Generalized hyperpigmentation of skin: A case of Carpenter s
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A 45-year-old woman presented with complained of darkening of skin color over the last 6 months. Hyperpigmentation started initially over the face and later progressed to involve palmar creases, knuckles and oral mucosa. Patient was a known case of hypothyroidism on treatment. Further history revealed dizziness on standing position, easy fatigability, polyuria, constipation, cold intolerance, irregular menstrual cycles occurring once in 6 months to 1 year.

On general examination, patient was diagnosed with thyromegaly. Vitals were stable except for postural hypotension. Dermatological examination showed generalized hyperpigmentation of skin with accentuation over the dorsae of hands, knuckles and palmar creases. It was associated with hyperpigmentation of lips, buccal mucosa and tongue. Blood reports and imaging studies confirmed the diagnosis of primary adrenal insufficiency. Also autoimmune polyglandular involvement was suspected.

On further evaluation, her serum thyroid-stimulating hormone, follicle-stimulating hormone and luteinizing hormones were elevated and estradiol was low. Fine-needle aspiration cytology of thyroid showed Hashimoto's thyroiditis. Among the antibodies screened anti-thyroid peroxidase and anti-glutamic acid decarboxylase were positive. With the absence of mucocutaneous candidiasis and primary involvement of thyroid, adrenal, ovary and pancreas, the diagnosis of Carpenter syndrome was confirmed.

Patient was treated with hydrocortisone 100mg TDS and fludrocortisone 100 g OD, and levothyroxine 50 g and was discharged with an advice for a regular follow-up of blood sugars, educated about symptoms of diabetic ketoacidosis and the need of insulin requirement in the future. The need for increased dosage of steroid requirement at the time of stress, infections and surgery was emphasized.

After three months, her skin and mucosal hyperpigmentation had improved. She developed type-I diabetes mellitus and was started on injectable insulin. Yearly follow-up with blood investigations is recommended. Family members are also at a risk of developing autoimmune polyglandular syndrome, therefore should be screened and followed up.