eMedicine Specialties > Dermatology > Internal Medicine

Addison Disease: Treatment & Medication

Author: Elizabeth A Liotta, MD, Assistant Professor, Department of Dermatology, Uniformed Services University of the Health Sciences
Coauthor(s): Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center; Alexander Brough, MD, Consulting Surgeon, Department of Dermatology, Sewell's Point Clinic
Contributor Information and Disclosures

Updated: Jan 21, 2009

Treatment

Medical Care

  • Promptly treat patients in whom acute adrenal insufficiency is suspected; follow up with a workup for adrenal insufficiency.
  • In Addison disease, the adequate replacement of glucocorticoids and mineralocorticoids is the primary goal.
  • Studies show that dehydroepiandrosterone therapy improves the patient's quality of life.10,11

Consultations

The need for consultation depends on the cause of the adrenal insufficiency and may involve the following specialists:

  • Endocrinologist
  • Rheumatologist
  • Infectious diseases specialist

Diet

  • Advise patients not to restrict salt in their diets.
  • In patients with concurrent primary hypertension, salt intake may be restricted instead of discontinuing mineralocorticoid replacement.
  • Advise patients who live in warm climates to increase their salt intake because of their increased loss of salt as a result of sweating.

Activity

  • No restrictions on activity are required.
  • Inform patients about salt loss during vigorous exercise.

Medication

With optimum dosing (which is often a challenge), the glucocorticoids are adequately replaced with minimal adverse effects. Underdosing of glucocorticoids results in continued adrenal insufficiency. In children, nocturnal hypoglycemia can result in seizures. Overdosing of glucocorticoids results in weight gain, increased blood pressure, and osteoporosis. The effects of steroid replacement are assessed with clinical examination.

The resolution of symptoms and the correction of electrolyte abnormalities are the customary signals in determining the adequacy of replacement. In patients at risk for osteoporosis, monitor serum and urine cortisol levels; this method appears to be the best available assessment of steroid dosing.

The titration of mineralocorticoid replacement is achieved by monitoring electrolyte levels and plasma rennin concentrations and by evaluating clinical findings such as dizziness or weight gain. Weakness, decreased diastolic blood pressure, low serum sodium levels, and increased plasma rennin concentrations occur with an underdosing of fludrocortisone. Overdosing is difficult to determine. Decreased serum potassium levels may be seen. Increased levels of atrial natriuretic peptide have been proposed to be more accurate in determining an overdose.12

Corticosteroids

These agents are used to restore corticosteroid levels. Some cases of multifocal disseminated lipoatrophy as a result of IV corticosteroid administration have been reported in these patients with adrenal insufficiency.13


Cortisone (Cortone)

DOC for patients with adrenocortical insufficiency.

Adult

25-300 mg/d PO/IM divided q12-24h; taper to physiologic replacement
Alternatively, 12-15 mg/m2/d PO, divided two-thirds am and one-third pm

Pediatric

0.5-0.75 mg/kg/d PO/IM or 20-25 mg/m2/d divided q8h
Alternative IM administration: 0.25-0.35 mg/kg/d IM qd or 12.5 mg/m2/d IM

Estrogen coadministration may increase corticosteroid levels; may increase digitalis toxicity secondary to hypokalemia; concomitant administration with bisphosphonates associated with increased incidence of osteonecrosis; concomitant administration with fluoroquinolones may increase risk of tendon rupture, especially in elderly persons

Documented hypersensitivity; viral, fungal, or tubercular skin lesions; infections (except during replacement therapy)

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Positive evidence of human fetal risk exists, but benefits from use in pregnant women may be acceptable despite risk (eg, if drug needed in life-threatening situation or for serious disease for which safer drugs cannot be used or are ineffective); caution in hyperthyroidism, cirrhosis, nonspecific ulcerative colitis, osteoporosis, peptic ulcer disease, diabetes, and myasthenia gravis


Hydrocortisone (Hydrocortone, Cortef)

DOC because of mineralocorticoid activity and glucocorticoid effects. Some cases of multifocal disseminated lipoatrophy as a result of IV corticosteroid administration have been reported in these patients with adrenal insufficiency.

Adult

100 mg IV bolus followed by continuous infusion of 100 mg q8h for 24-48 h
Once condition stable, initiate 50 mg q8h PO for 48 h; may taper to 30-50 mg/d in divided doses
Chronic dosing should approximate physiologic replacement.

Pediatric

<12 years: 1-2 mg/kg IV bolus followed by 25-150 mg/d divided q6-8h
>12 years: 1-2 mg/kg IV bolus followed by 150-250 mg/d divided q6-8h

Corticosteroid clearance may decrease with estrogens; may increase digitalis toxicity secondary to hypokalemia; concomitant administration with bisphosphonates associated with increased incidence of osteonecrosis; concomitant administration with fluoroquinolones may increase risk of tendon rupture, especially in elderly persons

Documented hypersensitivity; viral, fungal, or tubercular skin infections

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Positive evidence of human fetal risk exists, but benefits from use in pregnant women may be acceptable despite risk (eg, if drug needed in life-threatening situation or for serious disease for which safer drugs cannot be used or are ineffective); caution in hyperthyroidism, osteoporosis, peptic ulcer disease, cirrhosis, nonspecific ulcerative colitis, diabetes, and myasthenia gravis


Fludrocortisone (Florinef)

Used for partial replacement therapy in primary and secondary adrenocortical insufficiency.

Adult

0.1-0.2 mg PO 3 times/wk

Pediatric

Infants: 0.1 to 0.2 mg/d PO
Children: 0.05 to 0.1 mg/d PO

Antagonizes effects of anticholinergics; rifampin, hydantoins, and barbiturates decrease effects; decreases salicylate levels; concomitant administration with bisphosphonates associated with increased incidence of osteonecrosis; concomitant administration with fluoroquinolones may increase risk of tendon rupture, especially in elderly persons

Documented hypersensitivity; systemic fungal infections

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Positive evidence of human fetal risk exists, but benefits from use in pregnant women may be acceptable despite risk (eg, if drug needed in life-threatening situation or for serious disease for which safer drugs cannot be used or are ineffective); caution in Addison disease, potassium loss, and sodium retention


Dexamethasone (Decadron, Baldex, Dexone)

DOC for patients with adrenocortical insufficiency.

Adult

0.25-0.75 mg PO qhs

Pediatric

Not established

Effects decrease with coadministration of barbiturates, phenytoin, and rifampin; decreases effect of salicylates and vaccines used for immunization; concomitant administration with bisphosphonates associated with increased incidence of osteonecrosis; concomitant administration with fluoroquinolones may increase risk of tendon rupture, especially in elderly persons

Documented hypersensitivity; active bacterial or fungal infection

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Increases risk of multiple complications (eg, severe infections); monitor adrenal insufficiency when tapering; abrupt discontinuation may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections are possible complications

More on Addison Disease

Overview: Addison Disease
Differential Diagnoses & Workup: Addison Disease
Treatment & Medication: Addison Disease
Follow-up: Addison Disease
Multimedia: Addison Disease
References

References

  1. Bergthorsdottir R, Leonsson-Zachrisson M, Oden A, Johannsson G. Premature mortality in patients with Addison's disease: a population-based study. J Clin Endocrinol Metab. Dec 2006;91(12):4849-53. [Medline].

  2. Burk CJ, Ciocca G, Heath CR, Duarte A, Dohil M, Connelly EA. Addison's disease, diffuse skin, and mucosal hyperpigmenation with subtle "flu-like" symptoms--a report of two cases. Pediatr Dermatol. Mar-Apr 2008;25(2):215-8. [Medline].

  3. Lamey PJ, Carmichael F, Scully C. Oral pigmentation, Addison's disease and the results of screening for adrenocortical insufficiency. Br Dent J. Apr 20 1985;158(8):297-8. [Medline].

  4. Shah SS, Oh CH, Coffin SE, Yan AC. Addisonian pigmentation of the oral mucosa. Cutis. Aug 2005;76(2):97-9. [Medline].

  5. Prat C, Vinas M, Marcoval J, Jucgla A. Longitudinal melanonychia as the first sign of Addison's disease. J Am Acad Dermatol. Mar 2008;58(3):522-4. [Medline].

  6. Adams R, Hinkebein MK, McQuillen M, Sutherland S, El Asyouty S, Lippmann S. Prompt differentiation of Addison's disease from anorexia nervosa during weight loss and vomiting. South Med J. Feb 1998;91(2):208-11. [Medline].

  7. Albert E, Dalaker K, Jorde R, Berge LN. Addison's disease and pregnancy. Acta Obstet Gynecol Scand. 1989;68(2):185-7. [Medline].

  8. Kita M, Mandala E, Saratzis A, et al. Primary adrenal lymphoma presenting as Addison's disease. Case report and review of the literature. Exp Clin Endocrinol Diabetes. Jun 2008;116(6):363-5. [Medline].

  9. Baker DE, Glazer GM, Francis IR. Adrenal magnetic resonance imaging in Addison's disease. Urol Radiol. 1988;9(4):199-203. [Medline].

  10. Arlt W, Callies F, van Vlijmen JC, Koehler I, Reincke M, Bidlingmaier M, et al. Dehydroepiandrosterone replacement in women with adrenal insufficiency. N Engl J Med. Sep 30 1999;341(14):1013-20. [Medline].

  11. Oelkers W. Dehydroepiandrosterone for adrenal insufficiency. N Engl J Med. Sep 30 1999;341(14):1073-4. [Medline].

  12. Cohen N, Gilbert R, Wirth A, Casley D, Jerums G. Atrial natriuretic peptide and plasma renin levels in assessment of mineralocorticoid replacement in Addison's disease. J Clin Endocrinol Metab. Apr 1996;81(4):1411-5. [Medline].

  13. Bauerschmitz J, Bork K. Multifocal disseminated lipoatrophy secondary to intravenous corticosteroid administration in a patient with adrenal insufficiency. J Am Acad Dermatol. May 2002;46(5 Suppl):S130-2. [Medline].

  14. Ai J, Leonhardt JM, Heymann WR. Autoimmune thyroid diseases: etiology, pathogenesis, and dermatologic manifestations. J Am Acad Dermatol. May 2003;48(5):641-59; quiz 660-2. [Medline].

  15. Betterle C, Volpato M. Adrenal and ovarian autoimmunity. Eur J Endocrinol. Jan 1998;138(1):16-25. [Medline].

  16. Burke MP, Opeskin K. Adrenocortical insufficiency. Am J Forensic Med Pathol. Mar 1999;20(1):60-5. [Medline].

  17. Dunlop D. Eighty-six cases of Addison's Disease. Br Med J. Oct 12 1963;5362:887-91. [Medline].

  18. Grant DB, Barnes ND, Moncrieff MW, Savage MO. Clinical presentation, growth, and pubertal development in Addison's disease. Arch Dis Child. Oct 1985;60(10):925-8. [Medline].

  19. Grinspoon SK, Biller BM. Clinical review 62: Laboratory assessment of adrenal insufficiency. J Clin Endocrinol Metab. Oct 1994;79(4):923-31. [Medline].

  20. Hunt G, Todd C, Kyne S, Thody AJ. ACTH stimulates melanogenesis in cultured human melanocytes. J Endocrinol. Jan 1994;140(1):R1-3. [Medline].

  21. Jabbour SA. Cutaneous manifestations of endocrine disorders: a guide for dermatologists. Am J Clin Dermatol. 2003;4(5):315-31. [Medline].

  22. Jeffcoate W. Assessment of corticosteroid replacement therapy in adults with adrenal insufficiency. Ann Clin Biochem. Mar 1999;36 ( Pt 2):151-7. [Medline].

  23. Kong MF, Jeffcoate W. Eighty-six cases of Addison's disease. Clin Endocrinol (Oxf). Dec 1994;41(6):757-61. [Medline].

  24. Laureti S, Vecchi L, Santeusanio F, Falorni A. Is the prevalence of Addison's disease underestimated?. J Clin Endocrinol Metab. May 1999;84(5):1762. [Medline].

  25. Luken KK. Clinical manifestations and management of Addison's disease. J Am Acad Nurse Pract. Apr 1999;11(4):151-4. [Medline].

  26. Mason AS, Meade TW, Lee JA, Morris JN. Epidemiological and clinical picture of Addison's disease. Lancet. Oct 5 1968;2(7571):744-7. [Medline].

  27. Nerup J. Addison's disease--clinical studies. A report fo 108 cases. Acta Endocrinol (Copenh). May 1974;76(1):127-41. [Medline].

  28. Nieman LK, Chanco Turner ML. Addison's disease. Clin Dermatol. Jul-Aug 2006;24(4):276-80. [Medline].

  29. Orth DN, Kovacs WJ. The adrenal cortex. In: DeGroot LJ, Jameson JL, eds. William's Textbook of Endocrinology. 9th ed. Philadelphia, Pa: WB Saunders; 1998.

  30. Tobin MV, Aldridge SA, Morris AI, Belchetz PE, Gilmore IT. Gastrointestinal manifestations of Addison's disease. Am J Gastroenterol. Oct 1989;84(10):1302-5. [Medline].

  31. Villabona CM, Sahun M, Ricart W, Serres X, Maroto A, Fernandez-Real JM, et al. Tuberculous Addison's disease. Utility of CT in diagnosis and follow-up. Eur J Radiol. Nov 1993;17(3):210-3. [Medline].

Further Reading

Keywords

Addison disease, Addison's disease, primary adrenal insufficiency, chronic adrenal insufficiency, hypoadrenalism, polyglandular autoimmune diseases, polyglandular autoimmune disease I, PGAD I, polyglandular autoimmune disease II, PGAD II, Schmidt syndrome, addisonian crisis, adrenocorticotropic hormone, ACTH, corticotropin, melanocyte-stimulating hormone, MSH

Contributor Information and Disclosures

Author

Elizabeth A Liotta, MD, Assistant Professor, Department of Dermatology, Uniformed Services University of the Health Sciences
Elizabeth A Liotta, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Coauthor(s)

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Alexander Brough, MD, Consulting Surgeon, Department of Dermatology, Sewell's Point Clinic
Alexander Brough, MD is a member of the following medical societies: American Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Robin Travers, MD, Assistant Professor of Medicine (Dermatology), Dartmouth University School of Medicine; Staff Dermatologist, New England Baptist Hospital; Private Practice, SkinCare Physicians
Robin Travers, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Informatics Association, Massachusetts Medical Society, Medical Dermatology Society, and Women's Dermatologic Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey P Callen, MD, Professor of Medicine, Chief, Division of Dermatology, University of Louisville School of Medicine
Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology
Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Honoraria Consulting; Centocor Honoraria Consulting; Genetech Honoraria Consulting; Celgene Honoraria Consulting

CME Editor

Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital
Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

RELATED MEDSCAPE ARTICLES
Articles
 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.