Type 2 Autoimmune Polyglandular Syndrome Presenting With Hyperpigmentation and Amenorrhea

A 36-year-old female presented with lethargy, anorexia, nausea, hyperpigmentation, weight loss and amenorrhea for six months. On examination, she had hyperpigmentation of face, hands and oral mucosa. Investigations revealed adrenal insufficiency and subclinical hyperthyroidism with elevated anti-thyroid peroxidase antibodies. Adrenal insufficiency in combination with Grave's disease and/or type 1 diabetes mellitus occurs in type 2 autoimmune polyglandular syndrome. It is a polygenic disorder occurring due to mutations in the human leukocyte antigen complex on chromosome 6. The patient was treated with oral hydrocortisone which led to improvement in all the symptoms.

Keywords: adrenal insufficiency; amenorrhea; aps; autoimmune polyglandular syndrome; schmidt syndrome.