Autoimmune spontaneous chronic urticaria and generalized myasthenia gravis in a patient with polyglandular autoimmune syndrome type 3

Autoimmune polyglandular syndromes (APS) are a group of disorders characterized by the presence of autoimmune processes in several endocrine and non-endocrine organs that are classified into 4 types.

We describe the case of a woman affected with APS type 3 who presented initially with Hashimoto thyroiditis and pernicious anemia and subsequently developed autoimmune chronic urticaria, myasthenia gravis, and type 1 diabetes mellitus.

This patient had a combination of components of APS types 3b, 3a, and 3c. She was referred for evaluation of chronic spontaneous urticaria and subsequently developed severe generalized myasthenia gravis, which was apparently unmasked by antihistamines used to control urticaria.

Patients with APS should have a more thorough evaluation to better clarify their autoimmune background. Early detection of autoantibodies and latent organ-specific dysfunction may help physicians take appropriate action to prevent full-blown disease.