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   Table of Contents - Current issue
Coverpage
October-December 2017
Volume 18 | Issue 4
Page Nos. 261-353

Online since Friday, September 29, 2017

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CME ARTICLE  

Alopecia areata: Update on management p. 261
Julie S Kranseler, Robert Sidbury
DOI:10.4103/ijpd.IJPD_80_17  
Alopecia areata (AA) is a common autoimmune nonscarring alopecia. AA presents heterogeneously and is influenced by both environmental and genetic factors. Diagnosis is clinical after ruling out other local or systemic causes of alopecia. Standard first-line therapy is typically topical steroids, but the response can be frustrating. Novel treatment options have shown great promise in the management of the refractory disease. We review initial data on topical and systemic Janus kinase inhibitors (tofacitinib, ruxolitinib, and baricitinib), topical bimatoprost, simvastatin/ezetimibe, and excimer laser therapy among others within the context of a general approach to AA management.
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REVIEW ARTICLE Top

Inherited epidermolysis bullosa: A multisystem disease of skin and mucosae fragility p. 267
Guadalupe Maldonado-Colin, Carolina Hernández-Zepeda, Carola Durán-McKinster, María Teresa García-Romero
DOI:10.4103/ijpd.IJPD_16_17  
Epidermolysis bullosa (EB) is a blistering disorder that can be autosomic or dominantly inherited and has a wide spectrum of clinical presentations. The most recent classification divides EB into four basic subtypes: (1) EB simplex, (2) junctional EB, (3) dystrophic EB and (4) Kindler syndrome; depending on the level of the epidermal-dermal junction where the formation of blisters is present. With the use of immunohistochemistry and molecular biology, new mutation genes and proteins have been identified and more than thirty subtypes of EB have been described according to text phenotype, each affecting different key proteins for the structural integrity of the skin. The phenotype can be a mild one where blisters occur after minor trauma, but quality of life is preserved or a severe one with multisystemic manifestations affecting skin, mucous membranes, bones, joints, nutritional status, and cancer development leading to early death. We describe the clinical manifestations, diagnostic approach, and multidisciplinary management of EB subtypes.
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IJPD SYMPOSIUM Top

Treatment guidelines for atopic dermatitis by Indian Society for Pediatric Dermatology task force 2016 - Part-2: Topical therapies in atopic dermatitis p. 274
Deepak Parikh, Sandipan Dhar, Sahana Srinivas, Ramkumar Rammoorthy, Rashmi Sarkar, Arun Inamadar, Manish Shah, Raghubir Banerjee, Amrinder Jit Kanwar, Vibhu Mendiratta, Renu George, Ram Gulati
DOI:10.4103/ijpd.IJPD_99_17  
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ORIGINAL ARTICLES Top

The clinico-epidemiological profile of atopic dermatitis in residential schoolchildren: A study from South Chhattisgarh, India p. 281
Yasha Upendra, Naveen Keswani, Sampreeti Sendur, Abhishek Pallava
DOI:10.4103/ijpd.IJPD_52_17  
Introduction: Atopic dermatitis is a chronic, inflammatory, relapsing skin disorder with early age of onset in infancy and early childhood. Objective: The present study was conducted to study the clinico-epidemiological profile of atopic dermatitis among children of residential schools of naxal-infested South Chhattisgarh. Materials and Methods: Ninety children (4.6%) of atopic dermatitis were pooled out from a previous study by the same authors on 1943 children of eight residential schools. The various clinico-epidemiological factors were studied and analyzed using relevant statistical tools. Results: Out of ninety children having atopic dermatitis, boys:girls ratio was 1:1.73. The mean age of onset was 2.14 ± 0.52 years, and the mean duration of illness was 1.71 ± 0.38 years. The personal history of atopy was present in 34 (37.8%) children whereas a family history of atopy was seen in 47 (52.2%) children. Among clinical presentation, chronic type (n = 56, 62.2%) was most common, followed by subacute (n = 24, 26.2%) and acute (n = 10. 11.1%). Flexor (n = 42, 46.7%) was the most common site affected followed by extensor and face (n = 24, 26.7%) each. Mild severity was seen in 76 (84.4%) children followed by moderate severity (n = 12, 13.4%) and severe (n = 2, 2.2%) children. Sweating (n = 53, 58.9%) was the most common aggravating factor followed by seasonal (n = 51, 56.7%), wool (n = 24, 26.7%), stress (n = 15, 16.7%), dust (n = 10, 11.1%), physical exercise (n = 8, 8.9%), and food (n = 2, 2.2%). Conclusions: Atopic dermatitis of chronic type with mild in severity is more common in community.
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Pattern of pediatric dermatoses in Northeast India p. 286
Karthick Nagarajan, Nandakishore Singh Thokchom, Khaidem Ibochouba, Kapila Verma, NA Bishurul Hafi
DOI:10.4103/ijpd.IJPD_66_17  
Background: The prevalence of pediatric skin diseases varies worldwide. Pediatric dermatoses require a separate view from adult dermatoses as there are important differences in clinical presentation, treatment, and prognosis. Aims and Objectives: To study the clinical and etiological presentation of dermatoses in the pediatric population in Northeast India. Materials and Methods: The study was carried out at a tertiary care center in Northeast region for 18 months (from December 2012 to May 2014). Cases under the age of 12 years were selected based on purposive sampling method. Results: Infections (36.25%) were the most common cause of skin diseases in the study group comprising 400 patients. Eczematous diseases (14.5%), pigmentary disorders (14.25%), and infestations (13.25%) were the other common causes. Varicella (6%), impetigo contagiosum (5%), and candidiasis (4.5%) were the most common viral, bacterial, and fungal diseases, respectively. Scabies (12.25%) was the most common individual disease in the present study. The prevalence of hand, foot, and mouth disease was high constituting 4% of the study group. Dyshidrotic eczema (6.5%), pityriasis alba (7.5%), and vitiligo (3.5%) were also common. Miliarial dermatitis and polymorphic light eruption also contributed significantly (5.25%) reflecting the typical pattern of the tropical skin diseases. Atopic dermatitis, genetic disorders, and nutritional deficiency disorders had a very low prevalence. Conclusion: The study finding that the majority of the diseases could be grouped into fewer than 8 disease categories may help in more accurate diagnosis. Significant proportions of the dermatoses are infections followed by eczematous and pigmentary diseases. Age, sex, locality, the income of the parents, family history, and season of presentation have an influential role in the pattern of distribution of the pediatric skin diseases.
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A study of clinico-epidemiological and dermoscopic patterns of vitiligo in pediatric age group p. 292
Sneha Gandhi, Murugesh Shamanur, AR Shashikiran, Mamatha Kusagur, Sugareddy , Vijay Bhaskar
DOI:10.4103/ijpd.IJPD_107_16  
Context: Childhood vitiligo although similar to adult vitiligo has several distinct epidemiological, clinical, therapeutic and prognostic profile when compared with adult onset vitiligo. Aims: This study was conducted in an attempt to ascertain the clinico-epidemiological profile of patients in pediatric vitiligo patients (<18 years) and to conduct a dermoscopic analysis of the vitiligo patches. Subjects and Methods: This study was designed to study the clinic-epidemiological characteristics of childhood vitiligo between July 2015 and December 2015. A total of eighty childhood vitiligo patients were examined. In addition, 160 patches were studied for dermoscopic patterns to correlate with clinical stability and pattern of pigmentation. Results: Female to male ratio was 2.2:1. Average duration of the disease was 2.2 years. The mean age of onset was 7.6 years. A positive family history was seen in 18 patients. The pattern of viltigo in descending order of frequency was vitiligo vulgaris, segmental distribution, focal vitiligo, and acral vitiligo. The most common site was trunk. Dermatological associations were, history of atopy in 32 patients, alopecia aearata (1 patient) halo nevi (1 patient), and lichen nitidus (1 patient). The dermoscopic features of disease activity in order of frequency in our study were as follows: Trichrome pattern, nebulous pattern, star burst pattern, comet tailing of the lesion, and amoeboid pattern. 69 patients (43.12%) showed leukotrichia on dermoscopy, 74 (46.25%) showed perifollicular pigmentation, 32 patients (20%) showed marginal pigmentation, 26 (16.25%) showed both patterns whereas the rest did not show the signs of repigmentation. Under ultraviolet light examination, a diffuse white glow was seen in 147 (91.87%) of the patients. Conclusions: Vitiligo prevalence among children is on the rise and clinicoepidemiological data on this disease in between far and few. We found that dermoscopy was able to pick up disease activity earlier than the clinical onset of disease instability. This is the first study analyzing the dermoscopic pattern in pediatric vitiligo to the best of our knowledge.
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CASE REPORTS Top

Servelle-martorell syndrome with severe orthostatic hypotension in a pediatric patient p. 299
Yuri Igor Lopez-Carrera, Max Bernal-Moreno, Oscar Colin, María Teresa García-Romero, Carola Durán-McKinster
DOI:10.4103/ijpd.IJPD_18_17  
Servelle-Martorell syndrome (SMS) is an extremely uncommon vascular malformation characterized by venous malformations (VMs), limb overgrowth, and bone hypotrophy. The most common complications are venous thrombosis and pathological fractures. Ectasia and aneurysmal dilatations of the superficial veins may result in overgrowth of soft tissues but shortening of the affected limb. We report an 11-year-old Hispanic boy with an enlarged lower limb and VM. The patient suffered a fall at 9 years of age resulting in ankylosis of the knee without the possibility of limb extension or walking. When he tried to stand up, a severe orthostatic hypotension and almost loss of consciousness were present. An angio computed tomography and an arteriography demonstrated a complete absence of the deep venous system and bony hypotrophy compatible with SMS.
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An unusual case of familial trichomegaly p. 303
Joydeep Das, Debalina Sarker, Debes Pal, Sanjib Mondal, Balram Gupta
DOI:10.4103/ijpd.IJPD_50_17  
Trichomegaly is an unusual disorder of abnormally long eyelashes. Although the abnormality in itself does not cause any problems, except the cosmetic issues, however, it may be associated with syndromes, in which case it requires detailed investigations. Here, we describe a case of familial trichomegaly, involving mother and two siblings, which is an unusual entity.
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Erythroderma: An association or a manifestation extraordinary of hyper-immunoglobulin E p. 306
Virendra N Sehgal, Deepa Sehgal, Nazim Hussain Syed, Sonal Sharma
DOI:10.4103/ijpd.IJPD_51_17  
Erythroderma/generalized exfoliative dermatitis, presenting with intensely itchy, dry, scaly, widespread erythema covering whole of the skin surface is reported, the diagnosis of which was primarily clinical but was supplemented by histopathology. Other investigations such as hemogram, immunoglobulin E (IgE), and serum fluorescent enzyme immunoassay (FEIA) were supportive; while IgE was high, serum FEIA was extraordinary high. Identifying it as a clinical pearl of hyper-IgE syndrome, recovery of heavy coloniz ation of Klebsiella pneumoniae, anaerobic Gram-negative bacteria, was contrastingly unique responding affectively to metronidazole, a drug specific for the purpose.
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Giant nevus unius lateralis: Two cases p. 310
Ajay Govindrao Ovhal, Shruthi Vasanth Kumar Deshkulakarani, Viraj Jitendra Sadrani
DOI:10.4103/ijpd.IJPD_64_17  
Verrucous epidermal nevus is a common type of keratinocyte hamartoma present at birth or occurring later in life. Widespread multiple epidermal nevi (EN) may reflect genetic or chromosomal mosaicism. Blaschko has documented characteristic linear, zosteriform, unilateral or systematized patterns with streaks and swirls. They are resistant to treatment with the risk of recurrence. Giant congenital nevus unius lateralis (NUL) is known for its benign course with good supportive treatment however one particular case had a fatal neonatal outcome due to associated complications. Two interesting rare cases will be reviewed here.
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Craters of the moon: A marker for disease severity in alopecia areata? p. 314
Subrata S Malakar, Purva R Mehta, Surit S Malakar
DOI:10.4103/ijpd.IJPD_73_17  
In cases of severe alopecia areata in children, on close trichoscopic examination, we observed that the alopecic patch was closely packed with yellow dots of various sizes. In normal subjects, sebaceous glands of the scalp secrete sebum, but the sebaceous glands are attached to the hair follicle; hence, the sebaceous material remains attached to the hair follicle as it grows. However, in patients of alopecia areata, the sebum secreted by the sebaceous glands directly opens out onto the scalp surface as the alopecic patch is devoid of hair follicle to form a yellow dot. As it is secreted directly to the scalp surface, the sebum forms a depression on the surface of the scalp. The overview of these yellow dots is akin to the “craters of the moon.” We present a case series of severe alopecia areata wherein extensive yellow dots on trichoscopy resembled the “craters of the moon” and propose the term “craters of the moon” for yellow dots in severe alopecia areata.
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Goldenhar syndrome with rare clinical features p. 317
Vineet Relhan, Shankila Mittal, Khushbu Mahajan, Vijay Kumar Garg
DOI:10.4103/ijpd.IJPD_110_16  
Goldenhar syndrome is an uncommon syndrome with malformations of first and second branchial arches. Accessory tragus is a constant feature of this syndrome but may be associated with other syndromes as well. We report a case with a combination of rare findings, i.e., multiple bilateral preauricular skin tags with short stature and mental retardation with maintained facial symmetry.
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Subcutaneous panniculitis-like lymphoma in a 3-year-old child p. 321
Sudharani Chintagunta, Geetakiran Arakkal, Shahana Mohammed, Premajyothi Gopidi
DOI:10.4103/2319-7250.193028  
Panniculitis-like T-cell lymphoma is a rare cytotoxic T-cell lymphoma of the skin involving the subcutaneous tissue. We report panniculitis like lymphoma occurring in a 3 year old female child.She presented with multiple erythematous tender nodules and plaques on face,right thigh and genital region associated with systemic symptoms.The histopathology revealed lobular panniculitis composed of atypical lymphocytes . Immunophenotypic studies showed that the atypical lymphocytes are positive for CD3&CD8 and negative for CD20. The child improved with systemic antibiotics and supportive therapy. Now child is on regular follow up for the past four months without any new lesions.
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A pediatric case of primary cutaneous anaplastic large cell lymphoma: An unusual presentation p. 324
Gunjan Verma, Mukesh Manjhi, HK Kar
DOI:10.4103/2319-7250.193029  
Primary cutaneous anaplastic large cell lymphoma (PCALCL) is a rare form of ALCL, usually seen in elderly patients which is clinically characterized by single or multifocal nodules that ulcerate, autoregressive, and recurrent. Extracutaneous dissemination may occur, especially to regional lymph nodes. In this report, we present an unusual case of an 8-year-old boy with a noduloulcerativelesion present on the upper back with axillary painful lymphadenopathy, initially mimicking as scrofuloderma which was later histopathologically and immunohistochemically diagnosed as CD30+ PCALCL.
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Hematohidrosis: A rare case of a female child who sweat blood Highly accessed article p. 327
Geeta Kiran Arakkal, Srishilpa Poojari, G Narasimha Rao Netha, B Udaya Kumar
DOI:10.4103/2319-7250.193031  
Hematohidrosis is a rare clinical entity characterized by excretion of blood in the sweat. Hematohidrosis presents as unpredictable, recurrent episodes of spontaneous and self-limited bloody discharge from the intact skin. Diagnosis of hematohidrosis is confirmed by testing the secretions for the presence of blood components. It is an extremely rare condition, with very few case reports in literature. Herein, we describe a case of hematohidrosis in an 11-year-old female child with pain and tenderness who responded to propranolol.
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Fetal cardiomegaly: A rare presentation of parkes weber syndrome p. 330
Niraj Kumar Dipak, Ruchi Nimish Nanawati, Saumil Desai, Anita Ananthan
DOI:10.4103/2319-7250.206078  
Vascular malformations (VMs) are developmental abnormalities of the vascular system. VM can be divided into high flow (predominantly arteriovenous malformations) and low flow (capillaries, venous, lymphatic, and mixed). VMs may present antenatally, at birth, or may develop during childhood. The presence of high-flow lesion on Color Doppler favors the diagnosis of Parkes Weber syndrome (PWS) over Klippel-Trenaunay syndrome. The management of high output failure, skin, and orthopedic care remains the mainstay of therapy in PWS. We report a fetus with highly vascular swelling over left upper limb, which was detected when fetal cardiomegaly was alerted and looked out for its possible etiology. Fetal cardiomegaly demands meticulous sonographic screening of extremities of fetus as they often get missed.
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Kerion in a neonate p. 333
S Balasubramanian, K Vindhiya, K Dhanalakshmi, R Ramkumar
DOI:10.4103/2319-7250.206080  
Tinea capitis is predominantly an infection of children but extremely rare in neonates. Kerion is a T-cell mediated, severe pattern of an inflammatory fungal infection. It is characterized by tender, boggy nodular swelling with regional lymphadenopathy. There might be pus discharge and sinus formation that mimics bacterial folliculitis or an abscess of the scalp. Thick crusting with matting of hairs is common. Usually, area affected is limited, but multiple lesions are not rare. We report a 25-day-old neonate who presented with scalp swelling of 5 days duration. Baseline investigations were normal. Dermatological consult was obtained. Light and fluorescent microscopy along with fungal culture of the infected hair confirmed kerion due to Trichophyton mentagrophyte var. mentagrophyte. The infant was treated with griseofulvin and recovered completely.
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A sporadic case of epidermodysplasia verruciformis in a young boy p. 335
Rita V Vora, Rahulkrishna Sureshkumar Kota, Rochit R Singhal, Shailee S Gandhi
DOI:10.4103/2319-7250.206083  
Epidermodysplasia verruciformis (EV) is a rare disorder that is usually transmitted in an autosomal recessive manner, caused by human papillomavirus which presents with tinea versicolor-like or flat wart-like lesions. It has propensity for malignant transformation, especially squamous cell carcinoma. Here, we present a case of 7 year boy with complaints of asymptomatic hypopigmented macular lesions over the face, neck, and forearms. Histopathology was suggestive of EV and was given oral zinc, advised strict photoprotection.
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Bullous Henoch–Schonlein purpura with involvement of face p. 338
Devdeep Mukherjee, Ishita Majumdar, Priyankar Pal, Sandipan Dhar, Ritabrata Kundu
DOI:10.4103/2319-7250.188462  
Henoch–Schonlein purpura (HSP) with facial involvement with bullous rashes are extremely rare. A 12-year-old boy presented with abdominal pain and features of arthritis. He also had multiple purpuric rashes over his lower limbs. Gradually, he developed bullous rashes which were seen on his legs and hands and progressed to involve the face. He was confirmed to be suffering from HSP from clinical presentation and skin biopsy. The child responded well to oral steroids. Bullous lesions may be seen in HSP. However, there is neither prognostic significance of this nor does it alter the management. Other causes of bullous lesions should be ruled out. As facial involvement is associated with renal and gastrointestinal involvement, these children should be monitored for sequelae.
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Habitual biting of a finger in a child p. 341
KN Sarveswari, M Riswana Jasmine, N Mahesh
DOI:10.4103/2319-7250.193006  
A 3-year-old male child was brought by his parents with a nonhealing ulcer on the right middle finger having no significant history except for an injury sustained to the right elbow in December 2013. On further probing, the mother revealed that the child used to indulge in habitual biting of his right middle finger while watching TV. Initially he was investigated extensively by a vascular surgeon and no abnormality was detected. He was later referred to the dermatology department and on examination, the patient was attentive with normal behaviour. The right upper limb was slightly larger than left. There was no deformity of the right elbow. The right third fingertip was enlarged and mutilated. There was no nerve thickening or hypopigmented patch. There was loss of sensation on the right hand and arm. Differential diagnosis of Lesch–Nyhan syndrome and congenital sensory neuropathy were considered. The patient was referred to a neurologist who investigated further with magnetic resonance imaging (MRI), and the final diagnosis of syringomyelia was made based on MRI findings.
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Bullous pilomatricoma overlying a Bacillus Calmette–Guérin scar: A rare presentation p. 344
Ramchander , Pravesh Yadav, Puneet Pratap, Rubina Jassi
DOI:10.4103/2319-7250.206048  
Pilomatricoma is a benign hamartoma of hair matrix and the most commonly occurring hair follicle tumor. It classically presents as skin colored to erythematous, smooth, deep-seated nodule of firm consistency. Rarely, a bullous presentation of a pilomatricoma has also been reported. Herein, we illustrate a case of bullous pilomatricoma occurring over the Bacillus Calmette–Guérin scar site.
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Localized hypertrichosis at unusual sites p. 346
Anshul Maheshwari, Daulat Ram Dhaked, Ram Singh Meena
DOI:10.4103/2319-7250.215792  
Localized hypertrichosis is a rare entity characterized by circumscribed patches of terminal hair. It may be associated with many underlying systemic abnormalities. In our case, a 5-year-old girl presented with complaints of multiple patches of hypertrichosis without any systemic abnormality since the last 4 years. We are reporting this case due to the rarity of hypertrichosis at abnormal sites.
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LETTERS TO EDITOR Top

Drug reaction with eosinophilia and systemic symptoms mimicking Kawasaki disease Highly accessed article p. 349
Kunnummal Muhammed, Sabitha Saidharan Pillai, Shermin Nazrin, Deepthi N Sureshan, Minu Nagesh
DOI:10.4103/2319-7250.165677  
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Ocular abnormalities in childhood vitiligo - when to screen? p. 351
Manish K Shah
DOI:10.4103/2319-7250.215793  
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NOTICE OF RETRACTION Top

Retraction: Common superficial fungal infections in the pediatric age group p. 353

DOI:10.4103/2319-7250.216886  
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