VEXAS (vacuoles, E1-enzyme, X-linked, autoinflammatory, somatic) syndrome is a recently characterized autoinflammatory condition driven by myeloid cell dysregulation. We report two patients with concurrent diagnoses of VEXAS syndrome and autoimmune blistering diseases – pemphigus vulgaris and dermatitis herpetiformis – highlighting a previously undescribed association between innate immune dysregulation and autoantibody-mediated skin diseases.
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Bao et al. (2024) studied this question.
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