Acromegaly
Syn.: Somatotropinoma.
Def .: The consequence of excessive production of growth hormone (GH) in adults, most often caused by the presence of an eosinophilic pituitary adenoma (Hyperthyroidism). The children’s counterpart is gigantism.
Epid.: The disease occurs in 50-70 people out of a million and is diagnosed twice as often in women. men. Annually, it affects less than 4 people per million inhabitants.
Clin.: Skin symptoms result from the involvement of the epidermis and appendages (hyperplasia), as well as the dermis (increased amount of glycoaminoglycans and water retention).
Belong to them:
-thickness and pasty texture of the skin of the face, hands and feet;
– thickening of skin folds in the area of the face and neck; the face takes on a gloomy look (basset skin, basset hound look);
– thickening and swelling of the eyelids, enlargement and forward of the lower lip;
– deep furrows within the scalp
– skóra kręta ciemienia (rotated skin vertices);
– enlargement of the tongue (macroglossia) with numerous fissures;
– wide, “fleshy” nose;
– thickened skin covering the heels;
–Increased number of soft fibromas (fibroma molle);
– foci of dark keratosis (acanthosis nigricans);
– thickened and hard nail plates, rough, in the course of the disease soft and thinning hair, excessive activity of the sebaceous and sweat (apocrine glands – hence the unpleasant smell) – these symptoms may partly result from disturbances in the secretion of other pituitary gland hormones.
Symptoms related to the skeletal and cartilage system:
-Longation of the limbs, enhancement of bone elements in the frontal area;
– enlargement of hands and feet;
– teeth spacing at large intervals.
Symptoms resulting from compression of the tumor:
– visual disturbance (visual field limitation);
-headaches.
Secondary symptoms: hypothyroidism, hypogonadism, diabetes.
DL: Hormonal diagnostics – GH concentration and insulin-like growth factor-1 (IGF-1), imaging tests (MRI, CT), ophthalmological examination, exclusion of pituitary insufficiency, primary hyperthyroidism.
DR: Primary hyperparathyroidism, pancreatic islets in MEN 1 syndrome.
Treatment: Surgical removal of the tumor, drug treatment – somatostatin analogues with prolonged action, dopamine antagonists (bromocriptine), radiation therapy.
Year.: The life expectancy in patients with untreated acromegaly is shorter by about 10 years, and the mortality due to cardiovascular diseases, respiratory system and neoplastic diseases is 2-4 times higher than in the general population.
Lit .: [1] Barkan A.L.: New options for diagnosing and treating acromegaly. Cleve. Clin J Med 1998, 65; 347-9. [2] Coskeran P.: Management and treatment of patients with acromegaly. Nurs Times 1999, 31; 50-2. [3] Danzig J.: Acromegaly. BMJ 2007, 20; 824-5. [4] Daughaday W.H., Cryer P.E.: Growth hormone hypersecretion and acromegaly. Hosp Pract 1978, 13(8); 75-80. [5] von Werder K.: Acromegaly. Fortschr Med 1980, 28; 1202-5.
Source: A. Kaszuba, Z. Adamski: “Lexicon of dermatology”; XNUMXst edition, Czelej Publishing House