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Related Experiment Videos

Partially compensated hypoadrenalism presenting with persistent skin pigmentation.

E M Whitehead1, A B Atkinson, D R Hadden

  • 1Sir George E. Clark Metabolic Unit, Royal Victoria Hospital, Belfast, Northern Ireland.

Journal of Endocrinological Investigation
|March 1, 1989
PubMed
Summary
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This case study describes a patient with compensated hypoadrenalism, presenting with skin pigmentation and amenorrhea. Despite initial concerns, she maintained normal hormone levels over 20 years due to elevated ACTH and renin.

Area of Science:

  • Endocrinology
  • Immunology
  • Internal Medicine

Background:

  • A 33-year-old female presented with symptoms suggestive of Addison's disease, including hyperpigmentation and amenorrhea.
  • Initial endocrine assessment revealed normal basal cortisol but a lack of response to ACTH and vasopressin stimulation, leading to steroid replacement therapy.

Observation:

  • The patient discontinued treatment and was lost to follow-up for several years.
  • Reassessment after 20 years showed a healthy, pigmented individual with normal basal cortisol, circadian variation, and appropriate aldosterone response to posture.
  • Elevated plasma ACTH levels exhibited diurnal rhythm and incomplete suppression with dexamethasone, alongside high plasma renin levels.

Findings:

  • The patient demonstrated an autoimmune profile with positive adrenal, thyroid microsomal, and gastric parietal cell antibodies.

Related Experiment Videos

  • Despite the clinical presentation and immunological findings, there was no biochemical evidence of glucocorticoid or mineralocorticoid deficiency over the 20-year observation period.
  • Compensated hypoadrenalism was proposed, with high ACTH maintaining normal cortisol and high renin supporting aldosterone levels.

    • Implications:

    • This case highlights the possibility of compensated hypoadrenalism in patients with autoimmune markers and apparent adrenal insufficiency symptoms.
    • Sustained high ACTH levels were identified as the cause of the patient's characteristic skin hyperpigmentation.
    • The study underscores the importance of long-term monitoring in complex endocrine cases, even in the absence of overt deficiency symptoms.