ea0013p276 | Steroids | SFEBES2007
, Banerjee Ritwik
ACTH insensitivity syndromes comprise a rare, and underrecognised, group of disorders, which include familial glucocorticoid deficiency (FGD) and triple A or Allgrove syndrome (ACTH insensitivity, alacrima, achalasia and neurological involvement). They are characterised by undetectable cortisol and very high circulating ACTH with resultant hyperpigmentation and are inherited in an autosomal recessive manner. Distinction between FGD and triple A syndrome may be difficult and is...