Jaffe-Lichtenstein syndrome

Alternative eponyms

Related people

A monostotic form of fibrous dysplasia of bone.

Description

A monostotic form of fibrous dysplasia of bone. Constitutional, benign disease of the bones characterised by excessive proliferation of single or multiple circumscribed fibrous lesions, predominantly in the long bones, which predispose to painful bone deformities and pathological fractures, limping, unequal limp length, skull asymmetry, leontiaslike appearance, scoliosis, lordosis, and chest deformity. Irregular macular dermal pigmentation is a variable feature. Onset usually during first two decades of life. Aetiology unknown. No fever; characteristic X-ray. The disorder is usually sporadic, but familial cases have been reported.

It was described in 1942 by Lichtenstein and Jaffe who reported 15 cases. Schlumberger subsequently reported 67 additional cases. The syndrome occurs chiefly in children and is not accompanied by systemic, metabolic, endocrine, or other skeletal changes. A similar condition with patchy skin pigmentation and sexual precocity is known as McCune-Albright syndrome.

Bibliography

  • H. L. Jaffe:
    «Osteoid-osteoma,», benign osteoblastic tumor composed of osteoid atypical bone.
    Archives of Surgery, Chicago, 1935, 31: 709-728.
  • F. Albright, A. M. Butler, A. O. Hampton, P. Smith:
    Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation and endocrine dysfunction, with precocious puberty in females: report of five cases.
    New England Journal of Medicine, Boston, 1937, 216: 727-746.
  • L. Lichtenstein:
    Polystotic fibrous dysplasia.
    Archives of Surgery, Chicago, 1938, 36: 874.
  • H. L. Jaffe, L. Lichtenstein:
    Non-osteogenic fibroma of bone.
    American Journal of Pathology, Philadelphia, 1942, 18: 205.
  • H. L. Jaffe, L. Lichtenstein:
    Fibrous dysplasia of bone. A condition affecting one, several or many bones, the graver cases of which may present with abnormal pigmentation of skin, premature sexual development, hyperthyroidism or still other extraskeletal abnormalities.
    Archives of Pathology, Chicago, 1942, 33: 777-816.
    This review was based upon clinical, radiological and histological studies of 23 cases.
  • D. J. McCune, H. Bruch:
    Osteodystrophia fibrosa: report of a case in which the condition was combined with precocious puberty, pathologic pigmentation of the skin and hyperthyroidism, with a review of the literature.
    American Journal of Diseases of Children, Chicago, 1937, 54: 806-848.

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