Global Retinoblastoma Presentation and Analysis by National Income Level.

Importance: Early diagnosis of retinoblastoma, the most common intraocular cancer, can save both a child's life and vision. However, anecdotal evidence suggests that many children across the world are diagnosed late. To our knowledge, the clinical presentation of retinoblastoma has never been assessed on a global scale. Objectives: To report the retinoblastoma stage at diagnosis in patients across the world during a single year, to investigate associations between clinical variables and national income level, and to investigate risk factors for advanced disease at diagnosis. Design, Setting, and Participants: A total of 278 retinoblastoma treatment centers were recruited from June 2017 through December 2018 to participate in a cross-sectional analysis of treatment-naive patients with retinoblastoma who were diagnosed in 2017. Main Outcomes and Measures: Age at presentation, proportion of familial history of retinoblastoma, and tumor stage and metastasis. Results: The cohort included 4351 new patients from 153 countries; the median age at diagnosis was 30.5 (interquartile range, 18.3-45.9) months, and 1976 patients (45.4%) were female. Most patients (n = 3685 [84.7%]) were from low- and middle-income countries (LMICs). Globally, the most common indication for referral was leukocoria (n = 2638 [62.8%]), followed by strabismus (n = 429 [10.2%]) and proptosis (n = 309 [7.4%]). Patients from high-income countries (HICs) were diagnosed at a median age of 14.1 months, with 656 of 666 (98.5%) patients having intraocular retinoblastoma and 2 (0.3%) having metastasis. Patients from low-income countries were diagnosed at a median age of 30.5 months, with 256 of 521 (49.1%) having extraocular retinoblastoma and 94 of 498 (18.9%) having metastasis. Lower national income level was associated with older presentation age, higher proportion of locally advanced disease and distant metastasis, and smaller proportion of familial history of retinoblastoma. Advanced disease at diagnosis was more common in LMICs even after adjusting for age (odds ratio for low-income countries vs upper-middle-income countries and HICs, 17.92 [95% CI, 12.94-24.80], and for lower-middle-income countries vs upper-middle-income countries and HICs, 5.74 [95% CI, 4.30-7.68]). Conclusions and Relevance: This study is estimated to have included more than half of all new retinoblastoma cases worldwide in 2017. Children from LMICs, where the main global retinoblastoma burden lies, presented at an older age with more advanced disease and demonstrated a smaller proportion of familial history of retinoblastoma, likely because many do not reach a childbearing age. Given that retinoblastoma is curable, these data are concerning and mandate intervention at national and international levels. Further studies are needed to investigate factors, other than age at presentation, that may be associated with advanced disease in LMICs

A clinicomorphological study of childhood herpes zoster at a rural based tertiary center, Gujarat, India

Aims and Objectives: To study the clinical features of herpes zoster (HZ) in childhood along with prevalence of human immunodeficiency virus (HIV) seropositivity. Materials and Methods: The study was carried out in the Department of Dermatology at a Tertiary Care Centre of Gujarat, India, for 6 years. Children aged ≤12 years with a diagnosis of HZ seen in the Departments of Dermatology were enrolled in a predesigned pro forma. Diagnosis of HZ was made on clinical grounds, confirmed by tzanck smear as and when required. Sera of all cases were tested for HIV. Results: Total of 34 children aged ≤12 years were enrolled in the study. Nineteen (55.88%) were boys and 15 (44.12%) were girls. The mean age was 9.26 years. In 97.06% patient have localized dermatomal involvement. Most common symptom was burning pain seen in 30 (88.24%) patients. Previous history of chickenpox was present in 19 (55.88%) patients. Evidence of immunosuppression on history, clinical examination, and investigations was present only in one patient, who had HIV infection. Conclusion: Although there is increased incidence of HZ in childhood, atypical presentations are rare, multidermatomal involvement is not commonly seen. Majority of these children do not show immunosuppression. Hence, we conclude HZ in childhood occurs as a relatively mild and self-limiting disease

Michelin tire baby syndrome

Michelin tire baby syndrome (MTBS), is a rare genodermatosis, characterized by generalized folding of excess skin and may be associated with various phenotypic abnormalities. The pathogenesis of this condition is unclear. Various congenital anomalies can be associated with it. It may be a clinical finding associated with various syndromes. Diagnosis is mainly clinical, and skin folds gradually diminish and disappear with age without any intervention. Here, we report a case of MTBS associated with convergent squint and hydrocephaly

A sporadic case of epidermodysplasia verruciformis in a young boy

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

A study of fixed-drug reactions at a rural-based tertiary care center, Gujarat

Context: Adverse cutaneous drug reactions (ACDRs) are very common due to a wide variety of drugs available in markets. Fixed-drug reaction (FDR) is a type of ACDR that dermatologists are facing frequently nowadays. There is a need to identify various drugs responsible for FDR and to ensure the safety of the patients with proper counseling. Aim and Objective: The aim and objective was to study the demographic details, clinical patterns, and the offending drugs causing FDR. Setting and Design: This was a cross-sectional, observational study. Materials and Methods: The study was carried out from April 2010 to March 2015 in the Department of Dermatology at a rural-based tertiary care center of Gujarat, India, after taking approval from the hospital's research ethical committee. A detailed history taking and thorough clinical examination were done for all the patients having FDR and were recorded in a predesigned pro forma. Analysis was done using frequencies, proportions, and Chi-square test. All the patients were educated regarding ACDRs and given a list of drugs causing FDR to avoid recurrence. Results: A total of 59 patients were studied for FDR among which 32 (54.23%) were males and 27 (45.76%) were females. Fever (20.34%) was the most common illness for which patients had taken the culprit drug. Antimicrobials (26 [44.07%]) were the most common group of drugs causing FDR followed by nonsteroidal anti-inflammatory drugs (21 [35.59%]). As a single molecule, diclofenac was the most common drug causing FDR followed by metronidazole 5 (8.47%) and cotrimoxazole, fluconazole, and ciprofloxacin 4 (6.78%) each. The most common complaint following intake of the culprit drug was pigmented patch (31 [52.54%]) followed by blisters (13 [22.03%]). Conclusion: FDR is one of the important ACDRs seen in patients. Antimicrobials are the most common group while diclofenac is the most common drug causing FDR