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   Table of Contents - Current issue
Coverpage
April-June 2021
Volume 22 | Issue 2
Page Nos. 99-194

Online since Wednesday, March 31, 2021

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EDITORIAL  

Indian Journal Of Paediatric Dermatology climbs a new height! Highly accessed article p. 99
Sandipan Dhar
DOI:10.4103/ijpd.ijpd_38_21  
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REVIEW ARTICLES Top

Exanthema in pediatric dermatology - A confusing galaxy of myriad diseases Highly accessed article p. 100
Carlo Gelmetti
DOI:10.4103/ijpd.ijpd_144_20  
In the medical literature, exanthem is the name given to a widespread rash that is usually accompanied by systemic symptoms such as fever, malaise, and headache. The combination of these factors, for example, the mobility of human populations, the onset of new diseases such as COVID and the continuous availability of new drugs, make today the interpretation of the exanthems more and more difficult. In this article, we will revise briefly the conceptual problems that, during many years, have contributed to hinder its comprehension. At the end, two tables will help physicians to orientate in this field.
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Biologics in pediatric dermatology p. 107
Manjyot Gautam, Ratnakar Shukla
DOI:10.4103/ijpd.ijpd_24_21  
Biologics are the molecules of protein, produced by recombinant DNA technology that are used in various diseases to target the specific points in the immunopathogenesis of the diseases without interfering with rest of the pathogenetic pathways and thereby expected to have a lesser side effects profile when compared to conventional immunosuppressant. With the advent of biologic therapy, the treatment of various systemic and cutaneous diseases, especially autoimmune diseases, has been revolutionized; biologicals are goal-directed lethal weapons in treatment armamentarium of a dermatologist; they are a major breakthrough and game changer in the field of dermatology therapeutics or you can say in general as a therapeutic option. While adults have been enjoying the benefits of biologics in treating skin conditions such as psoriasis, eczema, and hidradenitis suppurativa for last few years, but the use of biologics in the pediatric population is still limited because of unknown long-term safety profile and absence of large-scale studies. Thankfully, times are changing, and Biologics are slowly being approved for pediatric use too, in coming future dermatologists will be able to assess which pathways, in particular, are overactive and then prescribing the exact biological as per the need of the patient. In this review, a brief description is given of different biologic agents that are known currently. An extensive literature search was done; all clinical trials, randomized double-blind or single-blind controlled trials, open-label studies, retrospective studies, reviews, case series, and case reports concerned with the use of biologics in various pediatrics dermatoses were screened. The selected articles were retrieved; the final manuscript was prepared, analyzed, and presented in a narrative fashion.
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ORIGINAL ARTICLES Top

Scurvy in children: Down but not out p. 118
Sukhjot Kaur, Jatinder Singh Goraya
DOI:10.4103/ijpd.IJPD_102_20  
Background: Scurvy, a common disease of the past, has become rare in the modern era. Because of lack of awareness regarding its clinical and radiological manifestations, diagnosis of scurvy is often missed or delayed resulting in unnecessary and potentially harmful diagnostic and therapeutic interventions. Dermatological manifestations are a very important component of the clinical picture and can suggest the diagnosis to the unsuspecting physicians. The objectives were to describe the clinical presentation of scurvy in children in the modern era and to highlight its dermatological manifestations. Methods: This was a retrospective chart review of children diagnosed with scurvy over a period of 10 years at a tertiary care hospital. Results: Eleven children, 6 girls and 5 boys, aged from 21 months to 16 years with scurvy were identified. All the children had an underlying disorder that predisposed them to scurvy, with cerebral palsy in 10 and autism spectrum disorder with celiac disease in one child. All had presented with musculoskeletal complaints of irritability, limb pain, painful restriction of movements, and in some cases, swelling of the affected limb. Dermatological signs consisting of various combinations of perifollicular hyperkeratosis and hemorrhages, petechiae, ecchymosis, corkscrew hair, and bleeding gums were present in 10 children. Characteristic radiographic changes were seen in all the 8 children who underwent this examination. All children responded dramatically to treatment with oral Vitamin C. Conclusions: Awareness of clinical and radiographic signs of scurvy allows early diagnosis and treatment of scurvy. Dermatological manifestations are the most noticeable and helpful clues to the diagnosis of scurvy.
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A comparative study in treatment of childhood vitiligo 0.03% tacrolimus versus 0.03% tacrolimus and topical steroids p. 123
Anandan Venkatesan, Rashmi Sriram, Sindhuja Balaji, TK Anandhi
DOI:10.4103/ijpd.IJPD_110_19  
Background: Vitiligo is an idiopathic, acquired cutaneous achromia, characterized by circumscribed milky white macules. It develops before the age of 20 years in 50% of the patients and before the age of 10 years in 25% of the patients. Methods: This was a 2-year, prospective, comparative study conducted in vitiligo patients with 1 year of active intervention. Fifty patients in the age group of 2–14 years were enrolled in the study. Randomization was performed according to computer-generated random code. Patients with code A received treatment with 0.03% tacrolimus alone and patients with code B received treatment with 0.03% tacrolimus and topical steroids. The primary efficacy variable was the Vitiligo Area Severity Index (VASI), and the secondary efficacy parameter includes the Physician's Global Improvement Score which was computed at the end of the study. The statistical analysis was carried out using statistical software STATA 6.0. Results: Out of these 50 patients, 47 patients completed the study for a period of 1 year. The area-wise VASI reduction and VASI reduction in vitiligo types were analyzed in patients with code A and code B. The mean Physician's Global Assessment Score was 3.041 in the tacrolimus group, and it was 3.261 in the tacrolimus with steroid group. Conclusion: This shows that 0.03% tacrolimus can be effectively used as topical monotherapy in patients with localized stable vitiligo without the adverse effects of topical steroids.
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Vulvar lichen sclerosus in children: A prospective study over a 3 year period p. 131
Kavitha Athota, Seetharam Anjaneyulu Kolalapudi
DOI:10.4103/ijpd.ijpd_153_20  
Context: Vulvar lichen sclerosus (VLS) is a chronic inflammatory disease affecting prepubertal girls and postmenopausal women. In prepubertal girls, it causes anxiety in parents and affects quality of life considerably. There is a paucity of data about VLS in children from India. Aims: We aimed to study the demographic and clinical profile of VLS in children. Settings and Design: It is a prospective study over a period of 3 years from October 2017 to September 2020. Materials and Methods: All the children up to the age of 15 years with VLS, attending to our outpatient department between October 2017 and September 2019, were enrolled in the study after taking informed consent from the parents/guardian. The age, duration, clinical features, and comorbidities were noted. Biopsy was done only in clinically doubtful cases. All the cases were treated with topical clobetasol 0.05% ointment once daily for 3 months, and tacrolimus 0.03% or pimecrolimus 1% ointment was given as maintenance therapy. Cases were followed up for a period of 6 months–3 years. Results: Twenty-five girls presented with VLS during the study period. The mean age was 8.44 years, and the mean duration at presentation was 1.14 years. Pruritus was present in 14 (56%); constipation and dysuria were noted in 2 each. Classical figure-of-eight appearance was observed in only 4 cases; atrophy and erythema were noted in 11 cases and 3 cases, respectively. All symptoms improved in 3–4 weeks after treatment and depigmentation completely subsided in 8 cases by 3 months. Limitations: The diagnosis of VLS was made clinically in most cases. Dermoscopy was not done. Biopsy was done in only clinically doubtful cases. Conclusions: VLS in children needs special attention as it causes anxiety in parents thinking it as vitiligo. Pruritus is the common symptom. Ultrapotent topical steroid helps in the management. It may not resolve completely at puberty.
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COMMENTARY Top

Diet in Pediatric Dermatology p. 136
Sahana M Srinivas
DOI:10.4103/ijpd.ijpd_10_21  
Relation between dietary habits and pediatric dermatoses is well established in literature. Every dermatologist needs to have a thorough evidence-based knowledge of all the skin conditions that are affected by food either directly or indirectly, especially in today's world, where patients are looking for holistic solutions for all their ailments. Nutritional deficiencies, metabolic conditions, dermatitis herpetiformis, and urticaria are well-known entities related to food. Apart from these, a few skin conditions such as atopy, acne, and psoriasis have some correlation to food intake. A thorough knowledge of diet and its effect on dermatological conditions is important where alternative systems lay a lot of stress on the kinds of food that may be consumed in certain skin conditions. Parents should be counseled about the myths related to dietary modifications as this may lead to nutritional deficiencies in children.
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RESIDENT CORNER Top

Mongolian spots p. 141
Neha Sud, Resham Vasani
DOI:10.4103/ijpd.ijpd_49_21  
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CASE REPORTS Top

Superadded chromomycosis in recessive dystrophic epidermolysis bullosa: An easily misdiagnosed entity p. 145
Gaurav Dash, Swetalina Pradhan
DOI:10.4103/ijpd.IJPD_47_20  
Recessive dystrophic epidermolysis bullosa (RDEB) (Hallopeau Siemens) is one of the most severe forms of epidermolysis bullosa (EB) with generalized vesiculation over the skin and mucous membrane resulting in extensive scarring with exuberant granulation tissue. Because of widespread erosions, patients are prone to secondary infection. We are reporting a 10-year-old male child having RDEB who presented with a nonhealing ulcer with verrucous growth over bilateral knees for 2 years which was considered as part of the disease by many dermatologists and was later diagnosed to be superadded chromoblastomycosis based on histopathology. We are highlighting this case to increase awareness among dermatologists regarding the occurrence of superadded deep fungal infections like chromoblastomycosis in patients of RDEB which is usually missed or misdiagnosed as part of the disease.
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An atypical manifestation of hand-foot and mouth disease involving the genitalia p. 148
Bharathi Ravikumar, Rajesh Munusamy, Manobalan Karunanandhan
DOI:10.4103/ijpd.IJPD_113_20  
Hand-foot and mouth disease (HFMD), an acute viral illness, is caused by the Coxsackievirus (CV) that commonly affects young children. We report a case of a 15-month-old female child with painful erosions over the genitalia, following which she developed erythematous macules over the palms and soles. Viral polymerase chain reaction was positive for CV. She was diagnosed to have atypical HFMD. HFMD with atypical manifestations involving the genitalia as initial presentation is a quite unusual or under-reported entity. Prompt recognition of atypical features of HFMD aids in early diagnosis and defers detailed evaluation.
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Ichthyosiform lesion at birth demystifying familial short stature p. 151
Chanchal Kumar, Sindhu Sivanandan, Nishad Plakkal
DOI:10.4103/ijpd.IJPD_129_20  
Conradi-Hünermann syndrome is a rare genetic condition characterized by skeletal malformations, skin abnormalities, cataracts and short stature. We report a term female infant presenting with a diffuse ichthyosiform erythroderma at birth. Physical examination also showed sparse lusterless hair with cicatricial alopecia, dystrophic nails, facial dysmorphism, and flexion contracture of fingers. Radiological examination revealed a shortening of the right femur and humerus, vertebral defects, scoliosis, and epiphyseal stippling of long bones on the right side. The infant's sister and mother had short stature and bony joint swellings. A clinical diagnosis of X-linked Conradi–Hünermann syndrome was considered based on clinical suspicion and radiological survey. Dermatologists and pediatricians should consider this diagnosis in children with suggestive features.
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Lipschutz ulcer – Nonsexual acute genital ulceration in a 10-year-old girl p. 154
SR Radhika, Kanathur Shilpa, HV Shree Lakshmi, TN Revathi
DOI:10.4103/ijpd.IJPD_80_20  
Acute genital ulcers are painful and distressing. Lipschutz ulcer (LU), also known as ulcus vulvae acutum, is a rare and underdiagnosed entity presenting as an acute painful vulvar ulcer in young women. Although the etiology is not known, recent reports have associated it with infections such as Epstein–Barr virus, cytomegalovirus (CMV), Mycoplasma pneumoniae, Mycoplasma fermentans, mumps, and parvovirus B19. The diagnosis is made by exclusion, after ruling out the usual causes of genital ulcerations such as sexually transmitted diseases, autoimmune causes, trauma, drug reactions, and local manifestations of systemic illnesses. We report the case of a 10-year-old girl who developed flu-like symptoms, followed by painful vulvar ulcers within 2 days. Lipshutz ulcer must br considered among the causes of genital ulcers.
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Co-occurrence of tuberous sclerosis with port wine stain p. 157
Rashmi Agarwal, S Suganya, BS Chandrashekar, Sahana M Srinivas
DOI:10.4103/ijpd.ijpd_179_20  
Tuberous sclerosis complex is a rare autosomal dominant neurocutaneous disorder characterized by the presence of multiple tumors influencing diverse body frameworks including the central nervous system, skin, eyes, heart, lungs, kidney, and bones. It is described by cutaneous changes, neurologic conditions, and the presence of hamartomas in numerous organs. The treatment of these patients requires a multidisciplinary approach. A port-wine stain is characterized as a telangiectatic macule that manifests during childbirth and stays all throughout life. They are associated with many syndromes. This article reports a rare case of co-occurrence of tuberous sclerosis with port-wine stain in a 2-year-old baby.
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Successful treatment of recalcitrant generalized pustular psoriasis in a young child with interleukin-36 receptor antagonist mutation with secukinumab monotherapy p. 160
Henrietta Albela, Sabeera Begum, Kin Fon Leong
DOI:10.4103/ijpd.ijpd_3_21  
Generalized pustular psoriasis (GPP) is a severe, life-threatening form of psoriasis. Deficiency of interleukin (IL)-36 receptor antagonist has been found in a subset of patients with GPP, which is usually more difficult to treat with conventional treatment. We report a case of a 4-year-old Malaysian-Chinese boy with GPP and homozygous mutation at c.115+6T>C within the interleukin-36 receptor antagonist (IL36RN) gene, who was treated successfully with off-label use of secukinumab monotherapy after failure of treatment with acitretin and cyclosporine A.
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Phenomenal role of nitroglycerin in the treatment of neonatal digital gangrene p. 162
Rahul Choudhary, Susil Choudhary, Garima Sachdeva, Arti Maria
DOI:10.4103/ijpd.ijpd_163_20  
Placement of peripheral venous catheter is a common requirement for neonates requiring intensive care unit admissions. This simple procedure can sometimes lead to catastrophic complications such as peripheral tissue ischemia/gangrene. This is particularly common in preterm neonates. Effective treatment is very important to prevent long-term sequelae or need for amputation. Nitroglycerin (NTG) is a potent smooth muscle relaxant and improves vascular flow. Here, we report a case of neonatal digital gangrene which was successfully treated with topical NTG with no adverse outcome.
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A double whammy! Rare occurrence of psoriasis vulgaris in a case of X-linked recessive ichthyosis p. 165
Akshay Kumar Jain, Shivani Saini, Nidhi Agrawal, Shail Agarwal
DOI:10.4103/ijpd.ijpd_167_20  
Ichthyosis and psoriasis both are inherently considered as disorders of abnormal keratinization. X-linked recessive ichthyosis (XLRI) is a genetic disorder, while psoriasis is sporadic in most cases. The underlying pathophysiology for their occurrence in the same patient is not well understood but may be attributed to genetic modifications or epigenetics. We hereby report a case of a 14-year-old male child with psoriasis vulgaris in the setting of XLRI. To our best knowledge, it has not been reported in the English literature to date.
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LETTERS TO EDITOR Top

Dermoscopic perspective of pressure purpura: A report of three cases p. 168
Samipa Samir Mukherjee, Balachandra S Ankad
DOI:10.4103/ijpd.IJPD_76_20  
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Porokeratoma: An uncommon entity p. 170
Piyush Kumar, Hiral Shah, Anupam Das
DOI:10.4103/ijpd.IJPD_67_20  
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Periocular fixed drug eruption presenting as periorbital hypermelanosis: Clinical diagnosis aided by dermoscopy p. 172
Namrata Chhabra
DOI:10.4103/ijpd.IJPD_114_20  
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“Mite bite” sign: A novel atypical eschar of pediatric scrub typhus p. 174
Thirunavukkarasu Arun Babu, Dinesh Kumar Narayanasamy
DOI:10.4103/ijpd.IJPD_109_20  
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Cutaneous manifestations among COVID-19 patients p. 177
Mangla Sood, Seema Sharma
DOI:10.4103/ijpd.IJPD_116_20  
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The cyclosporine syringe: A pragmatic approach for accurate calibration of cyclosporine dosing in children p. 179
Sandipan Dhar, Shikhar Ganjoo
DOI:10.4103/ijpd.IJPD_95_20  
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Lymphadenitis and lichen scofulosorum following bacille calmette-guérin vaccination in an infant p. 181
Taru Garg, Suvarna Samudrala, Varinder Singh, Shilpi Agarwal
DOI:10.4103/ijpd.IJPD_27_20  
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PHACE syndrome: An uncommon case p. 183
Anupam Das, Abhishek De, Deepika Halder, Sudip Das
DOI:10.4103/ijpd.IJPD_60_20  
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A painful mass on the scalp: An uncommon tumor at an uncommon site p. 186
Smita S Ghate, Priyanka Arun Kowe, Sandip Agrawal, Rachita S Dhurat
DOI:10.4103/ijpd.IJPD_74_20  
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Nipple eczema-rare minor criterion for diagnosis of atopic dermatitis in infants p. 189
Shyam Bhanushankar Verma, Resham Vasani
DOI:10.4103/ijpd.ijpd_174_20  
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CONFERENCE REPORT Top

ISPD DIGICON 2020: Celebrating 25 glorious years' of brilliant service in the field of pediatric dermatology, leaving lasting footprints amid the COVID pandemic p. 191
Sanober B Daruwalla
DOI:10.4103/ijpd.ijpd_50_21  
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IJPD AWARD 2020 Top

Best Papers p. 194

DOI:10.4103/2319-7250.312838  
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