74 Journal of The Association of Physicians of India ■ Vol. 63 ■ April 2015 Autoimmune Polyglandular Syndrome Type 2 with Alopecia Universalis and Hypoparathyroidism Priti Dave 1 , Deepak Bhosle 1 , Madhav Dharme 2 , Deepak Deshmukh 3 , Jay Patel 3 Abstract A 46 years old female, presented with severe fatigue, hypotension and hyperpigmentation. Her basal serum cortisol level at 8 a.m. was <1.0 µg/ dl which suggested a diagnosis of Addison’s disease. An association with latent autoimmune diabetes of adult and autoimmune hypothyroidism led to a diagnosis of Polyglandular Autoimmune Syndrome type II (PAS II). She also had alopecia universalis and hypoparathyroidism which are very rare in PAS type II syndrome. On treatment with hydrocortisone and fludrocortisone there was drastic improvement in the clinical features. 1 Professor, 2 Lecturer, 3 Postgraduate Resident, Department of Medicine, Bharati Vidhyapeeth Deemed University Medical College and Hospital, Pune Received: 20.05.2013; Revised: 26.06.2013; Re-revised: 12.08.2013; Accepted: 14.08.2013 Introduction W hen immune dysfunction affects two or more endocrine glands, in conjunction with other non-endocrine immune disorders the Polyglandular Autoimmune (PGA) syndrome should be considered. 1 There are two major subtypes of PGA syndrome. Type 1 is also called APECED (Autoimmune Polyendocrinopathy, Candidiasis and Ectodermal Dystrophy) or MEDAC PAS type II (Multiple Endocrine Deficiency Autoimmune Candidiasis Syndrome; Carpenter syndrome) is the coexistence of adrenal insufficiency with autoimmune thyroid disease, and/or type 1 diabetes mellitus (DM). PAS type II is more common than type 1. 2 The other types of Autoimmune Polyglandular Syndromes are 3 and 4. Type 3 is autoimmune thyroid disease associated with other autoimmune diseases (excluding Addison’s disease and or hypoparathyroidism while Type 4 is a combination of organ-specific diseases not included in the previous groups. 3 Case Report A 46 years old female, known to have diabetes for one year presented to hospital with complaints of severe fatigue and loss of energy for 1 month. She had also noticed darkening of skin of face and hair loss for 3 months, for which she took treatment from her general practitioner, without improvement. She was already on oral hypoglycemic drugs ((combination of glimepiride and metformin, 2 mg and 500 mg) twice a day for the past one year. She also complained of circumoral tingling, numbness and muscle cramps on and off. However there was no history of carpopedal spasm on detailed enquiry. She had two healthy male children, 16 and 13 years old. She had a normal menstrual history except for the last one year when she had scanty bleeding and prolonged menstrual cycles as she was approaching menopause. Her height was 160 cms, weight 61 kg and body mass index (BMI) was 23.82 kg/m 2 . On examination her pulse was 62/min, blood pressure was 80/60 mmHg. She had no anaemia or any obvious neck swelling. There was complete baldness as well as loss of hair in eyebrow (Figure 1), axilla and pubic region. On detailed examination there were bluish blackish patches on the tongue, (Figure 2), buccal mucous membrane, and there was bronze darkening of skin of face and knuckle (Figure 3). Lab investigations revealed hemoglobin of 12 gm/dl (normal 12.0-15.8 gm/dl), fasting blood glucose 234 mg/dl (normal <126 mg/dl), and post-prandial blood glucose of 317 mg/dl (normal < 200 mg/dl). Because of persistent hypotension the basal serum cortisol levels were sent. The basal serum cortisol level at 8 a.m. was <1.0 µg/dl (normal 5-25 µg/dl). One ampoule (250 mcg) cosyntropin was given intravenously and serum cortisol level was measured 45 minutes later. The cortisol level increased to 7 µg/dl (normal > 14 µg/dl). The test was positive and indicated primary adrenal insufficiency. Her ultrasensitive TSH was 7.25µIU/mL (0.34-4.25 Case RepoRts