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Σάββατο 2 Ιανουαρίου 2021

Paediatric Dermatology

Genetics of Vascular Malformations: Current Perspectives
Kin Fon Leong

Indian Journal of Paediatric Dermatology 2021 22(1):1-11

For decades, vascular anomalies are categorized as either vascular tumors or malformations based on their onset, clinical course, radiologic, and histologic features. Owing to the heterogeneity of vascular anomalies, they are frequently misdiagnosed. With the advent of massively parallel next-generation sequencing, the molecular landscape of vascular anomalies is rapidly evolving and recent discoveries have shed light on the genetic basis and classification of these vascular disorders. The genotype-phenotype correlation will provide a more precise classification of vascular anomalies and form the basis for future targeted pharmacologic therapy. Thus far, inhibitor of mTOR, AKT1, and PIK3CA (sirolimus, miransertib, and alpelisib) have promising clinical results. In vascular malformations, majority of sporadic cases are due to somatic mutations that disrupt the main endothelial receptor intracellular signaling pathways, i.e., PIK3CA-AKT-mTOR, RAS - MAPK – ERK, and SMAD signaling pathways. Most of the sporadic vascular malformations are caused by somatic mutations that are acquired after fertilization, instead of being inherited from his parents. In general, this type of mosaicism is not inherited, except when the mutation affects the gonads.


Cosmetic Procedures in Adolescents: What's Safe and What Can Wait


Indian Journal of Paediatric Dermatology 2021 22(1):12-20

Teenagers between 13 and 19 years are increasingly seeking cosmetic procedures. They are suffering from anxiety, depression, and low self-esteem as a result of an obsession with body image and celebrity culture, fueled by social networking sites. Teenagers seek cosmetic procedures most commonly for traumatic scars, acne and acne scars, pigmentary abnormalities, hypertrichosis, hirsutism, and tattoo removal. They demand plastic surgery for nose deformities, breast asymmetry, ear abnormalities, and congenital deformities. The physical, emotional, psychological, social, ethical, and legal aspects must be considered while counseling adolescents. Not every teenager seeking cosmetic surgery is well suited for a procedure, and teens must demonstrate emotional maturity and an understanding of the limitations of these procedures and the risks involved. There should be a 3-month cooling-off period, followed by another consultation, which should be done in the presence of a parent. Only very essential surgery should be performed, giving realistic expectations on the outcome of procedures, as they rely too much on physical appearance to gain confidence. A psychiatric evaluation is essential to rule out body dysmorphic disorders in those repeatedly seeking treatment for minor defects. Sometimes, procedures are necessary to avoid social withdrawal and loss of self-esteem. Proper informed consent should be taken, explaining the benefits, limitations, and risks involved. Ideally, teenagers should not receive cosmetic or surgical procedures unless there are compelling medical or psychological reasons to do so. A successful aesthetic procedure in a mature teenager can have a positive influence, whereas surgery on an immature, psychologically unstable adolescent can have an adverse impact. This review discusses what is safe and what can wait, still there is limited evidence. There is a strong need for guidelines for the use of cosmetic surgery on children and teenagers.


Role of Elimination Diet in Atopic Dermatitis: Current Evidence and Understanding


Indian Journal of Paediatric Dermatology 2021 22(1):21-28

Atopic dermatitis (AD) is a fairly common dermatosis of childhood. In many cases, parents are concerned that food allergy plays a role in their child's symptoms and they ask whether practicing elimination diets would help in controlling the symptoms. In this review, we try to provide concise answers to the questions raised by the parents, apart from addressing the myths associated with elimination diets in AD and provide scientific evidence in favor of or against the common notions. A comprehensive English-language literature search for the role of elimination diet in AD across multiple databases (PubMed, EMBASE, MEDLINE, and Cochrane) for keywords (alone and in combination) was performed. MeSH as well as non-MeSH terms such as “AD,” “diet elimination,” “treatment,” “prevention,” “evidence,” “blanket elimination,” and “specific exclusion diet” were taken into consideration. There is level I evidence to support specific exclusion diets in preselected and screened patients, but there is insufficient evidence to recommend strict (blanket) elimination diets.


Pattern of Dermatoses in Small Children in a Tertiary Care Hospital


Indian Journal of Paediatric Dermatology 2021 22(1):29-36

Aims: The aim of the study was to study the pattern of dermatoses in children up to 5 years of age. Settings and Design: It is an observational study done in a tertiary care hospital. Subjects and Methods: A total of 505 patients, aged up to 5 years, attending the department of dermatology, venereology, and leprosy for a duration of 18 months from January 2017 to June 2018 were included in the study. Statistical Analysis Used: All results were statistically analyzed by IBM SPSS software and Chi-square test was used to compare the associations. Results: Preschoolchildren (75.44%) formed the majority. Males (54.65%) outnumbered females. Infections constituted the most (45.94%) followed by eczema (21.98%), hypersensitivity disorders (14.25%), nevi (4.55%), papulosquamous (3.76%), pigmentary (3.76%), physiological and transient noninfective neonatal conditions (3.56%), sweat gland (2.97%), hair disorders (0.59%), and other dermatoses (2.97%). Conclusions: Infections/infestations constituted the most among the dermatoses in children up to 5 years of age. As they are preventable, educating the parents, guardian, care takers, and teachers in the preschool about communicable diseases can limit their transmission and improve the well-being of children.


Clinical Spectrum of Cutaneous Adverse Drug Reactions in Pediatric Population in East Coast of Andhra Pradesh: An Observational Study
VV V Satyanarayana, Suruthi Purushothaman, Bonu Chandipriya

Indian Journal of Paediatric Dermatology 2021 22(1):37-42

Context: Cutaneous adverse drug reaction (CADR) among pediatric population is underappreciated and often misdiagnosed in clinical settings. Furthermore, epidemiologic data on only cutaneous adverse reactions among this population are limited. Aim: The aim of this study is to analyze the CADRs with reference to the pattern, causative drugs, its subgroup analysis, and other clinical characteristics among children. Settings and Design: This was an observational, hospital-based study over a period of 12 months. Materials and Methods: The CADRs occurring and referred to the inpatient and outpatient department of dermatology were actively monitored and the collected reports were analyzed for CADR pattern, drug groups, clinicodemographic profile, and severity of adverse drug reaction (ADR). Statistical Analysis Used: Data were documented in Microsoft Excel and analyzed using SPSS Version 20.0. Results: A total of 33 CADRs were documented. Most of the ADRs (40%) occurred between the age of 11 and 15 years. Antimicrobials comprised the major group of drugs causing ADRs (42.4%). Maculopapular rash (54.5%) and urticaria (15.2%) were the most common type of ADR. A single case of death due to anticonvulsant-induced toxic epidermal necrolysis had been observed in the study period. There were more occurrences of ADRs with multiple drugs compared to single-drug therapy. About 80% of reactions were of mild to moderate. Conclusion: Knowledge of the pattern and the offending drug helps in better diagnosis, management, reduced complications in this population, and also helps in preventing recurrences, thereby helps in promoting the safe use of drugs, which can overall impact the quality of health care among children positively.


Acne Fulminans: A Case Report and Review of Literature


Indian Journal of Paediatric Dermatology 2021 22(1):43-47

Acne fulminans (AF), a variant of acne vulgaris, is a rare disease that occurs after treatment of AF with oral isotretinoin. Less than 200 cases of AF have been reported in literature, mostly in young men and all with varying clinical presentations. A thorough search of literature was performed for AF using PubMed, MEDLINE, EMBASE, and UK PubMed Central electronic databases. Pathogenesis is not clearly established. However, it is most likely due to hypersensitivity reactions to sebum or bacterial antigens. It presents as sudden eruption of friable, hemorrhagic crusting overlying the ulcers, plaques, and tender nodules, usually on the trunk, with or without systemic symptoms such as malaise, fever, and arthralgia a few days after initiation of oral isotretinoin therapy. Laboratory abnormalities including raised erythrocyte sedimentation rate, altered hematological profile, and radiological evidence of osteolytic bone lesions may be found. Treatment involves the use of oral steroids with subsequent addition of oral isotretinoin. Many other immunosuppressive therapies have been tried.


Clinical and Dermoscopic Spectrum: Novel Findings in Interesting Cases


Indian Journal of Paediatric Dermatology 2021 22(1):48-51

Uncommon, interesting clinical entities presenting in dermatological practice often challenges the diagnostic skills of a trained dermatologist. A non-invasive tool for recognition of these conditions is especially relevant in the paediatric population and dermoscopy plays a pivotal role in diagnosing these cases. A spectrum of novel clinical and dermoscopic findings is reported in four interesting cases. (vitiligo ponctue, cutaneous larva migrans, zosteriform angiokeratoma, linear milia en plaque).


Norwegian (Crusted) Scabies Involving Eyelids and Conjunctiva


Indian Journal of Paediatric Dermatology 2021 22(1):52-55

Scabies is a parasitosis caused by the mite sarcoptes scabiei var. hominis, with crusted scabies being more contagious than classic scabies because of a larger mite burden. It can lead to an epidemic of classic scabies if not diagnosed early and treated accordingly. We report a 10-year-old boy who presented with a complaint of an inability to open his left eye and irritation in the right eye. Examination showed the presence of multiple crusted lesions throughout the body, including face and eyelids, and a yellowish patch in the right bulbar conjunctiva. The histopathological evaluation of the specimen of the conjunctival lesion confirmed it to be sarcoptes infestation. Face and eyelids involvement are very rarely in crusted scabies and only a few cases have been reported in the past. Our report demonstrates conjunctiva as an unusual site of involvement in these cases.


Localized Pseudoxanthomatous Mastocytosis of Vulva


Indian Journal of Paediatric Dermatology 2021 22(1):56-58

Cutaneous mastocytosis (CM) is a mast cell proliferative disorder that is common in the pediatric age group. Common entities are urticaria pigmentosa, solitary mastocytoma, telangiectasia macularis eruptive perstans. and diffuse CM. Pseudoxanthomatous mastocytosis presents in the form of yellowish nodular lesion resembling xanthoma and is a rare variant and not reported much in the literature. We, hereby report a rare case of localized vulvar pseudoxanthomatous mastocytosis in a 10-year-old female child.


Curious Subcutaneous Swellings: A Case of Pediatric Myocysticercosis


Indian Journal of Paediatric Dermatology 2021 22(1):59-61

Cysticercosis is a parasitic infection caused by the larval stage of the tape worm, Cysticercus cellulosae, chiefly affecting the subcutaneous tissue, central nervous system, orbit, and the skeletal muscles. It occurs in human due to ingestion of contaminated water or raw meat of pig. We hereby present a case of a 6-year-old female presenting with multiple subcutaneous swellings of 3 months' duration, associated with painful movement and associated with a history of on and off fever and malaise. Local part ultrasonography revealed multiple cystic lesions in the muscular plane, with peripheral streak of calcification indicative of scolex. Fine-needle aspiration cytology of the lesions showed revealed degenerated and viable polymorphs, macrophages, few lymphocytes, and foreign body giant cells with foci of necrosis, with occasional hooklets of parasite, with findings suggestive of parasitic infection in neck with acute on chronic inflammation. Computed tomography (CT) scan of brain was normal. Thus, clinical, radiological, and cytological findings confirmed the diagnosis of myocysticercosis. This case is presented because of the rareness of its occurrence.



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Medicine by Alexandros G. Sfakianakis,Anapafseos 5 Agios Nikolaos 72100 Crete Greece,00302841026182,00306932607174,alsfakia@gmail.com,
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