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Ackerman syndrome


FieldValue
synonymsPyramidal molar-glaucoma-upper abnormal lip syndrome, interstitial granulomatous dermatitis with arthritis (IGDA)
nameAckerman syndrome
imageAutosomal recessive - en.svg
captionAckerman syndrome is inherited in an autosomal recessive manner

Ackerman syndrome or interstitial granulomatous dermatitis is a familial syndrome of fused molar roots with a single canal (taurodontism), hypotrichosis, full upper lip without a cupid's bow, thickened and wide philtrum, and occasional juvenile glaucoma. It was described by James L. Ackerman, A. Leon Ackerman, and A. Bernard Ackerman.

Signs and symptoms

  • Fused molar roots
  • Single root canal
  • Juvenile glaucoma
  • Sparse body hair
  • Distinct facial features: full upper lip, absence of cupid's bow, thick philtrum
  • Syndactyly
  • Increased pigmentation of finger joints
  • Clinodactyly of fifth finger.

References

References

  1. (1973). "Taurodont, pyramidal and fused molar roots associated with other anomalies in a kindred". Am. J. Phys. Anthropol..
  2. (2006). "Interstitial granulomatous dermatitis with arthritis (Ackerman syndrome)". J. Rheumatol..
  3. (2003). "Arthritis and interstitial granulomatous dermatitis (Ackerman syndrome) with pulmonary silicosis". Semin. Arthritis Rheum..
  4. (1973). "A New Dental, Ocular and Cutaneous Syndrome". International Journal of Dermatology.
  5. "Symptoms of Ackerman syndrome - RightDiagnosis.com".
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