Precocious sexual development In a boy with Addison's disease

Abstract

After a short episode of fever and dehydration an 11 year old boy was diagnosed to have idiopathic Addison's disease with an ACTH level of over 4500 pg/ml. Since the age of 6 years he had manifested hyperpigmentation of the skin and buccal mucosa. At admission he showed advanced pubertal signs (P4 - Tanner) for genitalia and pubic hair, and his bone age was 13 years. The plasma levels of testosterone were 63ng/dl and the LH and FSH response to LRH stimulation (50 μg/m2, i.v.) - 2.2 to 25.0 and 2.6 to 3.5 mIU/ml, respectively - confirmed the diagnosis of central precocious puberty. Basal plasma DHEA (51 ng/dl) and androstenedione (17 ng/dl) levels were very low for age (normal 180 and 40 ng/dl, respectively). It is proposed that the association of precocious puberty in this boy with latent untreated Addison's disease is due to a “drift phenomenon” of LRH and/or gonadotrophins, following the prolonged elevation of ACTH secretion. It is also possible that earlier activation of adrenal androgens by the ACTH contributed to the induction of the precocious pubertal process.