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   Table of Contents - Current issue
October-December 2021
Volume 22 | Issue 4
Page Nos. 293-386

Online since Friday, October 1, 2021

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Prenatal diagnosis in dermatology Highly accessed article p. 293
Manjyot Gautam, Faaria Ali
Prenatal diagnosis (PND) of inherited skin disorders comprises all the diagnostic modalities carried out in utero for couples who are at high risk of producing a child with congenital abnormalities. With the constant research being carried out in the field of molecular diagnosis and genetics, it is of utmost importance for dermatologists to be abreast about all the available means of genetic testing in utero. This will enable us to provide the best of options to the couples who are at risk of having children with inheritable skin disorders so that they can get the tests done at the appropriate time during gestation and the pregnancy can be continued safely till term. This article talks about all the invasive and noninvasive methods of PND practised at present and the ones that may gain popularity in the near future.
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Revisiting pediatric alopecia areata: Newer insights Highly accessed article p. 301
Yashpal Manchanda, Ramkumar Ramamoorthy
Alopecia areata (AA) is an autoimmune disorder that results from the targeted attack of hair follicles by multiple T-cell subsets leading to hair loss. Childhood-onset AA is characterized by a severe clinical course with a poorer treatment outcome. However, it does carry a significant detrimental impact on the quality of life and the self-confidence of the child, besides impairing the emotional health of parents in severely affected cases. The data on pediatric AA is limited. Despite numerous advances made in recent years for the management of AA in children, Food and Drug Administration approved systemic treatment required for severe AA cases is still elusive. In this review, we review the recent literature on clinical manifestations, comorbidities, investigations, and the treatment modalities of AA in children.
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Childhood lichen planus p. 306
Arun Joshi, Sanjay Kumar Rathi, Yashpal Manchanda
Lichen planus (LP) is a relatively uncommon chronic skin condition with varied clinical presentations seen mostly in adults. It is rarely encountered in children, although increasingly more pediatric cases are being recognized and reported. LP can affect skin, mucosae, hair and nails, alone or in various combinations. All forms of LP seen in adults also occur in children. Certain types (eruptive, generalized) are observed more commonly in children than adults whereas appendageal (follicular and nail), mucosal (oral, genital, esophageal), hypertrophic and bullous variants have also rarely been reported in pediatric population. The exact etiopathogenesis of LP is not known. Immune dysregulation, infections, environmental, and genetic factors have been studied extensively and speculated to play some roles. A wide variety of treatment modalities for LP have been used in adults, and the same are employed in children. There is dearth of randomized controlled trials of evidence based treatments in LP, especially in children probably because of the rarity of the condition, heterogeneity of presentations, and gaps in understanding the exact etiology. This review attempts to present the up to date current information on the epidemiology, etiopathogenesis, clinical features and therapeutic options for lichen planus in children.
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Current perspectives in the treatment of childhood lichen planus p. 316
Sharad D Mutalik, Vasudha A Belgaumkar, Yashashree D Rasal
Childhood lichen planus (CLP) is relatively common in India. With a mean age of onset at 7–8 years, CLP poses a therapeutic challenge as most guidelines for treatment cater to adult lichen planus (LP) with no defined evidence-based recommendations for CLP. Each case of CLP needs customized therapy with a step-wise approach. Topical corticosteroids are ably supported by topical calcineurin inhibitors, while oral retinoids, immunosuppressants like cyclosporine, and immunomodulators such as dapsone and narrowband ultraviolet B phototherapy are taking center stage in place of oral corticosteroids as systemic treatment modalities. However, the management of mucosal and appendageal LP requires systemic steroids more often than not. For the present review, we extracted the available data published in the English literature (Google Scholar databases, PubMed, and Medline) using search terms “lichen planus,” “treatment,” “children,” “pediatric,” and synthesized documented evidence regarding the risk–benefit profile of each therapeutic modality used in CLP. Undoubtedly, reporting of anecdotal successes, case studies, and conducting randomized controlled trials will help to structure consensus guidelines for the therapeutic conundrum of CLP.
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Measurement of epidermal hydration and skin ph in infants with atopic dermatitis before and after a bath p. 326
Pirabakaran Sivanesan, Thulasi Weerasinghe, Kavindra Nanayakkara, Praneeth Ratnayake, Sriyani Samaraweera, Jayamini Seneviratne
Introduction: The skin barrier function resides primarily in the stratum corneum (SC). Defect in SC hydration and skin surface pH leads to barrier impairment causing atopic dermatitis (AD). We have noticed a clinical improvement in infants with AD after a bath. We aimed to assess barrier functions in infants with AD before and after a single bath. There are no large controlled studies that clarify the optimal parameters of bathing. However, there are few studies recommending bath in AD. We proposed a “twenty minutes immersion bath.” Materials and Methods: We conducted an interventional analytic study without comparison group. The main instruments used were the corneometer and skin pH meter. Results: The measured values of SC hydration were significantly higher on the lesional skin immediately after a bath (P < 0.001). The mean SC hydration dropped dramatically within one hour and gradually reduced further over the next three hours. However, healthy looking skin had short climb and slow drop of SC hydration. The measured values of skin pH were lower on the lesional skin after bathing (P = 0.026). Discussion: An immersion bath improves SC hydration and helps to maintain skin pH and warrants further evaluation in the context of atopic dermatitis.
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Muscle weakness in a 4-year-old girl: A rare presentation of deep morphea p. 333
Mary Augustine, Sumedha Ballal
Introduction: Morphea is a rare fibrosing disorder of the skin and underlying tissues. Deep morphea involves the deep dermis, subcutis, fascia, muscle, and bone. The above structures may be involved independently or in combination. Case report: We describe a case of deep morphea presenting as muscle weakness, independent of skin lesions, in a child with generalized morphea. Discussion: Muscle weakness, even in the absence of overlying or progressive skin sclerosis, can be deep morphea, especially when segmental and associated with atrophy.
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Orofacial-digital syndrome type 1 with patchy scalp alopecia in an Indian child p. 336
Sameeksha Chand
Introduction: Oral-facial-digital syndrome (OFDS) is a rare type of ectodermal dysplasias characterized by malformations of the face, oral cavity, and digits. The OFDS type 1 is most common and has an X-linked dominant inheritance. Case report: We report a child with a single patch of scalp alopecia who was found to have typical oral malformations such as high arched palate, accessory frenulae, tongue hamartoma, and dentition abnormalities. . She had associated central nervous system malformations and was diagnosed with OFDS 1. Discussion: The case highlights importance of recognizing this syndrome to detect systemic abnormalities on time.
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Atypical manifestations of cutaneous staphylococcal infection in infants – Generalized bullous impetigo and asymmetrical peripheral gangrene p. 339
Anuradha Bishnoi, Dinesh Raj, Rahul Mahajan
Introduction: Impetigo is the bacterial infection of the skin most commonly seen in infants and children. Bullous and nonbullous are the two forms of impetigo. It usually presents with vesicles, bullae, crusted erosions, or ulcers. Case report: We report two cases in which the first case showed crusted erosions all over the body and the second one had bullous lesions over the extremities, which eventually lead to gangrene of fingers and toes. These were due to the bacterial infections of the skin. Discussion: Superficial bacterial infection should be one of the differential diagnoses, whenever there is diffuse exfoliation of the skin or peripheral gangrene.
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A rare association of incontinentia pigmenti with congenital heart disease in a newborn p. 342
Asha Gowrappala Shanmukhappa, Mounica Chimbili, Leelavathy Budamakuntla, Shilpa Kanathur
Introduction: Incontinentia pigmenti (IP) is a rare genodermatosis. It classically has manifestations of linear vesicular lesions, evolving into verrucous lesions within few weeks, followed by a peculiar swirled pigmentation lasting for many years. In addition, IP can affect other ectodermal tissues such as the teeth, eyes, bones, and the central nervous system. Case report: We report a case of IP in a newborn female baby who presented to us with verrucous lesions at birth and on examination was found to have congenital heart disease (atrial septal defect, with pulmonary hypertension, tricuspid regurgitation, and predominant right to left shunt). Discussion: We wish to draw attention to the association of IP with congenital heart disease.
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Lipoatrophic linear morphea in a 5-year-old girl: A novel variant? p. 346
Vibhu Mendiratta, Mansi Bansal, Shivangi Rana, Kiran Agarwal
Introduction: Morphea profunda or subcutaneous morphea is a variant of morphea in which inflammation and sclerosis found in deep dermis, subcutaneous tissue, fascia or muscle. Case report: We are report an atypical case of subcutaneous morphea in five year-old girl, presenting with atrophy of subcutaneous fat over the left lower limb in a liner pattern, closely mimicking linear lipoatrophy. Discussion: Early intervention in such cases can halt progression of the disease.
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Case of mandibuloacral dysplasia with type B lipodystrophy p. 349
Sanober Burzin Daruwalla, Rachita S Dhurat, Smita Ghate, Rutuja Arali
Introduction: Mandibuloacral dysplasia with type B lipodystrophy (MADB) caused by compound heterozygous mutation in the ZMPSTE24 gene is characterized by generalized lipodystrophy and short stature. Cutaneous manifestations of these disorders vary broadly. Case report: We present a genetically confirmed case of MAD Type B in a two year-old boy born out of second degree consanguinity. Discussion: The report aims to increase awareness of the rare condition and emphasize the utility of genetic analysis in differentiating it from other differentials with a phenotypic overlap.
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Conundrum of recurrent erythema nodosum leprosum and dapsone hypersensitivity syndrome complicating pediatric leprosy p. 352
Anwita Sinha, Vidhu Dangwal, Achinta K R. Mallick, Anchit Raj Singh, Asmita Sinha
Introduction: Leprosy, a chronic infectious disease affecting the skin and peripheral nerves, has been considered a disease of adults due to its long incubation period, but in endemic regions like the Indian subcontinent, pediatric leprosy cases continue to rise reflecting active disease transmission in the community. Case report: We describe a case of bordeline lepromatous leprosy in a child complicated by severe type 2 lepra reaction and dapsone hypersensitivity syndrome. Conclusion: Pediatric leprosy cases though commonly paucibacillary, can also present with a high bacillary burden. Complicated leprosy cases need nuanced care and highlight the importance of adequate clinical exposure to leprosy cases, so that such cases can be confidently managed.
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Atypical cases of cydnidae pigmentation: Digging deeper through dermoscope p. 356
Vineet Relhan, Pallavi Hegde
Arthropods can cause variety of cutaneous manifestation including pigmentation. Recently, burrowing bug pigmentation is described in the literature which though is a very common occurrence and goes underreported. Hereby, we report 2 cases of this entity presenting with atypical features to expand the clinical spectrum and to create awareness about this self-limiting condition. We have also added dermoscopic features of this entity to provide new dimension to the diagnosis.
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Chikungunya rickettsial coinfection with fatal outcome: A diagnostic dilemma p. 360
Sahana M Srinivas, Shantini Vijayasuriar, Priyajeevamani Chandrasekaran, Keshavmurthy M Lakshmikantha
Acute febrile illness caused by multiple infectious etiologies is not uncommon, especially in endemic countries like India. Recently, there has been increased mortality with chikungunya infection in children. There are several studies of coinfection of chikungunya with dengue, but coinfection with rickettsial infection is rare. We describe a 14-year-old boy presenting with chikungunya with probable rickettsial coinfection with a fatal outcome.
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Chronic foot ulcers – Rule out cutaneous tuberculosis! p. 363
Sunita Arora, Tejinder Kaur, Guneet Gandhi
Cutaneous tuberculosis is a form of extrapulmonary tuberculosis and is seen in a small but significant subset of patients seeking dermatology outpatient services. Children constitute a significant proportion of overall cases of cutaneous tuberculosis. We report the case of an 8-year-old boy who presented with multiple discharging ulcers over bilateral feet. High suspicion of cutaneous tuberculosis led to early diagnosis and initiation of therapy. Cutaneous tuberculosis can have a varied clinical presentation. We wish to draw attention to mycetoma-like presentation of cutaneous tuberculosis characterized by chronic discharging foot ulcers.
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Congenital syphilis – Re-emergence of a much forgotten entity? p. 367
Rutva Amlani, Savita Agrawal, Ram Gulati, Kishor Singh, Sonal Jain
Congenital syphilis is a severe, disabling infection that occurs due to transmission of Treponema pallidum across the placenta. An increase in the incidence of congenital syphilis has been observed in developing countries. Here, we are reporting a case of early congenital syphilis who presented with desquamating papulosquamous lesions over multiple body parts along with moist erythematous and erosive lesions in the perianal and perioral regions. Reactive venereal disease research laboratory and T. pallidum hemagglutination assay test in child and parents confirmed the diagnosis of congenital syphilis.
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Kin with no skin: Johanson–Blizzard syndrome in siblings: A rare association of aplasia cutis congenita p. 370
GS Asha, K Kavya Shree, TN Revathi, Kanathur Shilpa
Johanson–Blizzard syndrome (JBS) is a rare autosomal recessive disease which was first described in 1971 by Johanson and Blizzard in three unrelated girls. Less than 100 cases have been reported to date. It is characterized by exocrine pancreatic insufficiency, hypoplastic nasal alae, scalp cutis aplasia, and other features including developmental delay, failure to thrive, hearing loss, mental retardation, hypothyroidism, dental anomalies, and anomalies of the genitourinary system. The condition is caused by mutations in the UBR1 gene, which encodes the E3 ubiquitin ligase protein responsible for regulating the degradation of proteins. Aplasia cutis congenita is a rare entity characterized by localized or widespread absence of skin from birth. Scalp is the most common site to be involved. Here, we are reporting two interesting cases of JBS in siblings who had aplasia cutis as their initial cutaneous manifestation.
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Response of papillon–Lefevre syndrome to acitretin p. 374
Priyanka Arun Kowe, P Lavanya, Vaishali H Wankhade, Rajesh P Singh
Papillon–Lefevre (PLS) is a rare autosomal recessive disorder of keratinization characterized by symmetric, trans-gradient type palmoplantar keratoderma (PPK), rapidly progressive periodontopathy, and precocious loss of dentition. The management of this condition is difficult and needs a combined approach of both dermatologist and periodontologist. Different treatment options tried previously include keratolytic agents such as salicylic acid, urea, topical steroids in combination with keratolytic, propylene glycol, and systemic retinoids such as etretinate, isotretinoin, and acitretin. Both skin and dental manifestations show good therapeutic responses to systemic retinoids. Thereby, we report a case of PLS in a young female child who showed an excellent response to oral acitretin therapy.
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Diet in pediatric dermatology p. 378
Sanober Burzin Daruwalla
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Halo nevus and halo phenomenon in dermatology p. 381
Manjyot Gautam, Reeya Patel
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Eruptive vellus hair cyst: Treatment with topical tretinoin 0.025% cream p. 385
Rashmi Agarwal, S Suganya, BS Chandrashekar
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