Addison's disease E27.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 18.12.2020

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Synonym(s)

Addison's disease; Addison`s melanoderma; Addison's syndrome; Addison's Syndrome; Addison`s syndromes; Bronze skin disease; Hypocortisolism; Hypocortisolism primary; Primary hypocortisolism

History
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Addison, 1849

Definition
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Primary adrenocortical insufficiency due to bilateral destruction or damage to the adrenal cortex (NNR).

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Occurrence/Epidemiology
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Incidences (of hypocortisolimus)

- imary NNRI: 0.5/100,000 population (synonymous with Addison's disease)

- condary NNRI: 2,0/100.000 inhabitants

-tertiary NNRI: about 1,0/100 (!)) inhabitants (most frequent form due to therapy with glucocorticoids)

Etiopathogenesis
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Autoimmune processes (destruction of NNR), so-called immune addison adrenalitis (autoimmune adrenalitis) -80% of cases-. Detection of 21-hydroxylase antibody, a central enzyme in steroid synthesis.

less often through:

  • systemic fungal infection (histoplasmosis, cryptococcosis, blastomycosis, coccidioidomycosis)
  • Traumas
  • metastatic processes (especially metastases from breast, bronchial, renal carcinoma.
  • leukemic infiltrations, especially non-Hodgkin lymphomas
  • Tuberculosis, CMV infections, HIV infections (adrenalitis)
  • Amyloidosis
  • Waterhouse Friderichsen syndrome.

The consequence is an increase in adrenocorticotropic hormone (ACTH) with partial conversion to melanocyte-stimulating hormone (alpha-MSH and beta-MSH).

Manifestation
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w>m;

Clinical features
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Integument: Generalized brown hyperpigmentation with emphasis on light exposed areas (initially indistinguishable from normal sun tan). Further pigmentation of armpits, nipple, genital area; pigmentation of hand lines, scars, pressure points; greyish-brownish mucous membrane pigmentation (due to increased ACTH and thus simultaneously MSH secretion), mostly porcelain white nails (negative to the surrounding brown skin, s.a. Leukonychia totalis), less frequently diffuse brown nail pigmentation, loss of secondary hair, tendency to hyperhidrosis. S.a.u. Addison, whiter.

General: Variable expression of the symptoms up to the unexpected Addison crisis with shock symptoms. Weakness and rapid fatigue, muscle weakness and pain, adynamia, enophthalmus. Weight loss and dehydration, cachexia, vomiting, diarrhoea, abdominal pain, loss of appetite, arterial hypotension, bradycardia, tendency to hypothermia, therapy-refractory shock. Also nausea, saltiness, tendency to spontaneous hypoglycemia.

Hyperpigmentation of Addison's disease is usually diagnostically pathological. Its absence does not exclude the diagnosis ("White Addison's disease, about 8% of cases in autoimmune primary NNR insufficiency). Addison's disease without hyperpigmentation is mainly found in blonde or red-blonde individuals who have little or no tanning.

Addison's disease can also occur in polyendocrinological syndromes, such as in combination with Hashimoto's thyroiditis and diabetes mellitus type 1 in autoimmune polyendocrinological syndrome.

Laboratory
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Blood changes: Anemia, leukopenia, relative lymphocytosis, eosinophilia, hypoglycemia, hyponatremia, hypochloremia, hyperkalemia, decreased excretion of corticoids and corticoid metabolites in urine, low concentration of cortisol and high concentration of ACTH.

Diagnosis
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Cortisol in plasma and 24-h-urine, no increase of NNR-hormones in the ACTH-stimulation test, NNR-autoantibodies, imaging diagnostics of the adrenal gland

Differential diagnosis
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Hemochromatosis, melanoerythroderma, argyria, scleroporphyria, arsenic melanosis, pituitary tumors producing ACTH and/or MSH or mediastinal carcinomas.

Complication(s)
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Life-threatening Addison's crisis during physical stress; in Waterhouse-Friderichsen syndrome.

Therapy
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Cooperation with endocrinologists. Avoid direct sunlight.

External therapy
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Textile as well as physical/chemical sun protection (e.g. Anthelios, Eucerin Sun, see also sunscreen).

Internal therapy
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Permanent substitution p.o. with glucocorticoids 20-37.5 mg/day ( hydrocortisone, glucocorticoids, systemic) (2/3 of the dose in the morning, 1/3 in the evening) and 0.05-0.2 mg/day fludrocortisone in the morning (e.g. Astonin H, fludrocortisone), dose adjustment during stress (surgery, infections, sports) to 2-5 times the dose.

Literature
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  1. Addison T (1849) Chronic Suprarenal Insufficiency, usually due to tuberculosis of suprarenal capsule. First announcement. London Medical Gazette N.S. 8: 517-518
  2. Addison T (1855) On the constitutional and local effects of disease of the suprarenal capsules. In: A collection of the published writings of the late Thomas Addison MD. London: New Sydenham Society, 1868
  3. Banba K et al (1999) Hyperpigmentation caused by hyperthyroidism: differences from the pigmentation of Addison's disease. Clin Exp Dermatol 24: 196-198
  4. Basketti R (2003) Chronic fatigue syndrome and Addison's disease. J Pediatr 142: 217
  5. Jabbour S (2003) Cutaneous manifestations of endocrine disorders: a guide for dermatologists. At J Clin Dermatol 4: 315-331
  6. Lovas K et al (2003) Sleep disturbances in patients with Addison's disease. Eur J Endocrinol 148: 449-456
  7. Ten S et al (2001) Clinical review 130: Addison's disease 2001 J Clin Endocrinol Metab 86: 2909-2922

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Last updated on: 18.12.2020