Late onset adrenal insufficiency and achalasia in Allgrove syndrome

Abstract

A 26-year-old man presented with progressive difficulty in swallowing for 2 years and increased skin pigmentation for 1 year. This was associated with a loss of weight despite a preserved appetite. He had a lack of tears since childhood. His parents had a second-degree consanguineous marriage and had four sons. Examination was normal except for increased pigmentation over his lips (figure 1) and around his joints (figure 2). His pulse rate was normal; he had asymptomatic postural hypotension (supine 90/70 mm Hg, standing 56/46 mm Hg). Routine blood results including thyroid function were also normal. Figure 1 Generalised hyperpigmentation of face with marked melanin pigmentation over both lips. Figure 2 Generalised hyperpigmentation over proximal posterior aspect of forearm with marked hyperpigmentation over olecranon. A barium study showed dilation of the oesophagus with abrupt narrowing …