Acromegaly E22.0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Pachyakrie; (Pierre-)Marie syndrome; Pierre Marie syndrome

History
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Saucerotte, 1772; Marie, 1886

Definition
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Selective increase in size of the acra, especially of the nose, ears, chin, hands and feet after the end of the epiphyseal fugues, at the same time of the internal organs (visceromegaly) after the end of the growth age due to an overproduction of somatotropic hormone (STH).

Occurrence/Epidemiology
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Rarely. Incidence: 1/3-5 million inhabitants/year.

Etiopathogenesis
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Discussed are acromegaly and others:

  • Overproduction of growth hormone (STH = somatotropic hormone), usually due to an eosinophilic adenoma of the anterior pituitary gland.
  • Mutations of regulatory genes of adenylate cyclase ( G-proteins of the signal transduction pathway).
  • Ectopic neuroendocrine active tissue: GH-RF stimulates the oncogene c-fos in STH-producing cells in extrahypophyseal tumors (pancreas, lung, ovary, mamma).
  • Excessive secretion of growth hormone releasing factor (GH-RF): eutop in hypothalamic hamartoma, ganglioneuroma, ectop in tumors (carcinoid, pancreas, small cell carcinoma of lung, adrenal glands, pheochromocytoma).
  • Increased action or secretion of somatomedins ("acromegalodism").

Manifestation
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Predominantly in adulthood, especially 40th-50th LJ.

Clinical features
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  • Integument: Effluvium, hair trophic disorders (in female patients often mild hirsutism, in male patients alopecia of the androgenetic type); hyperhidrosis (especially palmar and plantar); hypertrichosis (in 40-50% of patients); onychodystrophy; cutis verticis gyrata; hyperpigmentation (in 40-50% of patients); acanthosis nigricans.
  • Extracutaneous manifestations of acromegaly: facial features coarsening, thickened and wrinkled facial skin, cutis verticis gyrata, enlargement of hands, feet, acromias, visceromegaly. Cute language due to a macroglossia. Frequently hypertension, visual disturbances, carpal tunnel syndrome, pathological glucose tolerance, hemianopsia, congestion papilla, headache, goiter (nodosa colloides), amenorrhea, dysmenorrhea, loss of potency (gonadal atrophy), adenomatous colonic polyps (higher prevalence of colon carcinoma), kyphosis of the thoracic spine. In pituitary tumour: bitemporal hemianopsia and congestive papilla.

Laboratory
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Growth hormone STH in the sense of increased, in the OGT no suppression below 2 ng/ml after administration of 100 g glucose. Increased somatomedin (IGF-I) levels, hyperprolactinaemia (in 20-40% of patients). Secondary diabetes mellitus (determine several FC values in the daily profile!). Somatomedin C reduced.

Diagnosis
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Clinic; Laboratory; X-ray and CT of the skull: Sella enlargement; X-ray of the paranasal sinuses, hands, feet (enlargement, cortical thickening). Comparison with previous photographs of the patient.

Therapy
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Treatment of the underlying disease, e.g. tumour removal surgically or by radiotherapy. Otherwise, inhibition of STH secretion with bromocriptine (e.g. Pravidel) or application of octreotide by the endocrinologist.

Literature
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  1. Braham C et al (2002) Skin tensile properties in patients treated for acromegaly. dermatology 204: 325-329
  2. Klibanski A, Zervas NT (1991) Diagnosis and management of hormone-secreting pituitary adenomas. N Engl J Med 324: 822-831
  3. Lami MC et al (2003) Hair loss in three patients with acromegaly treated with octreotide. Br J Dermatol 149: 655-656
  4. Marie P (1886) Sur deux cas d'acromégalie: hypertrophy singulière non congénitale des extrémités supérieures, inférieures et céphalique. Rev de Med 6: 297-333
  5. Melmed S (1992) Acromegaly. Endocrinology and Metabolism Clinics of North America 21: 483-765
  6. Melmed S et al (2002) Guidelines for acromegaly management. J Clin Endocrinol Metab 87: 4054-4058
  7. Saucerotte N (1801) Mélanges de surgery. Paris, Vol. 1, p. 477
  8. Umahara M et al (2003) Glucose-Dependent Insulinotropic Polypeptides Induced Growth Hormone Secretion in Acromegaly. Endocr J 50: 643-650
  9. Verstraeten (1889) L'acromégalie. Rev méd 9: 377-493
  10. Wass JA (2003) Dynamic testing in the diagnosis and follow-up of patients with acromegaly. J Endocrinol Invest 26: 48-53

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020