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Addison disease - Skin in Adult
See also in: Oral Mucosal Lesion
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Addison disease - Skin in Adult

See also in: Oral Mucosal Lesion
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Contributors: Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH, Michael D. Tharp MD
Other Resources UpToDate PubMed

Synopsis

Addison disease is adrenal insufficiency resulting from dysfunction of the adrenal glands. This can be due to a variety of causes, which include autoimmune diseases, infections (eg, tuberculosis), and neoplasms. Patients with acquired immunodeficiency syndrome may develop Addison disease due to cytomegalovirus infection. Other less common causes are drugs (eg, antifungals), congenital adrenal hypoplasia, and disorders such as sarcoidosis and amyloidosis. Patients with loss-of-function mutations in sphingosine-1-phosphate lyase may have nephrosis with ichthyosis and adrenal insufficiency.

The adrenal insufficiency leads to decreased levels of glucocorticoids, mineralocorticoids, and adrenal androgens. The disease is characterized by general symptoms of fatigue, weight loss, weakness, vomiting, dizziness, cold intolerance, and muscle aches. Because these symptoms are nonspecific, the diagnosis may go unrecognized until the patient presents with a life-threatening adrenal crisis.

The cutaneous manifestation of patients with chronic Addison disease is hyperpigmentation of skin and mucous membranes. This is generalized and mainly affects sun-exposed areas. These and the other clinical signs of the disease appear only after 90% of the adrenal gland is nonfunctioning. The hyperpigmentation is due to increased production of melanocyte-stimulating hormone (MSH) and adrenocorticotrophic hormone (ACTH) following decreased cortisol production. These hormones stimulate melanogenesis.

Men and women are equally affected, and although all ages are affected, it is more common between the ages of 30 and 50. Estimated prevalence of the disease is at 120 per million population.

Addisonian Crisis:
Acute adrenal failure presents with severe penetrating abdominal pain accompanied by vomiting and diarrhea, low blood pressure, and loss of consciousness. This is a life-threatening emergency that must be managed aggressively.

Codes

ICD10CM:
E27.1 – Primary adrenocortical insufficiency

SNOMEDCT:
363732003 – Addison disease

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Diseases that produce pigmentation of the skin have to be differentiated from Addison disease.

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Updated: 03/07/2017
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Addison disease - Skin in Adult
See also in: Oral Mucosal Lesion
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Addison disease (Common Features) : Abdominal pain, Fatigue, Hyperkalemia, Muscle weakness, Weight loss, Anorexia, Darkening skin lesion, BP decreased, Hyperpigmentation, Na decreased, Depression
Clinical image of Addison disease
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