Raoultella ornithinolytica is a Gram-negative encapsulated aerobic bacillus belonging to family Enterobacteriaceae. It is an extremely rare causative organism of infections in humans. We reported R. ornithinolytica sepsis and purpura fulminans in a preterm female baby. Our case is the second newborn case of human infection by R. ornithinolytica that has been reported in the literature.

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Topical corticosteroids (TC) have been in use for more than half a century and are useful for the treatment of various inflammatory disorders in all age groups including children. Children, however, form a special age group because their skin, being more thin and tender, is more susceptible to the side-effects of the drug. Hence dermatologists must be aware of this special situation where TC must be prescribed with utmost caution. Counselling of parents and others handling the children is also imperative for judicious use of the drug. Planned withdrawal of the drug as early as possible helps in avoiding tachyphylaxis and most of the side-effects of the drug.

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Parakeratosis pustulosa (PP) is not so rare an entity and remains under reported in the literature perhaps for the simple reason of mistaken identity. The literature on PP was reviewed for epidemiology, etiology, clinicopathologic features, differential diagnosis, clinical course, and the therapeutic measures described. A Medline search (PubMed, Google, IndMed) carried out on 30 December 2012 revealed only 10 reports under the heading "parakeratosis pustulosa"; three were in languages other than English. This chronic dermatosis is characterized by erythemato-squamous lesions affecting the periungual skin/nail unit in children aged ≤5 years, mostly females. Nails show pitting and cross-ridging of one or more digits especially of the fingers. Its etiology remains obscure, and onychomycosis, psoriasis or pustular psoriasis, and atopic or contact dermatitis affecting the nail are important differentials. Acrodermatitis continua of Hallopeau remains the most common misdiagnosis in these patients. Topical emollients remain the recommended treatment, while topical corticosteroids or tretinoin are needed for recalcitrant cases. Evaluation of this as a distinct entity having benign, self-limiting clinical course and good prognosis will save the patient from expensive therapy and unnecessary distress associated with other more mutilating pustular dermatoses affecting the nail unit.

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We present a 5-year-old girl with Type A woolly hair nevus. This is a rare non-hereditary focal condition characterized by unruly and tightly coiled hair. It may be associated with epidermal or melanocytic nevus. Children with woolly hair must be examined completely and monitored regularly to rule out cardiofaciocutaneous and Noonan syndrome.

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Cutaneous drug reactions (CDR) are common in pediatric age group and can be important not only for further therapeutic strategy but also for future of the child. CDR can be classified based on the pathogenesis, morphology, dose relation and predictability. About 3% of patients admitted in hospital have adverse drug reaction. CDR can be due to both immunological and nonimmunological reactions, with nonimmunological reactions being more common. Morphologically CDR in children is divided into five subtypes with exanthematous type being most common. Meticulous history and examination are the basis of diagnosis of CDR. Management is primarily withdrawal of the offending agent and supportive and symptomatic care. Such patients should be advised to carry a drug diary so as to maintain a record of the offending drug/drugs and also to guard against future adverse events.

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Background: Leprosy is a major public-health problem in developing countries. According to National Leprosy Elimination Program report of March 2012, there were a total of about 0.13 million cases of leprosy in India, 9.7% of which were children. Studies pertaining to proportion and characteristics of pediatric cases are few in number. Aim: The aim of the following study is to examine clinical, bacteriological and histopathological characteristics of pediatric leprosy cases in community. Methods: A retrospective, analytical study of patients seen from 01 January 2009 to 30 June 2013 in dermatology out-patient department of tertiary care center in Mumbai, Maharashtra. Results: Study yielded 24 pediatric cases of leprosy. The age of child leprosy cases ranged from 3 to 12 years with mean of 9.25 ΁ 2.33. Most of the cases were multibacillary (MB) (95.8%). A large proportion of children (45.8%) had single skin lesion (SSL). Of the 11 SSL cases examined histopathologically, 8 (72.7%) showed features of borderline tuberculoid, 1 (9%) each of borderline lepromatous, indeterminate type and tuberculoid leprosy. Overall, one case had deformity (Grade 2) and 8.7% of MB cases were smear positive. Overall 2 patients had history of contact. Conclusion: The clinical, bacteriological and histopathological characteristics of cases in children, especially the high percentage of MB cases evidently indicate the grave nature of the problem of undetected child leprosy, recent active transmission and highlight implications on individual patients and the community.

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Kindler syndrome is a rare genodermatosis with autosomal recessive inheritance and less than 150 cases reported worldwide. It results from loss of function mutation in the KIND1 gene (FERMT1), which has been localized to the short arm of chromosome 20. Onset is just after birth with traumatic blistering healing without scarring, skin fragility, progressive poikiloderma and photosensitivity improving with age. Systemic complications and fatally aggressive squamous cell carcinoma have also been reported. Here, we report a case with classic dermatologic manifestations in an 8-year-old female along with a brief review of literature.

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A 12-year-old non-obese male presented with multiple yellowish, non-tender soft papulonodular lesions over multiple sites along with firm, subcutaneous swellings along achilles tendons bilaterally since the age of 2 years. There was no history of similar complaints in family. Lipid profile revealed high levels of serum cholesterol and low density lipoproteins. Histopathological examination revealed diffuse dermal infiltrate of foamy histiocytes. He was diagnosed as a case of Type IIa hyperlipoproteinemia with different types of xanthomas. The patient was adviced dietary fat intake control and started on tablet atorvastatin 40 mg daily. Complete work up of all the other family members was adviced.

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Toxic epidermal necrolysis (TEN) is a severe adverse cutaneous drug reaction characterized by widespread keratinocyte death. Clinically, TEN is characterized by rapidly progressing erythematous, purpuric rash to widespread blistering and denudation of skin and severe mucositis, with fatal complications like sepsis and multiorgan failure. TEN is considered to be rare in infants and usually has a fatal outcome due to sepsis. As yet, only few reports have been documented in the literature. We hereby report a case of TEN in a young infant successfully treated with intravenous immunoglobulin (IVIG).

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Background: Reports from different parts of the country have revealed wide variation in the clinical profile of childhood vitiligo. Variations exist with respect to age, sex, family history, Koebner phenomena and presence of other cutaneous disease. Aim: The aim of the following study is to assess the clinical profile and other cutaneous disease in childhood vitiligo. Materials and Methods: This study was designed to find the clinical characteristics of childhood vitiligo (in the age group < 12 years) in the Outpatient Department of Dermatology, Venereology and Leprology, Government medical college, Kota, between July 2012 and June 2013. A total of 35 childhood vitiligo patients were examined. Results: Out of 35 children, 20 patients (57.14%) were female and 15 patients (42.86%) were male. In most of the patients, disease onset was < 10 year of age and mean age of onset was 6.64 ΁ 1 year. The most common pattern observed in childhood vitiligo was vitiligo vulgaris (17 patients) followed by focal (9 patient). Other less common pattern were segmental (4 patients), mucosal (2 patients), mixed (1 patient), contact (1 patient), acrofacial (1 patient). Out of 35 patients, 6 patients had a family history, 12 patients had Koebner phenomena, and 3 patients had leucotrichia. Other cutaneous dermatoses was found in 4 patients out of which 3 patients had halo nevus and 1 patient had atopic dermatitis. Conclusion: Childhood vitiligo in Hadoti region showed a preponderance in females and most common pattern were vitiligo vulgaris followed by focal vitiligo.

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Congenital cutaneous candidiasis is an uncommon and benign infection presenting at birth or within 6 days of life in neonates exposed to candida in utero. It presents with generalized erythematous macules, papules, vesicles and pustules with or without systemic symptoms. We describe a full term newborn child with congenital cutaneous candidiasis born to an asymptomatic mother. Awareness of this condition is necessary to make an early diagnosis and differentiate from other neonatal infections.

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Ectodermal Dysplasia (ED) is a group of rare disorders that affects various tissues of ectodermal origin. The commonest being hypohidrotic (anhydrotic) ED. We report a case of 2 year old female child, brought with complaints of cough, cold and fever since 1 week and breathlessness that preceeded 3 days before admission. There was h/o heat intolerance, and hypohydrosis. The other two siblings were unaffected. Examination, revealed a febrile, tachypnoeic, tachycardic child, with sparse hair and eyebrows, coarse dry paper-like skin, and periorbital hyperpigmentation. Dentition included 4 peg like incisors. Respiratory examination revealed decreased air entry on the left side and crepitations over the right side. Chest x-ray and ultrasonography were consistent with left sided pleural effusion. Pathological and biochemical examination of pleural fluid were suggestive of empyema. Other systems were normal. Treatment included intercostal drainage and antibiotics for a period of four weeks in a cool environment. Symptoms resolved without sequelae and tube was removed on 5 ^th day of stay. Skin biopsy corroborated the diagnosis of ED. Immunoglobin profile was normal. Mutational analysis could not be done. This case is being reported due to a rare occurrence of Hypohidrotic ED and its much rarer association with underlying empyema.

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A 16-year-old female with a travel history to Peru and a developing subcutanous mass previously misdiagnosed as an abscess presented in our out-patient clinic. Upon inspection a parasite was detectable at a small entrance-channel on top of the lesion. We removed the parasite after local anesthesia. It was recognized as a Dermatobia hominis larva. A sequencing analysis was also performed to confirm the exact dipteran subspecies. Infestations with D. hominis are fairly common in tropical America but rather unknown to physicians in the northern hemisphere, although they comprise up to 10% of imported diseases from overseas. A travel history and a failure of antibiotic treatment are hallmarks that led to the correct diagnosis in previous reports. As world-wide travels become even more common a detailed travel history is mandatory to consider non-resident pathogens as possible cause of diseases.

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Epidermolysis bullosa (EB) represents a group of hereditary skin disorders manifested by an exceptional tendency of the skin and mucosa to form bullae and vesicles following minor friction and trauma. The pathogenesis of these disorders is unknown. Depending on the type, the clinical manifestations vary from very mild to a disfiguring and disabling disease. Along with the systemic complications, the oral findings like early childhood caries, microstomia, and limited mouth opening are quite evident in these children. Health care professionals should be aware of preventive and therapeutic measures to be adopted in order to provide a safe and effective dental treatment in these patients. The present case documents a child diagnosed of EB with early childhood caries, describing the phases and difficulties of dental treatment, and also presents a novel impression technique.

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