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   Table of Contents - Current issue
April-June 2019
Volume 20 | Issue 2
Page Nos. 93-190

Online since Friday, March 29, 2019

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Prevention of atopic dermatitis: Etiological considerations and identification of potential strategies Highly accessed article p. 93
John C Su, Adrian J Lowe
Research during the last decade has given many new insights into factors contributing to the development and evolution of atopic dermatitis (AD). Factors identified include skin barrier defects, proinflammatory predispositions, anomalies in the microbiome, and some environmental influences. There is now the possibility of personalizing therapy and potentially, of disease prevention. However, discerning the respective roles of early factors leading to AD development is complex. Stringent analysis for confounders must precede attribution of causality to any factor. This review examines current understandings of AD pathogenesis and related research approaches in AD primary prevention.
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Disorders of nail in infants and children Highly accessed article p. 101
Archana Singal, Kavita Bisherwal
Nail disorders in infants and children do not contribute to substantial pediatric consultations as they are relatively uncommon. Nail changes are often missed as specific nail examination is not routinely done in this age group. The presentation and management of nail disorders in children are different from adults; few being specific to the children. Physiological alterations are common in infants and pediatric age. These should be known to a clinician so as to differentiate from pathological conditions, to reassure parents, and to avoid unnecessary medical intervention. The congenital nail disorders can be a part of major hereditary syndromes requiring further evaluation. Several acquired causes may cause nail dystrophy. Some of them are self-limiting while others may require long-term management. Meticulous and careful nail examination is, therefore, important in neonates, infants and children for early diagnoses, management and to prevent complications. There are few epidemiological studies delineating nail changes in infants and children. In this script, we have comprehensively reviewed nail conditions seen in pediatric population through all stages, i.e., neonates, infants, and children.
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Childhood leprosy: A review Highly accessed article p. 112
Swetalina Pradhan, Bibhu Prasad Nayak, Gaurav Dash
Leprosy in children is of special importance as it is the indicator of transmission in community. It affects both the child and family members psychologically and functionally. In this review, we will discuss regarding epidemiology of childhood leprosy in detail, types of leprosy in children, diagnostic difficulties in children, prevention of disabilities in children, and effect of childhood leprosy on the community.
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Hot Topics in Paediatric Dermatology Highly accessed article p. 117
Vishal Thakur, Dipankar De
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A study assessing the knowledge, attitude, and practices of parents regarding childhood hypopigmented lesions p. 122
Akila Ravindra, Veranna Shastry, Chetana Prakash, Jaydev Betkerur
Background: Pigmentary disorders are believed to be the most common group of dermatoses in the pediatric age group. Loss of pigment can have a profound psychological impact on parents of the affected child. There are few studies available in India about the evaluation of hypopigmented lesions in the pediatric age group. Objectives: The objective of this study was conducted to assess the knowledge and attitude and various practices of parents toward hypopigmented disorders. Materials and Methods: A total of 130 pediatric patients were evaluated for hypopigmented lesions. Parent of each child was given a preformed questionnaire for the assessment of knowledge and attitude and various practices of their skin condition. Results: The frequency of hypopigmentary disorders among children was 3.28/1000. The mean age was 8.41 years. Nearly 9.33% of patients had onset at birth. In the study of 130 parents, 82 had low, 32 had moderate, and 14 had high knowledge levels, and 84 had unfavorable, and 46 had favorable attitudes. The parents, who had incorrect practices, were 53% and 35.67% had correct practices. Conclusion: The most common hypopigmentary conditions are benign and self-limiting, which requires proper counseling of the parents. A good knowledge and attitude will not only liberate them from traditional beliefs and home remedies that have been used in most of the Indian households but will also make them understand the magnitude of the problem their child could face if they do not seek proper advice from a doctor at the right time.
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Childhood vitiligo: A hospital-based study on 200 patients in Northeast India p. 128
NA Bishurul Hafi, Nandakishore Singh Thokchom, Shyamsunder Ch Singh, Romita Bachaspatimayum
Background: Vitiligo is a depigmenting disorder which can be psychologically devastating. Childhood-onset vitiligo has different epidemiological and clinical characteristics as compared to adults. Aims and Objectives: The aim was to study the clinico-epidemiological and hematologic investigation profiles of childhood vitiligo. Materials and Methods: First 200 pediatric patients younger than 18 years, with vitiligo who attended the dermatology outpatient department of a tertiary center in Northeast India, between September 2015 and August 2017 were included in the study. A detailed history and examination along with autoimmune diseases and laboratory parameters were recorded. Results: Among the 200 patients, 62% were girls. The mean age was 10.3 ± 4.9 years. The mean age at onset was 9.1 ± 4.9 years (ranging 2 months–17 years), with duration of disease varying from 1 month to 8 years with mean duration of 1.39 ± 1.63 years. Most common pattern of vitiligo was vulgaris (39.5%) followed by focal (25%), segmental (15.5%) genital (10%), acral and lateral lip (8%), and acrofacial (4%). Family history of vitiligo was seen in 12% of patients. In 96% patients, only <5% of body surface area was affected. Nearly 8.5% had Koebnerization while 9% showed leukotrichia. Thyroid-stimulating hormone and antithyroid peroxidase abnormalities were seen in 4.5% and 1% patients, respectively. Low Vitamin D level was seen in 21.5%. Conclusion: Any depigmented lesion in children should be evaluated and followed up properly to rule out vitiligo. Dermatologists and pediatricians should understand the characteristics of childhood vitiligo properly since it behave differently from adult-onset disease.
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Factitious disorders in children: A diagnostic labyrinth of cases p. 134
Chinmoy Raj, Maitreyee Panda, Debasmita Behera, Bikash Ranjan Kar
Factitious disorders are among the most underdiagnosed and less explored group of disorders in pediatric patients. Studies from different parts of the world have reported varying prevalence rates. In this article, we present a series of nine cases of factitious disorders that we encountered in the pediatric age group. Factitious disorder remains highly elusive in developing countries like India. It is highly necessary to remain vigilant to clinch an early diagnosis and manage appropriately.
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A rare early presentation of staphylococcal scalded skin syndrome in a Neonate p. 138
Srinivas Abhishek Gaddam, Srinidhi Thirunagari
Staphylococcal Scalded Skin Syndrome (SSSS) is caused by the exfoliative toxins (ETs) of Staphylococcus aureus. We present a case of a 3 days old infant with complaints of fever and diffuse erythematous exfoliation all over the body with bilateral conjunctivitis. The diagnosis of SSSS was reached based on clinical features and positive blood culture report. He responded well to the antibiotics paracetamol, and intravenous fluids for rehydration. He was discharged after 14 days, with complete resolution of symptoms. Having a high clinical suspicion for SSSS, early diagnosis/treatment, and following strict aseptic measures in neonatal intensive care unit are important.
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Congenital erythropoietic porphyria in an Indian Child p. 141
Archana Singal, MN Kayarkatte, Deepika Pandhi
Congenital Erythropoetic Porphyria (CEP) also called the “Günther disease”, is a rare variant of porphyria. It is caused by the deficiency of uroporphyrinogen III synthase (URO-III-synthase), an enzyme in the heme biosynthetic pathway. Clinically, CEP presents with blistering over face and extremities, scarring, hypertrichosis and dyspigmentation. Acral blistering leads to mutilation of the fingers with acro-osteolysis of distal phalanx We, hereby, report an 8-years-old boy with classical clinical features and porphyrin assays.
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Vincristine, aspirin, and prednisolone therapy in Kasabach–Merritt phenomenon: Response in 2 cases p. 145
Shikha Gupta, Subhash Bharti, Niyaz Ahmed Khan, Lavleen Singh
Kasabach–Merritt phenomenon (KMP) is a severe thrombocytopenic coagulopathy which usually occurs in the presence of enlarging vascular tumors such as kaposiform hemangioendothelioma (KHE) and tufted angioma. The treatment for this potentially fatal condition is challenging without a consensus on appropriate management. The authors report two cases of KHE with KMP, wherein improvement in size of tumor and coagulopathy occurred after treatment with prednisolone, vincristine, and aspirin.
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Spitzoid melanoma in a child with xeroderma pigmentosum p. 148
Bijayanti Devi, Anup Goswami, Y Imlongchaba, Angila Dorjee
Xeroderma pigmentosum (XP) is an autosomal recessive condition associated with a marked increase in the development of malignancies. We report a 10 year girl with freckles, lentigens and keratosis on the face and neck which had gradually developed from the age of two. History of marked photophobia was present. Few months prior she developed single firm, pigmented, non-tender, freely- mobile, sessile nodule over left ala of nose. Multiple frecklings with atrophic hypopigmented patches were distributed all over face and sparsely on neck. Histopathology examination (HPE) from growth over nasal ala revealed malignant tumour composed of spindloid to epithelioid cells arranged in sheets and nests in dermis suggestive of spitzoid melanoma. Fine needle aspiration cytology (FNAC) from left post-auricular lymph nodes revealed high cellularity comprising of many pleomorphic cells in clusters having enlarged irregular hyperchromatic nuclei. Wide local excision was done with nasolabial transposition of flap cover.
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Rubinstein–Taybi Syndrome in a Male Child: Unusual Manifestations of a Rare Disease p. 151
Deepak Balikai, Varsha M Shetty, Raghavendra Rao, B Sathish Pai
Rubinstein–Taybi syndrome (RSTS) is a rare genetic disorder, characterized by intellectual disability, growth retardation, characteristic facial features, skeletal deformities, and other systemic involvement. Cutaneous involvement includes hirsutism, capillary hemangiomas, pilomatricomas, keloids, and hair abnormalities. Diagnosis of this condition is at times difficult due to overlapping clinical features and requirement of genetic mutation analysis which is not feasible in majority of the cases. Hence, it is imperative to know the typical dermatological features of RSTS which will help in clinical diagnosis in most of the cases.
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Early congenital syphilis: Resurgence of an entity nearing elimination p. 154
Khushali H Shah, Ashish G Jagati, Santoshdev P Rathod, Raju G Chaudhary
A 45-day-old female infant was brought to a tertiary care center with the chief complaints of skin lesions over both hands and feet and perianal area since birth. Associated systemic complaints included rhinitis and diarrhea. Clinical examination revealed erosions with moist, erythematous base in an acral distribution, cheilitis, and perianal moist erythematous lesion. Based on the history and clinical examination findings of a congenital bullous rash, acrodermatitis, and periorificial moist lesion, rapid plasma reagin test was advised which came 1:512, thus confirming the diagnosis of congenital syphilis.
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Pyoderma gangrenosum in childhood acute lymphoblastic leukemia p. 157
Ajeitha Loganathan, Arathi Srinivasan, Julius Xavier Scott, Ramkumar Ramamoorthy
Pyoderma gangrenosum (PG) is a sterile neutrophilic dermatosis which occurs either idiopathically or associated with various systemic diseases and malignancies. Although the association of PG with myeloid malignancies is well known, its association with lymphoid malignancy especially in children is extremely rare. We describe PG in association with acute lymphoblastic leukemia in a 4-year-old child, an occurrence which has been reported just once previously to the best of our knowledge.
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A rare case of childhood mucous membrane pemphigoid involving both skin and mucosa p. 160
Monali Pattnaik, Maitreyee Panda, Mrutunjay Dash, Chinmoy Raj
Mucous membrane pemphigoid has been documented previously in only 19 patients under the age of 20 years. We report a 14-year-old female who had erosions of the oral, ocular, anal and genital mucosa, congestion, hoarseness of voice, dysphagia, and recurrent cough. Symblepharon formation in both eyes necessitated surgical correction. Biopsy of tissue from a solitary cutaneous lesion demonstrated a subepidermal bulla. Direct immunofluorescence showed the deposition of IgG in linear pattern along the dermoepidermal junction. Therapy was begun, consisting of prednisolone at 1 mg/kg/day, in combination with dapsone at 2 mg/kg/day. Azathioprine was added later for control of the disease. The patient showed good clinical response.
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Dorfman-Chanarin syndrome: An unusual presentation p. 163
Anupa Mary Job, Vijay Aithal, Carol Lobo, AM Shanthala Devi
Dorfman-Chanarin syndrome is a rare autosomal recessive neutral lipid storage disorder with congenital ichthyosis characterized by triglyceride deposition in multiple organs. The usual clinical presentation has been reported to correspond to that of nonbullous congenital ichthyosiform erythroderma. We report a case of a 1-year-old boy who presented with minimally scaly figurate plaques with double-edged scaling in the periphery and clinically mimicking Netherton's Syndrome. The diagnosis was confirmed by the presence of characteristic lipid vacuoles in the leukocytes (Jordan's anomaly).
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Aplasia cutis congenita of the scalp: The success of conservative approach in treatment of a large defect p. 166
Zoran Barcot, Marko Baskovic, Andrija Car, Marina Juric
Aplasia cutis congenita is a rare malformation that usually involves the scalp. It may occur as an isolated defect or be combined with congenital malformations. We present a case of a female infant at the age of 2 days with aplasia cutis congenita of the scalp. Although there is no consensus about the treatment, we have decided for a conservative approach because sagittal sinus, large veins, or brain were not exposed. Ten months since the onset of conservative treatment, the aplastic area was almost completely cured.
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Clavicular skin-colored nodule p. 169
Amairani Manriquez-Robles, Alexandra Maza-De Franco, Anahi Castañeda-Zarraga, Ana Florencia Lopez-Ornelas, Sonia Toussaint-Caire, Maria Elisa Vega-Memije
An accessory tragus (AT) is a congenital flesh-colored nodule occasionally covered with vellus hair. It can arise anywhere along the line from the preauricular area to the corner of the mouth. It can also be found on the glabella or suprasternal region. The tragus is derived from the first branchial arch, which also develops into the mandible and mouth. The rest of the external ear develops from the second branchial arch. AT may occur as an isolated defect or in chromosomal first branchial arch syndromes with ear anomalies, cleft lip and palate, and mandibular hypoplasia.
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Sporadic Case of dyschromatosis universalis hereditaria showing moderate response to narrow-band ultraviolet-B p. 172
Sarita Sasidharanpillai, Amal Shyam, Shiny P Manakkad, Ettapurath N Abdul Latheef, Saleem Rahima, Nina Paul
Dyschromatosis universalis hereditaria (DUH) is a rare genodermatosis where the affected manifests mottled pigmentation with intermingled hyper- and hypo-pigmented macules. On most occasions, the lesions begin on limbs and then extend to trunk. Most of the cases reported in literature are from Japan. It is rarer in Indians. In this report, we describe a 12-year-old male child with DUH, who showed response to narrow-band ultraviolet-B therapy.
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Congenital acral verrucous hemangioma: A rare entity p. 174
Kamal Nain Rattan, Rajnish Kalra, Renuka Verma, Sunita Singh, Monika Gupta, Megha Ralli
Verrucous hemangioma is an uncommon capillary or cavernous hemangioma with reactive epidermal changes of hyperkeratosis, acanthosis, and papillomatosis. Lesions are mostly seen at birth or appear during childhood. These growths are usually located on the lower extremities, and involvement is generally unilateral. Diagnosis of this unusual presentation can only be confirmed following histopathology. Surgical excision resulted in a satisfactory functional and cosmetic outcome.
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Cutaneous horn, juvenile xanthogranuloma in the first decade of life: A clinico-dermatoscopic correlations p. 177
Ebtisam Elghblawi
Juvenile xanthogranuloma (JXG) is a rare disorder that is present at birth, in infancy, or in adults. It can be either solitary or multiple nodules of various sizes. It can be associated with internal organ involvements, especially eyes, lung, and liver. The diagnosis can be made with confidence by clinical examination alone and confirmed by histology in doubtful cases. Hereby, I report the useful benefit of dermoscopy in evaluating and diagnosing a skin lesion that is suggestive of JXG. A 1-year-old skin type I girl presented with gradual onset of a nodular lesion on her forearm that showed a surface projecting horn. A biopsy was planned and scheduled. Clinical features were in keeping with JXG. JXG is on the spectrum of histiocyte disarrays which should be examined thoroughly in children to exclude underlying malignancy. The purpose of this article is to describe the clinical dermatoscopic correlated features of JXG. This is an interesting case of JXG presenting in a young White Swedish female child.
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Disseminated congenital cutaneous candidiasis caused by Candida ciferri: A rare case report p. 180
B Puneetha, Sahana M Srinivas, S Mahantesh, Naveen Benakappa
Congenital cutaneous candidiasis (CCC) is an uncommon disease affecting newborns which manifests within first 6 days of life following an ascending infection from birth canal or as transplacental infection. Diagnosis is challenging due to its varied clinical manifestation ranging from mild asymptomatic illness to fatal disseminated disease. We describe a full-term newborn child with CCC with dissemination born to mother with vaginal candidiasis, positive for Candida ciferri by fungal culture. This highlights the importance of maternal history of vaginal discharge during pregnancy to diagnose and prevent neonatal disseminated infection which further aids for adequate early treatment to prevent subsequent mortality.
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Relevance of circular hair by trichoscopy in pediatric age group p. 183
Tarang Goyal, Sakshi Kohli
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Eruptive vellus hair cysts in a child p. 187
Vikrant A Saoji, Kinjal Deepak Rambhia, Mayank M Goyal
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Linezolid-induced black pigmentation of tongue and perioral region p. 189
Angoori Gnaneshwar Rao, Karnam Aparna, Vangali Srikanth Reddy, Syeda Saba Farheen, Ruhi Hakkani, Divya Parimala, M Tejal, Sneha Gupta
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