White Addison's disease: what is the possible cause?
A case of chronic primary adrenal insufficiency without hyperpigmentation in a 64-year-old woman is reported. Due to the absence of hyperpigmentation the diagnosis was delayed and she became critically ill. During endocrine evaluation, in order to investigate the mechanism responsible for the absence of hyperpigmentation, skin biopsy was done and hormones responsible for the skin pigmentation were measured. Absence of hyperpigmentation is explained by high degree of melanosome degradation in secondary lysosomes called "compound melanosomes", which overwhelmed increased stimulation of the skin pigmentation. Melanocyte-stimulating hormones were elevated with a strikingly high beta-LPH/ACTH ratio. To our knowledge, this is the first study of pathogenic mechanisms responsible for the absence of hyperpigmentation in white Addison's disease.
Kendereski
A
A
Institute of Endocrinology, Clinical Centre of Serbia, Military Medicine Academy, Belgrade, Yugoslavia.
Micić
D
D
Sumarac
M
M
Zorić
S
S
Macut
D
D
Colić
M
M
Skaro-Milić
A
A
Bogdanović
Z
Z
D000224:Addison Disease / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D000324:Adrenocorticotropic Hormone / Q000097:blood; D001706:Biopsy; D005260:Female; D006801:Humans; D008247:Lysosomes / Q000473:pathology; D008543:Melanins / Q000378:metabolism / Q000652:urine; D009074:Melanocyte-Stimulating Hormones / Q000097:blood; D020460:Melanosomes / Q000473:pathology; D008875:Middle Aged; D010858:Pigmentation; D012867:Skin / Q000473:pathology; D008083:beta-Lipotropin / Q000097:blood
D008543:Melanins; D000324:Adrenocorticotropic Hormone; D009074:Melanocyte-Stimulating Hormones; D008083:beta-Lipotropin