Familial Addison's disease

Familial prepubertal Addison's disease is presented in two sets of brothers. Predominance of males with normal sex differentiation, lack of defects in steroid biosynthesis and absence of antibodies and associated endocrinopathies, separate this entity from other forms due to enzyme deficiencies or disordered cellular immune response. Distributed as a Mendelian recessive characteristic, familial Addison's disease of childhood may represent a milder form of the same genetic disorder responsible for congenital adrenal hypoplasia of infancy.