Pierre Marie disease

A rare chronic condition, usually caused by an adenoma. It is characterized by hyperfunction of the anterior pituitary gland associated with hypersecretion of growth hormone, resulting in enlargement of bones and soft tissue of hands, feet, and face.

Alternative eponyms
Marie’s disease
Pierre Marie disease
Marie’s syndrome II

Description

A rare chronic condition, usually caused by an adenoma. It is characterized by hyperfunction of the anterior pituitary gland associated with hypersecretion of growth hormone, resulting in enlargement of bones and soft tissue of hands, feet, and face. Affected persons show prominent cheek bones; frontal bossing; grossly overdeveloped mandible and broadening of the hands, fingers, and feet; large ears and nose, and thick lips. Associeted manifestations may be sexual disorders, diabetes mellitus, hemianopsia, and increased intracranial pressure. The term gigantism refers to the childhood form of this disease. Onset usually between 30 and 40 years of age. Hereditary factors are unusual.

Pierre Marie disease. Enlargement of hand bones.
Pierre Marie disease. Enlargement of hand bones.

Many descriptions preceded those of Marie (1886) who first correlated the clinical and pathological findings.

Probably first described in 1801 by Nicolas Saucerotte (1741-1814), described in 1883 by Friedrich Daniel von Recklinghausen (1833-1910), in 1884 by Chr. F. Fritsche and Theodor Albrecht Edwin Klebs (1834-1913); Pierre Marie in 1886.

 

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