Alopecia universalis in a patient with Sheehan's syndrome.

Sir,

Sheehan's syndrome results from ischemic necrosis of the pituitary gland due to severe postpartum hemorrhage. This condition is still being seen frequently in developing countries such as India. Alopecia in Sheehan's syndrome usually consists of loss of axillary and pubic hair and results due to gonadotropin deficiency.[1]

A 55-year-old nondiabetic woman presented with fever of 5 days and altered sensorium of 2 days duration. She had no history of headache, vomiting, seizure, trauma, or radiation exposure. Since the last 10 years, she had been having gradually progressive fatigue and generalized hair loss. She gave a history of postpartum hemorrhage during her last delivery 16 years ago, which was followed by lactational failure and amenorrhea. She was also a known case of coronary artery disease with severe systolic dysfunction since 10 years. On examination, jugular venous pressure was raised; she had pedal edema, and generalized loss of hair including scalp, eyebrows, axillary, and pubic hair [Figure 1]. There was no evidence of any scarring or atrophy. Her ankle jerk reflex was delayed. and rest of the systemic examination was normal. She had normocytic normochromic anemia with hemoglobin of 105 g/L. Her renal and liver functions were mildly deranged. Her electrolyte, arterial blood gas analysis, and ultrasound abdomen were normal. Routine urine examination showed 30–40 pus cells/hpf. Her hormonal profile was suggestive of panhypopituitarism with low levels of serum cortisol, ACTH, fT3, fT4, TSH, GH, FSH, LH and prolactin [4.74 µg/dL (3.9–22.4), 10.12 pg/mL (0–46), 1.36 pg/dL (1.8–4.6), 0.631 ng/dL (0.93–1.71), 0.11 mIU/mL (0.27–4.2), 0.09 ng/mL (0.06–5.0), 0.497 mIU/mL (25.8–134.8), 0.10 mIU/mL (7.7–58.5), and 3.2 ng/mL (3.4–24.1), respectively]. Magnetic resonance imaging (MRI) of pituitary gland showed empty sella [Figure 2]. Pituitary antibodies could not be done due to financial constraints. In addition to continuing treatment of her coronary ailment and urinary tract infection, she was started on hormone replacement therapy. However, patient succumbed to sepsis and related complications.

Figure 1

Photograph of patient showing alopecia

Figure 2

Sagittal section of brain showing empty sella on magnetic resonance imaging

The pituitary gland undergoes major physiological, anatomical, and immunological changes during pregnancy, which predisposes to various disorders such as Sheehan's syndrome, lymphocytic hypophysitis, apoplexy of pituitary adenoma, and others. Sheehan's description of postpartum hypopituitarism promoted the belief that PPH leads to necrosis of the enlarged pituitary gland of pregnancy and hypopituitarism develops. The fact that only one third of women with severe postpartum bleeding develop hypopituitarism and Sheehan's syndrome has been reported to develop even after uncomplicated pregnancy and delivery suggest factors other than ischemia in the pathogenesis of Sheehan's syndrome.[12] Development of alopecia universalis six years after her last delivery reflects slow clinical progression and suggests factors other than ischemia in its pathogenesis. Tissue necrosis could release sequestered antigens, triggering autoimmunity of the pituitary, and delayed hypopituitarism in Sheehan's syndrome.[3] The presence of antipituitary antibodies (APAs) and antihypothalamus antibodies (AHAs) have been demonstrated in patients with SS, suggesting that autoimmunity may play a role in the pathogenesis of this syndrome.[34]

Alopecia is generally reported as loss of pubic hair and axillary hair in Sheehan's syndrome due to loss of gonadotrophic function. However, our patient had alopecia universalis. Alopecia universalis is characterized by loss of hair of entire scalp including eyebrows and body hairs. It is a severe form of alopecia areata, which is thought to be an autoimmune condition. It occurs in <1% of total cases of alopecia areata. Patients with alopecia universalis usually have a poorer prognosis; treatment failure is seen in most of these patients. Chance of spontaneous remission is low, and that of recurrence higher. Our patient had alopecia universalis since 10 years without any remission. Detection of hypopituitarism and Sheehan's syndrome was delayed as the patient mistakenly attributed her symptoms to her cardiac ailment.

Alopecia areata has been described in association with a number of autoimmune diseases including vitiligo, Hashimoto's thyroiditis, diabetes mellitus, systemic lupus erythematosus, and so on.[5] Alopecia areata has rarely been reported with hypopituitarism related to idiopathic primary hypophysitis and Sheehan's syndrome.[67] Alopecia universalis has been reported with dysfunction of hypothalamic–pituitary–adrenal axis in triple H syndrome in few cases and the mechanism involved is thought to be autoimmune.[8] The association of panhypopituitarism due to Sheehan's syndrome and alopecia universalis in our patient can be explained by autoimmunity, which could be an etiological factor for both. To the best of our knowledge, this is the first report of alopecia universalis associated with Sheehan's syndrome–related panhypopituitarism.

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