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Harlequin Ichthyosis (HI) Associated with Atrial Septal Defect (ASD) and Choanal Atresia

Abstract Harlequin ichthyosis (HI) is a severe form of congenital ichthyosis with autosomal recessive inheritance. Incidence of harlequin ichthyosis is 1 in 3,00,000 live births. We report a case of HI associated with bilateral choanal atresia and atrial septal defects, which is a rare association in this skin disorder. A-month-old preterm male baby born out of consanguineous marriage presented with features of armour-like scales and erythema all over body, ectropion, eclabium and fissures over flexures. The patient was born with a colloidion membrane at birth. The baby was operated for bilateral choanal atresia soon after birth because he developed cyanosis upon breast feeding which improved on crying. Upon flexible nasal endoscopy, diagnosis of membranous type of choanal atresia was confirmed by ENT (ear, nose, throat) surgeon. Heart auscultation revealed a murmur in our patient. Electrocardiogram and 2D Echocardiography was reported as atrial septal defect (4.5 mm OsASD). The patient was started on acitretin (1 mg/kg/day) and emollients after complete evaluation and is currently on regular follow up. Harlequin ichthyosis is linked to mutation of ABCA12 gene. It is often associated with eclabium, ectropion, hypoplastic nose, ears and fingers. Congenital heart diseases are rarely reported with HI in literature. This makes it mandatory to screen HI patients for internal defects.

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Salicylic Acid Peel and Fusion Peel with Salicylic & Mandelic Acid Peel for Treatment of Acne Scars - A Comparative Study

Abstract Introduction. Acne vulgaris is one of the most common skin diseases affecting more than 85% of individuals worldwide. The study aimed to compare the therapeutic efficacy of Salicylic acid peel and Salicylic acid - mandelic acid peel for treatment of acne scars. Material and Methods. A comparative study was conducted on 41 patients, divided into two groups based on treatment choice by the subjects. Group A (23) were treated with 30% salycilic acid peels, and group B (18) were treated with 20% salycilic acid and 10% mandelic acid peels at an interval of 2 weeks for three sessions. The physician’s global assessment scale and clinical photographs were used at the end of three sessions. All the data were analysed using SPSS considering P<0.05 as significant. Results. Of the total 41 patients analyzed, the mean age in both the groups were comparable. (Group A: 20.4 years; Group B: 20.5 years). Duration of acne was >12 months in both the groups 13 (56.52%) & 10 (55.56%)). Thirteen patients (56.52%) in group A and 1 (5.56%) in group B had no side effects which was statistically significant (p=0.01). Using the physician’s global assessment scale 51-75% (Good) an improvement was seen in grade I 4 (17%) and 18 (78%) in grade II in group A and 2 (11%) in grade I and 12 (67%) in grade II in group B, respectively which was statistically significant (p=0.001). Conclusion. Both agents showed almost equal efficacy in improving mild-to-moderate acne scars. Adverse effects were less with Salicylic acid peel compared with mandelic acid peel..

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