Ackerman syndrome

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.^[1][2][3] It was described by James L. Ackerman, A. Leon Ackerman, and A. Bernard Ackerman.^[4]

Ackerman syndrome
Other names	Pyramidal molar-glaucoma-upper abnormal lip syndrome, interstitial granulomatous dermatitis with arthritis (IGDA)
Ackerman syndrome is inherited in an autosomal recessive manner

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.^[5]

Diagnosis

Treatment

References

1. Ackerman JL, Ackerman AL, Ackerman AB (1973). "Taurodont, pyramidal and fused molar roots associated with other anomalies in a kindred". Am. J. Phys. Anthropol. 38 (3): 681–94. doi:10.1002/ajpa.1330380305. PMID 4349385.
2. Busquets-Pérez N, Narvaez J, Valverde-García J (2006). "Interstitial granulomatous dermatitis with arthritis (Ackerman syndrome)". J. Rheumatol. 33 (6): 1207–9. PMID 16755676.
3. Kroesen S, Itin PH, Hasler P (2003). "Arthritis and interstitial granulomatous dermatitis (Ackerman syndrome) with pulmonary silicosis". Semin. Arthritis Rheum. 32 (5): 334–40. doi:10.1053/sarh.2003.50016. PMID 12701044.
4. Ackerman JL, Ackerman AL, Ackerman AB (1973). "A New Dental, Ocular and Cutaneous Syndrome". International Journal of Dermatology. 12 (5): 285–89. doi:10.1111/j.1365-4362.1973.tb00056.x. PMID 4355828. S2CID 27774823.
5. "Symptoms of Ackerman syndrome - RightDiagnosis.com". www.rightdiagnosis.com. Retrieved 2016-04-14.

External links

• synd/133 at Who Named It?