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 edit
- 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 edit
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Treatment edit
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References edit
- ^ 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.
- ^ Busquets-Pérez N, Narvaez J, Valverde-García J (2006). "Interstitial granulomatous dermatitis with arthritis (Ackerman syndrome)". J. Rheumatol. 33 (6): 1207–9. PMID 16755676.
- ^ 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.
- ^ 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.
- ^ "Symptoms of Ackerman syndrome - RightDiagnosis.com". www.rightdiagnosis.com. Retrieved 2016-04-14.