-cause ASD/ADHD appears to be commoner in males. This is a new description. Germline genetic disorders caused by gain- or loss-of-function variants in ACTB are associated with neurocognitive abnormalities. Genotyping is ongoing to examine any association between the neurocognitive phenotype and mosaic ACTB variants in this clinical cohort.

Persistent umbilicated papules on the left ear and neck in an adolescent boy.

Pediatric DermatologyVolume 40, Issue 3 p. 562-564 PHOTOQUIZ Persistent umbilicated papules on the left ear and neck in an adolescent boy Shibhani Sudheer Hegde MD, FRGUHS, Corresponding Author Shibhani Sudheer Hegde MD, FRGUHS [email protected] orcid.org/0000-0002-5415-3702 Department of Pediatric Dermatology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India Correspondence Shibhani Sudheer Hegde, Department of Pediatric Dermatology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India. Email: [email protected]Search for more papers by this authorSahana M. Srinivas DNB, DVD, FRGUHS, Sahana M. Srinivas DNB, DVD, FRGUHS orcid.org/0000-0002-4356-2535 Department of Pediatric Dermatology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, IndiaSearch for more papers by this authorRajalakshmi Tirumalae MD, Rajalakshmi Tirumalae MD Department of Pathology, St John's Medical College and Hospital, Bengaluru, Karnataka, IndiaSearch for more papers by this author Shibhani Sudheer Hegde MD, FRGUHS, Corresponding Author Shibhani Sudheer Hegde MD, FRGUHS [email protected] orcid.org/0000-0002-5415-3702 Department of Pediatric Dermatology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India Correspondence Shibhani Sudheer Hegde, Department of Pediatric Dermatology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India. Email: [email protected]Search for more papers by this authorSahana M. Srinivas DNB, DVD, FRGUHS, Sahana M. Srinivas DNB, DVD, FRGUHS orcid.org/0000-0002-4356-2535 Department of Pediatric Dermatology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, IndiaSearch for more papers by this authorRajalakshmi Tirumalae MD, Rajalakshmi Tirumalae MD Department of Pathology, St John's Medical College and Hospital, Bengaluru, Karnataka, IndiaSearch for more papers by this author First published: 22 May 2023 https://doi.org/10.1111/pde.15221Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL No abstract is available for this article. Volume40, Issue3May/June 2023Pages 562-564 RelatedInformation

Pediatric alopecia areata following COVID‐19 infection

Pediatric alopecia areata following <scp>COVID</scp>‐19 infection

A Retrospective Study on Alopecia Areata in Children: Clinical Characteristics and Treatment Choices

Background: Although children are affected frequently with alopecia areata (AA), data are limited on clinical characteristics and treatment choices. Materials and Methods: We retrospectively reviewed the records of the pediatric dermatology department over a 12-year period to identify children with AA. Clinical data were collected. Results: Three hundred and sixty-four children with AA were identified, aged 1–12 years, 214 males and 150 females. The mean age of onset was 6.6 years (±3.3). The disease presented with patches on the scalp in the majority (90.7%), whereas only 6 children had alopecia totalis or universalis. The most commonly prescribed treatment was topical steroids (69.1%), followed by the combination of topical steroids and minoxidil 2% (14.3%). Oral steroids were prescribed in only 16 children. Follow-up at 3 months was available for only 70 children and the majority (84.3%) had some hair regrowth. Hair regrowth was unrelated to the number of plaques (p = 0.257), disease location (p = 0.302), and atopy (p = 0.999). Hair regrowth only correlated with the type of treatment (p = 0.003) with potent topical and intralesional steroids giving the best results. Conclusion: AA usually presents with a mild form in children, and potent topical steroids are the mainstay of treatment.

Chilblains in children in the time of COVID-19: New evidence with serology assay.

In a previous report in Pediatric Dermatology, we described chilblains-like lesions in four pediatric patients. From April 18 to May 10, 2020, 45 children presented to our Pediatric Dermatology department with similar acral lesions. The clinical appearance ranged from red to violaceous macules and dusky, purpuric plaques on the heels, soles and lateral margin of the feet, often accompanied by painful edema, consistent with chilblains.

Classification of aplasia cutis congenita: a 25-year review of cases presenting to a tertiary paediatric dermatology department.

Aplasia cutis congenita (ACC) is a rare, congenital disorder characterized by localized or widespread absence of skin at birth with heterogeneous clinical presentation. The classification proposed by Frieden in 1986 is widely used. To establish whether, 34years on, the Frieden classification still meets the needs of dermatologists. We conducted a retrospective chart review of all patients with a diagnosis of ACC presenting over a 25-year period to a tertiary paediatric dermatology department. We compiled demographic data, clinical characteristics (e.g. number, location and morphology of the lesions), imaging and genetic results where available, and other associated abnormalities, and grouped them according to the Frieden classification. For Type 6 ACC (Bart syndrome) we reviewed neonatal photographs of all babies born with epidermolysis bullosa (EB) over 5years. Excluding Type 6, there were 56 children with ACC. The scalp was involved in 82.1%, and Type 1 was the commonest type. Over 5years, 13 of 108 neonates (12%) with EB were born with the appearance of Type 6 ACC. Two children did not fit Frieden's original classification and one had a previously undescribed association of ACC with cleft lip/palate-ectodermal dysplasia 1 syndrome. We conclude that the Frieden classification remains valid with some modifications. Type 3 ACC probably represents a mosaic RASopathy syndrome, while Type 7 could cover nongenetic ACC attributable to trauma. Type 8 should be subdivided into two subgroups: teratogenic and infective. Type 9 covers at least four subgroups. The classification will continue to evolve as new genes and pathomechanisms emerge.

Case 1: Peripheral Intravenous Catheter Site Swelling/Abscesses in the Neonate

1. Daniel Loeb, MD* 2. Jonathan J. Lee, MD† 3. Silvia Song, MD† 4. Arivarasan Karunamurthy, MD† 5. Robin P. Gehris, MD‡ 6. Sharmilarani H. Nanda, MD§ 1. *Department of Pediatrics and 2. ‡Department of Pediatric Dermatology, Department of Pediatric Teledermatology, and Department of Dermatology, Children’s Hospital of Pittsburgh of UPMC, Pittsburgh, PA 3. †Department of Dermatology, University of Pittsburgh Medical Center, Pittsburgh, PA 4. §University of Pittsburgh Physicians, Pittsburgh, PA Pediatrics in Review came into being as I was beginning my pediatric training. The journal encouraged and educated me through a mostly US-based career as well as during a year in Europe and six years in central Africa. The educational content of Pediatrics in Review informed my care of tens of thousands of patients, and my first contribution to the journal was published in 1993. It has been a privilege to serve a ten-year term on the editorial board and to help guide the content of Index of Suspicion. This month, I turn 65. While I am not retiring, I am transitioning away from my editorial responsibilities with the journal. May each of you be as blessed as I have been by the career-enhancing education and encouragement offered by Pediatrics in Review ! Philip R. Fischer, MDAssociate Editor, Index of Suspicion An infant girl is born via cesarean delivery at 38 weeks’ gestation to a 21-year-old gravida 2, para 1 mother whose antenatal course is complicated by a recent primary vaginal herpes simplex virus (HSV) type 2 infection detected 5 days before the onset of labor. After cesarean delivery the baby briefly receives positive pressure ventilation via bag valve mask and has Apgar scores of 8 and 8 at 1 and 5 minutes, respectively. Her birthweight is 3,015 g. She requires continuous positive airway pressure for 2 days for respiratory distress. She is treated with ampicillin and gentamicin for 7 days for suspicion of clinical sepsis and with acyclovir for 10 days due to spontaneous rupture of membranes and the presence of active maternal genital …

Pediatri Departmanlarından Dermatoloji Polikliniğine yönlendirilen konsültasyon olgularının analizi: Retrospektif Çalışma

Objective: There are a few studies in the literature about dermatology consultations requested from Pediatrics Department. In the present study, it was aimed to assess the clinical features, pre-diagnosis and dermatologic diagnosis of patients consulted to the Dermatology Department from Pediatrics Department, and also to detect possible different approaches between the clinics.Methods: The list of dermatology consultations requested from Pediatrics Department between 1 September 2016 and 1 September 2017 were retrospectively evaluated by means of pre-diagnosis, dermatological diagnosis and unit consultations.Results: The mean age of patients was 7.4±5.3 years. Of the 628 patients, 52.1% were males, and 85% of the consultations were requested from outpatient clinics and 15% were from the inpatient clinics. Among all consultations 28.7% general pediatrics unit and 17.5% pediatric emergency unit required consultations. The most common diagnosis reported from Dermatology Department were eczema (28.2%), viral diseases (13.2%), parasitic diseases (8.4%) and fungal diseases (5.4%). Although, the ratio of an accurate pre-diagnosis was significantly higher in consultations requested from the inpatient clinics than those from outpatient clinics(p&amp;lt;0.001), 42.7% of the patients were referred to Dermatology Outpatient Clinic with an incorrect pre-diagnosis.Conclusion: Almost half of the patients, who were referred to Dermatology consultation, were diagnosed with incorrect pre-diagnosis. It was seen that pediatricians were difficult to recognize dermatoses. The awareness of Pediatric Dermatology Department should be raised for both specialties.

Pigmented lesions: when should I worry?

Abstract Pigmented lesions in children are very common. The majority are benign melanocytic naevi that develop during childhood but, rarely, sinister lesions such as melanoma and atypical Spitz naevi can also occur. The group of benign pigmented lesions includes benign acquired and congenital melanocytic naevi, blue naevi, halo naevi and Spitz naevi. This article will enable clinicians to be aware of the concerning features, use the ABCDE criteria appropriately, understand how to use the available diagnostic tools and when to refer to a Paediatric Dermatology department for further opinion and management.

Child with flagellate skin eruptions

Pediatric DermatologyVolume 35, Issue 5 p. 676-677 PHOTOQUIZ Child with flagellate skin eruptions Sandipan Dhar MD, DNB, FRCP, Corresponding Author Sandipan Dhar MD, DNB, FRCP doctorsandipan@gmail.com Department of Pediatric Dermatology, Institute of Child Health, Kolkata, West Bengal, India Correspondence Sandipan Dhar, MD, DNB, FRCP, Department of Pediatric Dermatology, Institute of Child Health, Kolkata, West Bengal, India. Email: doctorsandipan@gmail.comSearch for more papers by this authorSahana M. Srinivas DNB, DVD, FRGUHS, Sahana M. Srinivas DNB, DVD, FRGUHS orcid.org/0000-0002-4356-2535 Department of Pediatric Dermatology, Indira Gandhi Institute of Child Health, Bangalore, Karnataka, IndiaSearch for more papers by this authorSubhra Dhar MD, Subhra Dhar MD SRL Diagnostics, SRL Reference Laboratory, Kolkata, West Bengal, IndiaSearch for more papers by this author Sandipan Dhar MD, DNB, FRCP, Corresponding Author Sandipan Dhar MD, DNB, FRCP doctorsandipan@gmail.com Department of Pediatric Dermatology, Institute of Child Health, Kolkata, West Bengal, India Correspondence Sandipan Dhar, MD, DNB, FRCP, Department of Pediatric Dermatology, Institute of Child Health, Kolkata, West Bengal, India. Email: doctorsandipan@gmail.comSearch for more papers by this authorSahana M. Srinivas DNB, DVD, FRGUHS, Sahana M. Srinivas DNB, DVD, FRGUHS orcid.org/0000-0002-4356-2535 Department of Pediatric Dermatology, Indira Gandhi Institute of Child Health, Bangalore, Karnataka, IndiaSearch for more papers by this authorSubhra Dhar MD, Subhra Dhar MD SRL Diagnostics, SRL Reference Laboratory, Kolkata, West Bengal, IndiaSearch for more papers by this author First published: 13 September 2018 https://doi.org/10.1111/pde.13521Citations: 2Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article.Citing Literature Volume35, Issue5September/October 2018Pages 676-677 RelatedInformation

Nasal Colonization by Staphylococcus aureus in Children with Atopic Dermatitis

Background: The cutaneous and nasal microbiota, especially Staphylococcus aureus (S. aureus), plays a key role in atopic dermatitis (AD). Although this association is well known, it is difficult to establish whether colonization by these bacteria is a cause or a consequence of the disease. Objectives: The primary objectives of our study are to determine the prevalence of nasal colonization by S. aureus and its relationship with disease severity in children with AD, as well as to describe the magnitude of the association between the two. Methods: This was an analytical case-control study in children with and without AD. Participants were recruited consecutively upon presenting to the Department of Pediatric Dermatology at the General University Hospital until reaching the estimated sample size of 157 cases and 314 controls (N=471; ratio 1:2). Results: The prevalence of S. aureus nasal colonization was significantly higher in children with AD than in those without (32.5% vs. 23.9%; odds ratio (OR) 1.5, 95% CI 1.0, 2.3; p=0.047). Prevalence of colonization was not higher in the children with severe AD than in those with less severe AD. In multivariable analysis, an independent association (borderline significant) between nasal colonization by S. aureus and AD persisted after adjusting for all other study variables (OR 1.5; 95% CI 0.9, 2.6; p=0.11). Conclusions: Our paper provides further evidence of an association between atopy and nasal colonization by S. aureus, though not between nasal colonization and AD severity. The role of nasal S. aureus and the microbiota in people with AD is a controversial but crucial area of study. Greater knowledge of this topic could be clinically applicable in AD patients.

Lesion Morphology in Multifocal Infantile Hemangiomas.

Multifocal infantile hemangiomas (MIHs; previously called neonatal hemangiomatosis) can be associated with extracutaneous hemangiomas. We observed different morphologic types of hemangiomas in children with MIHs and sought to find out whether they are related to the clinical course. This was a retrospective study of 103 infants with MIHs and a control group of 261 age-matched patients with solitary focal infantile hemangiomas (IHs) seen at an academic pediatric dermatology department between 2004 and 2014. Two morphologic subtypes of hemangiomas were identified: miliary focal hemangiomas (MFHs; small, lens shaped) in 58 of 103 MIH patients (56.3%), and classical nonmiliary focal IHs (NMIHs; larger, irregularly shaped) in 17 of 103 patients (16.5%). MIHs featuring both types (mixed type) were observed in 28 of 103 patients (27.2%). MFH lesions were significantly smaller (mean 5.3mm [range 1-20mm] vs 22.0mm [range 2-100mm]), more numerous (23.4±27.3 [range 5-175] vs 7.4±2.8 [range 5-15] p<0.001), and occurred up to an older age (6.0±5.8months [range 0-27months] vs 3.8±2.6months [range 0-9months]) than NMIHs. There was a weakly positive correlation between the number and presence of extracutaneous IHs in children with MFHs. Significantly more children with MIHs were delivered preterm than those with solitary IHs. The number of IHs correlates inversely with their size. MFHs follow a clinical course different from that of classical IHs, are associated with prematurity, and may confer greater risk of extracutaneous hemangiomas. Miliary hemangiomas thus appear to present a separate IH subset requiring special attention.

Anatomical patterns of infantile hemangioma (IH) of the extremities (IHE)

From 18% to 30% of infantile hemangiomas (IH) are located on the extremities (IHE). They can be divided into localized, segmental, and minimal or arrested growth (IH-MAG) subtypes. We sought to correlate localization of IHE with the anatomy of the arterial vascular supply. All children with segmental IHE and IH-MAG presenting to our department of pediatric dermatology from 2002 to 2015 were evaluated. Hemangiomas were mapped and their patterns were analyzed. Most IHE were unilateral (105/109). Two thirds (68/109) were located on the upper, and one third (41/109) on the lower extremities. Distal locations were more frequently affected. Segmental IHE were more common (upper extremities 83.8%; lower extremities 56.1%) than IH-MAG (16.2% and 43.9%, respectively). Ulceration occurred in 5.5%. Localization of IHE was found to correspond to supply areas of embryonic arterial variants. This was a retrospective study. Only segmental IHE and IH-MAG were evaluated. Angiographic studies were not performed. The location of IHE may be related to variant anatomy of arterial supply during embryo fetal development. We hypothesize that this contributes to temporary regional tissue hypoxia during early fetal development, which is a known stimulus for the proliferation of hemangioma stem cells.

Otologic Manifestations of Autosomal Recessive Congenital Ichthyosis in Children

BackgroundFew studies have investigated ear involvement in nonsyndromic autosomal recessive congenital ichthyosis (ARCI). ObjectivesTo assess the type and frequency of otologic manifestations of ARCI in patients under follow-up at the pediatric dermatology department of our hospital. Materials and methodsWe prospectively studied the presence of ear pain, ear itching, tinnitus, otitis, cerumen impaction, accumulation of epithelial debris, and hearing loss. Daily hygiene measures, topical treatments, medical-surgical interventions, and frequency of visits to an ear, nose, and throat (ENT) specialist were noted in the patients’ medical records. Ear examination and hearing tests were performed in all cases. ResultsTen patients were studied: 2 had a self-healing collodion baby phenotype and 8 had ichthyosis. There was mention of otologic manifestations in the records of all 8 patients with ichthyosis (100%); 6 of these patients (75%) had abnormalities in the external auditory canal examination and 2 (25%) had conductive hearing loss. Our findings are limited by the small number of patients studied, all of whom were younger than 19 years. ConclusionsThe involvement of both dermatologists and ENT specialists in the management of patients with ichthyosis is crucial to ensure the application of the best therapeutic and preventive measures. More studies are needed to assess the prevalence and impact on quality of life of ear involvement in patients with ichthyosis and to determine the optimal interval between ENT visits for these patients.

Manifestaciones otológicas en los niños con ictiosis congénitas autosómicas recesivas

BackgroundFew studies have investigated ear involvement in nonsyndromic autosomal recessive congenital ichthyosis (ARCI). ObjectivesTo assess the type and frequency of otologic manifestations of ARCI in patients under follow-up at the pediatric dermatology department of our hospital. Materials and methodsWe prospectively studied the presence of ear pain, ear itching, tinnitus, otitis, cerumen impaction, accumulation of epithelial debris, and hearing loss. Daily hygiene measures, topical treatments, medical-surgical interventions, and frequency of visits to an ear, nose, and throat (ENT) specialist were noted in the patients’ medical records. Ear examination and hearing tests were performed in all cases. ResultsTen patients were studied: 2 had a self-healing collodion baby phenotype and 8 had ichthyosis. There was mention of otologic manifestations in the records of all 8 patients with ichthyosis (100%); 6 of these patients (75%) had abnormalities in the external auditory canal examination and 2 (25%) had conductive hearing loss. Our findings are limited by the small number of patients studied, all of whom were younger than 19 years. ConclusionsThe involvement of both dermatologists and ENT specialists in the management of patients with ichthyosis is crucial to ensure the application of the best therapeutic and preventive measures. More studies are needed to assess the prevalence and impact on quality of life of ear involvement in patients with ichthyosis and to determine the optimal interval between ENT visits for these patients.

G70(P) Radiological investigations of midline infantile haemangiomas overlying the scalp or spine: 5 year single centre experience

Aim To review the outcomes of investigations for midline infantile haemangiomas (IH) overlying the head or spine in a tertiary paediatric dermatology department. Methods Case notes and radiological results were reviewed retrospectively for all patients with a clinical diagnosis of HI overlying the midline of the scalp, neck or spine seen over the 5 year period October 2009–14. Results 43 patients were identified (31 girls). The median age at the first specialist clinic appointment was 5 months (range 2–23 months). In 26 patients the IH were located on the scalp, in five on the back of the neck, in four overlying the thoracic spine and in eight overlying the lumbar spine and or sacrum. 24 lesions were more than 5 cm and 19 less than 5 cm in size at the time of the first appointment, 10 of which were plaque-type IH. 18/43 patients had MRI of either the brain or spine following the initial assessment. MRI was normal in 15 patients, including in all lumbosacral lesions. In two cases of plaque-type IH a diagnosis of PHACES syndrome was confirmed. In one child where the IH was a palm-sized lesion over the thoracic spine, MRI showed extension of the IH into the spinal canal, with complete compression of the cord between T4 and T9. The child was neurologically asymptomatic at the first assessment, but the result prompted treatment with propranolol. Conclusion This retrospective series of midline IH seen over a five year period in a tertiary centre has identified a significant underlying lesion in a child with a thoracic spinal IH. The current literature from experts in the field recommends MRI for lumbosacral lesions. Our internal guidelines will now dictate an MRI of the underlying CNS in all infants with a midline IH on the scalp, neck or spine.

Pediatric dermatology inpatient consultations: a retrospective study.

To describe the profile of inpatient consultations seen in department of pediatric dermatology at a tertiary care children's hospital. The authors performed a retrospective review of all pediatric dermatology inpatient consultation data from medical record section over a period of 42mo from January 2010 through June 2013. All children 18y and below were included in the study. A total of 486 inpatient consultation services were given by pediatric dermatology department. About 124 (25.5%) of consultations were in the age group of infancy followed by 103 (21.1%) consultations in the school going age children. Most frequent consultation request was from general pediatrics (49.4%) followed by pediatric intensive care (37.9%). The most common diagnostic categories included cutaneous infection (115, 23.7%), emergency skin conditions (62, 12.8%), genodermatosis (58, 11.9%) and skin disorders secondary to systemic illness (55, 11.3%). This study highlights the spectrum of conditions that clinicians must be aware that can be seen in general pediatric wards and intensive care unit. This study also highlights the role of pediatric dermatologist in giving care to hospitalized children.