en آلرژی و ایمونولوژی بالینی Allergy and Clinical Immunology Case & Review Case & Review <div style="text-align: justify;"><strong>Introduction</strong>: Netherton Syndrome (NS) is a rare hereditary autosomal recessive disorder with ichthyosiform cutaneous lesions, specific hair shaft defect, and atopic diathesis. The incidence of NS is estimated to be approximately 1 in 200000. The objective of this case report is to present NS in a patient with severe eczema atopic dermatitis-like eruption.<br> <strong>Case Presentation</strong>: A 41-month-old boy was referred to the clinic of Allergy and Immunology, Hazrat Rasoul Hospital with generalized erythema, and scaling cutaneous lesions. The patient underwent clinical examinations and laboratory analysis. His laboratory data revealed only an elevated IgE level with a leukocyte count of 7800/&mu;L containing 10% eosinophil. His hair shaft indicated classic trichorrhexis invaginata (Bamboo hair). Based on the clinical and laboratory findings, he was diagnosed with NS. A brief review was also done related to this case.<br> <strong>Conclusion</strong>: This case was reported because of the severity of the disorder and other differential diagnoses in severe and refractory atopic dermatitis-like eruptions. The importance of the case is related to other differential diagnoses, especially with early onset disorders at neonatal age.</div> <div class="ginger-module-correctionBubble" contenteditable="false" style="padding: 0px; top: 50px; left: 464.188px; display: none;"> <div class="ginger-module-correctionBubble-container ginger-module-correctionBubble-container-loggedout" style="display: none;"> <div class="ginger-module-correctionBubble-login">Log in</div> <div class="ginger-module-correctionBubble-body">to use Ginger</div> </div> <div class="ginger-module-correctionBubble-container ginger-module-correctionBubble-container-loggedin"> <div class="ginger-module-correctionBubble-limited">Limited mode</div> <div class="ginger-module-correctionBubble-body"><gwbm class="ginger-module-correctionBubbleService-mistake-type-1 ginger-module-correctionBubbleService-mistake-nosuggestion" id="gwbm-15481303008443964430959">ichthyosiform<gwbmo><a class="ginger-module-correctionBubbleService-mistake-bubble-add" title="Add to Personal Dictionary"></a></gwbmo></gwbm></div> <a class="ginger-module-correctionBubble-close" href="ja<illegal tag>vascript:" title="Ignore mistake">&times;</a></div> </div> Netherton syndrome, Congenital ichthyosis, Trichorrhexis invaginata 223 228 http://jpr.mazums.ac.ir/browse.php?a_code=A-10-797-1&slc_lang=en&sid=1 Fereshteh Salari drfereshtehsalari@gmail.com 10031947532846003997 10031947532846003997 No Department of Allergy and Clinical Immunology, Hazrat Rasoul Hospital, Iran University of Medical Sciences, Tehran, Iran. Leila Bani Adam dr.leilabaniadam@gmail.com 10031947532846003998 10031947532846003998 No Department of Allergy and Clinical Immunology, Hazrat Rasoul Hospital, Iran University of Medical Sciences, Tehran, Iran. Saba Arshi sabaarshi@yahoo.com 10031947532846003999 10031947532846003999 No Department of Allergy and Clinical Immunology, Hazrat Rasoul Hospital, Iran University of Medical Sciences, Tehran, Iran. Mohammad Hassan Bemanian mhbemanian@yahoo.com 10031947532846004000 10031947532846004000 No Department of Allergy and Clinical Immunology, Hazrat Rasoul Hospital, Iran University of Medical Sciences, Tehran, Iran. Morteza Fallahpour fallahpour.m@iums.ac.ir 10031947532846004001 10031947532846004001 No Department of Allergy and Clinical Immunology, Hazrat Rasoul Hospital, Iran University of Medical Sciences, Tehran, Iran. Mohammad Nabavi mnabavi44@yahoo.com 10031947532846004002 10031947532846004002 Yes Department of Allergy and Clinical Immunology, Hazrat Rasoul Hospital, Iran University of Medical Sciences, Tehran, Iran.