Tehran University of Medical Sciences Immunology and Genetics Journal 2645-4831 9 1 2025 12 22 222 222 Shirin Mohamadi Division of Neonatology, Department of Pediatrics, Shohadaye Tajrish Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Fahimeh Abdollahimajd Department of Dermatology, Shohada-e Tajrish Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Soheila Sotoudeh Department of Dermatology, Children's Medical Center, Center of Excellence, Tehran University of Medical Sciences, Tehran, Iran 2025 12 15 2025 12 15 Incontinentia Pigmenti (IP) is a rare X-linked dominant genetic disorder primarily affecting the skin, with cutaneous manifestations present in all cases. Other ectodermal tissues—including the central nervous system, eyes, hair, nails, and teeth—may also be involved to varying degrees. In this report, we present the case of a newborn female who exhibited widespread vesiculopustular skin lesions at birth, predominantly affecting the upper and lower extremities. At 9 hours old, the newborn with skin lesions suspicious for generalized impetigo, transferred to the NICU at Children’s Medical Center in Tehran, Iran, for further evaluation and management. No abnormalities were observed in the hair, nails, oral mucosa, eyes, or central nervous system during the initial assessment and the final diagnosis was IP. https://igj.tums.ac.ir/index.php/igj/article/view/222

Tehran University of Medical Sciences Immunology and Genetics Journal 2645-4831 9 1 2025 12 22 221 221 Pegah Mehdiyar Department of Pediatrics, Faculty of Medicine, Tehran Medical Sciences, Islamic Azad University, Tehran, Iran. Hassan Abolhassani Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran Nima Rezaei Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran Sepideh Darougar Department of Pediatrics, Tehran Medical Sciences Branch, Islamic Azad University, Tehran, Iran Samin Sharafian Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Zahra Chavoshzadeh Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran 2025 11 20 2025 12 22 Background: Severe combined immunodeficiencies (SCIDs) are a group of disorders with variable clinical phenotypes, usually presenting with life-threatening infections. This type of immunodeficiency results from defective differentiation of hematopoietic stem cells into mature T lymphocytes leading to various identified affected genotypes of severe immunodeficiency. Omenn syndrome is an autosomal recessive immunodeficiency disorder characterized by generalized erythroderma, lymphoadenopathy, and eosinophilia. The aim of this study was to provide specific information about the clinical, immunological, and genetic characteristics in this context. Methods: A retrospective case review was conducted at Shahid Beheshti, Children Medical Center and Azad University Hospitals of Tehran so that the patients with a previously diagnosis of Omenn syndrome, admitted between years 2016 and 2023, were selected and included in this study. Results: Eleven patients with known Omenn syndrome were included in our study. The mean age of onset in the patients was 45 days old. Six (54.5%) were female and 5 (45.5%) were male. There was a history of parental consanguinity in 10 out of 11 studied children (91%). BCG dissemination, erythroderma, hepatosplenomegaly, lymphadenopathies, failure to thrive, recurrent infections, and gastrointestinal manifestations were more prominent. Other presentations in order of frequency were failure to thrive (90.9%), recurrent infections (63.6%), erythroderma (63%), hepatosplenomegaly (45.5%), lymphadenopathy (36.4%), and BCG dissemination (27.3%). Conclusion: As Omenn syndrome is a type of SCID and a pediatric immunologic emergency, awareness about the various clinical manifestations of the disease among people of different ethnicities is highly essential for timely and accurate diagnosis, treatment, and family counseling. https://igj.tums.ac.ir/index.php/igj/article/view/221

Tehran University of Medical Sciences Immunology and Genetics Journal 2645-4831 9 1 2026 02 14 225 225 Reza Yazdani Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran, Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran Samaneh Abdolahzadeh Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Marjan Aghajani Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran Mina Ahmadi Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Mostafa Alavi-Moghaddam Department of Emergency Medicine, Imam Hossein Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Saba Arshi Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Maryam Asarehzadegan Dezfouli Allergy and Clinical Immunology Clinic, AbuRehan Polyclinic, Social Security Organization, Tehran, Iran Marzieh Asgharyan Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Parisa Ashournia Department of Allergy and Clinical Immunology, Bahrami Hospital, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran Ayda Askarisarvestani Allergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran Raheleh Assari Pediatric Rheumatology Research Group, Rheumatology Research Center, Tehran, Iran 11. Department of Pediatrics, Children's Medical Center, Pediatrics Center of Excellence, Tehran University of Medical Sciences, Tehran, Iran Hadi Badiee Shahid Beheshti University of Medical Sciences, Tehran, Iran Sima Bahrami Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Maryam Behfar Pediatric Cell and Gene Therapy Research Centre, Gene, Cell & Tissue Research Institute, Tehran University of Medical Sciences, Tehran, Iran Nasrin Behniafard Children Growth Disorder Research Center, Shahid Sadoughi University of Medical Sciences, Yazd, Iran Zahra Chavoshzadeh Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Mahsa Choroom Kheirabadi Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran Zahra Daneshmandi Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran Mohammad Hassan Bemanian Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Taher Cheraghi Department of Pediatrics, 17th Shahrivar Children's Hospital, Guilan University of Medical Sciences, Rasht, Iran Kian Darabi Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Sepideh Darougar Department of Pediatrics, Faculty of Medicine, Tehran Medical Sciences, Islamic Azad University, Tehran, Iran Sarehsadat Ebrahimi 18. Department of Immunology and Allergy, Kerman University of Medical Sciences, Kerman, Iran; Endocrinology and Metabolism Research Center, Institute of Basic and Clinical Physiology Sciences, Kerman University of Medical Sciences, Kerman, Iran Golnaz Eslamian Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Maryam Esmaeilbeig Department of Allergy and Clinical Immunology, Shiraz University of Medical Sciences, Shiraz, Iran Morteza Fallahpour Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Nader Faraji Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Shahin Farzadmanesh Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran Kimia HajiAbbasi Someesaraie Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Arash Kalantari Tehran University of Medical Sciences, Imam Khomeini Hospital Complex, Tehran, Iran Mahdieh Karimizadeh Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Elham Kaviani Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Mehrnaz Mesdaghi Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Majid Marjani Clinical Tuberculosis and Epidemiology Research Center, National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran, Iran Mohammadreza Modaresi Pediatric Pulmonary Disease and Sleep Medicine Research Center, Pediatric Center of Excellence, Children's Medical Center, Tehran, Iran Iraj Mohammadzadeh Non-communicable Pediatric Diseases Research Center, Health Research Institute, Babol University of Medical Sciences, Babol, Iran Mahshid Movahedi Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran; Tehran University of Medical Sciences, Imam Khomeini Hospital Complex, Tehran, Iran Masoud Movahedi Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran Mostafa Moosavian Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran Mohammamd Nabavi Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Leyla Norouzi-Barough Immunodeficiency Disease Research Center, Isfahan University of Medical Sciences, Isfahan, Iran Anahita Razaghian Department of Allergy and Clinical Immunology, Hakim Children’s Hospital, Tehran University of Medical Sciences, Tehran, Iran Mansoureh Shariat Department of Immunology and Allergy, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran Sahar Seraj Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Soodeh Seyedi Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran Seyedehshabnam Seyedsalehi Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Alireza Shafiei Department of Allergy and Immunology, Bahrami Children’s Hospital, Tehran University of Medical Sciences, Tehran, Iran Zahra Shahraki Ghadimi Department of Pediatrics, Clinical Immunology Research Center, Zahedan University of Medical Sciences, Zahedan, Iran Behzad Shakerian Department of Immunology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran Samin Sharafian Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Sima Shokri Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Marziyeh Tavakol Non-Communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran Ahmad Vosughi Motlagh Department of Pediatrics, North Khorasan University of Medical Sciences, Bojnurd, Iran Nima Rezaei Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Science, Tehran, Iran, Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran 2026 02 14 2026 02 14 At present, a national consensus on hematopoietic stem cell transplantation (HSCT) for patients with inborn errors of immunity (IEI) is lacking. This consensus is written based on a combination of scientific literature and comments from the expert panel of Iranian immunologists. We formed a panel of clinical immunologists at a meeting titled “Second Meeting on the Diagnosis of IEI by IEI Experts” to receive their comments in this field. All authors reviewed and agreed on the current consensus. This consensus guideline provides recommendations on donor selection, stem cell source, conditioning regimen, mobilization and collection, stem cell infusion, engraftment and chimerism assessment, and post-transplant care for patients with IEI. The current recommendations reflect Iranian practice and do not necessarily represent global preferences. https://igj.tums.ac.ir/index.php/igj/article/view/225

Tehran University of Medical Sciences Immunology and Genetics Journal 2645-4831 9 1 2026 02 16 226 226 Saba Fekrvand Research Center for Immunodeficiencies, Children’s Medical Center, Tehran University of Medical sciences Samaneh Abdolahzade Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Marjan Aghajani Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran Mina Ahmadi Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Mostafa Alavi-Moghaddam Department of Emergency Medicine, Imam Hossein Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Saba Arshi Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Maryam Asarehzadegan Dezfouli Social Insurance Organization Marzieh Asgharyan Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Parisa Ashournia Department of Allergy and Clinical Immunology, Bahrami Hospital, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran Aida Askari Sarvestani Allergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran Raheleh Assari Pediatric Rheumatology Research Group, Rheumatology Research Center, Tehran, Iran Hadi Badiee Shahid Beheshti University of Medical Sciences, Tehran, Iran Sima Bahrami Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Maryam Behfar Pediatric Cell and Gene Therapy Research Centre, Gene, Cell & Tissue Research Institute, Tehran University of Medical Sciences, Tehran, Iran Nasrin Behniafard Children Growth Disorder Research Center, Shahid Sadoughi University of Medical Sciences, Yazd, Iran Zahra Chavoshzadeh Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Mahsa Choroom Kheirabadi Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran Zahra Daneshmandi Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran Mohammad Hassan Bemanian Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Taher Cheraghi Department of Pediatrics, 17th Shahrivar Children's Hospital, Guilan University of Medical Sciences, Rasht, Iran Kian Darabi Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Sepideh Darougar Department of Pediatrics, Faculty of Medicine, Tehran Medical Sciences, Islamic Azad University, Tehran, Iran Sarehsadat Ebrahimi Department of Immunology and Allergy, Kerman University of Medical Sciences, Kerman, Iran Golnaz Eslamian Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Maryam Esmaeilbeig Department of Allergy and Clinical Immunology, Shiraz University of Medical Sciences, Shiraz, Iran Morteza Fallahpour Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Nader Faraji Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Shahin Farzadmanesh Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran Kimiya HajiAbbasi Someesaraie Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Arash Kalantari Tehran University of Medical Sciences, Imam Khomeini Hospital Complex, Tehran, Iran Mahdieh Karimizadeh Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Elham Kaviani Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Mehrnaz Mesdaghi Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Majid Marjani Clinical Tuberculosis and Epidemiology Research Center, National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran, Iran Mohammadreza Modaresi Pediatric Pulmonary Disease and Sleep Medicine Research Center, Pediatric Center of Excellence, Children's Medical Center, Tehran, Iran Iraj Mohammadzadeh Non-communicable Pediatric Diseases Research Center, Health Research Institute, Babol University of Medical Sciences, Babol, Iran Mahshid Movahedi Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran Masoud Movahedi Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran Mohammad Nabavi Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Leyla Norouzi-Barough Immunodeficiency Disease Research Center, Isfahan University of Medical Sciences, Isfahan, Iran Anahita Razaghian Department of Allergy and Clinical Immunology, Hakim’s Children Hospital, Tehran University of Medical Sciences, Tehran, Iran Mansoureh Shariat Department of Immunology and Allergy, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran Sahar Seraj Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Soodeh Seyedi Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran Seyedehshabnam Seyedsalehi Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Alireza Shafiei Department of Allergy and Immunology, Bahrami Children’s Hospital, Tehran University of Medical Sciences, Tehran, Iran Zahra Shahraki Ghadimi Department of Pediatrics, Clinical Immunology Research Center, Zahedan University of Medical Sciences, Zahedan, Iran Behzad Shakerian Department of Immunology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran Samin Sharafian Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Sima Shokri Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Marzieh Tavakol Non-Communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran Ahmad Vosughimotlagh Immunology and Allergy Department, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Reza Yazdani Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Science, Tehran, Iran Nima Rezaei Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Science, Tehran, Iran 2026 02 16 2026 02 16 None. https://igj.tums.ac.ir/index.php/igj/article/view/226

Tehran University of Medical Sciences Immunology and Genetics Journal 2645-4831 9 1 2025 12 22 223 223 Farid Imanzadeh Hepatology and Nutrition Research Center, Research Institute for Children's Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran Samaneh Vahdat nia Hepatology and Nutrition Research Center, Research Institute for Children's Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran 2025 12 15 2025 12 15 The most prevalent reason of trichobizoar diseases are hair pulling (trichotillomania) and hair eating (trichophagia). It occurs most often in women under 30, which associated with trichotillomania. Trichobiosis of the stomach is very rare. If diagnosed on time and treated successfully, it does not cause considerable complications. In this study, we report a case of a 10-years-old female with epigastric pains for 2 months. The patient underwent diagnostic endoscopy due to a palpable mass in her epigastrium revealing gastric trichobezoar for which, she underwent surgical intervention. Detailed history taken from her revealed the history of trichotillomania and trichophagia since 3 years of age. https://igj.tums.ac.ir/index.php/igj/article/view/223