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Association of Facial Pustular Neutrophilic Eruption With Messenger RNA–1273 SARS-CoV-2 Vaccine

Eric Dean Merrill, Sakeen W. Kashem, Erin Amerson, Laura B. Pincus, Ursula E. Lang, Kanade Shinkai, Aileen Y. Chang

2021JAMA Dermatology21 citationsDOI

Abstract

diagnostic and therapeutic challenge.Cytotoxic Tlymphocyte antigen-4 is expressed on regulatory T cells and binds ligands CD80 and CD86 on antigen-presenting cells to halt T-lymphocyte activation. 2 When dysfunctional, the T-cell activation leads to lymphoproliferation and autoimmunity and clinically presents with common recurrent infections, such as sinus, ear, respiratory (61%), hypogammaglobinemia (84%), autoimmune cytopenias (62%), gastrointestinal symptoms (59%), endocrinopathies (33%), neurological features (including headaches) (29%), and arthritis (14%). 1,2Nonmalignant Tcell lymphoproliferation, such as hepatosplenomegaly, lymphadenopathy, and lymphocytic lung infiltrates, can be seen in 73% of patients. 1,3Symptom onset occurs at a mean age of 11 years, but disease manifestations may occur as late as age 50 years. 3Treatment of CTLA-4 haploinsufficiency includes management of autoimmune symptoms with standard therapies.Abatacept, a fusion protein comprising human CTLA-4 that is linked to a modified Fc portion of IgG1, acts as a selective Tcell costimulation modulator that can be useful in disease regulation. 1 Cytotoxic T-lymphocyte antigen-4 haploinsufficiency cutaneous findings are still poorly characterized, despite being present in 56% of patients. 3Atopic dermatitis (AD) is the most commonly described skin comorbidity, with recent reports of vitiligo, psoriasis, and alopecia. 3Cytotoxic T-lymphocyte antigen-4 variations have been associated with childhood AD and the severity of AD symptoms, and it can be the initial presenting symptom in 2% of patients. 4Similarly, CTLA-4 variations have been associated with AA in European populations and psoriasis in Eurasian populations. 5,6 This case highlights the occurrence of novel immunodeficiency syndromes and alerts dermatologists to the clinical constellation of chronic relapsing skin diseases, frequent respiratory infections, and autoimmune conditions.

Topics & Concepts

MedicineImmunologyAutoimmunityCytotoxic T cellCTLA-4T cellImmune systemIn vitroBiochemistryChemistryImmunodeficiency and Autoimmune DisordersAutoimmune and Inflammatory DisordersDermatological and COVID-19 studies