Type of DI with similar dental abnormalities usually an autosomal dominant trait with variable expressivity but can be recessive if the associated osteogenesis imperfecta is of recessive type.Recent genetic studies have identified that mutations in the genes coding for the collagen type 1 proteins, COL1A1 and COL1A2, are associated with this type of DI.Not all individuals with OI have dentinogenesis imperfecta, and the prevalence of DI varies depending on the sub-type of OI:

Higher prevalence of DI among individuals with OI type III and IV at 43-82% and 37-100%, respectively
Lower prevalence of DI among individuals with OI type I at 8-40%
No data available for other OI sub-types

Type II
DI not associated with OI. Occurs in people without other inherited disorders (i.e. Osteogenesis imperfecta). It is an autosomal dominant trait. A few families with type II have progressive hearing loss in addition to dental abnormalities. Also called hereditary opalescent dentin. Type III
Brandywine isolate. This type is rare with occurrences only in the secluded populations in Maryland, USA. Similar to DI type II, this type is also not associated with OI. Its predominant characteristic is bell-shaped crowns, especially in the permanent dentition. Unlike Types I and II, it involves teeth with shell-like appearance and multiple pulp exposures.Mutations in the gene coding for the dentine sialophosphoprotein (DSPP) are associated with DI type II and III. DSPP is a polypeptide which gives rise to 3 proteins; dentine sialoprotein (DSP), dentine glycoprotein (DGP), and dentine phosphoprotein (DPP).
Alopecia mucinosa (also known as "Follicular mucinosis," "Mucinosis follicularis", "Pinkus follicular mucinosis," and "Pinkus follicular mucinosis–benign primary form") is a skin disorder that generally presents, but not exclusively, as erythematous plaques or flat patches without hair primarily on the scalp, neck and face. : 649 : 188   This can also be present on the body as a follicular mucinosis and may represent a systemic disease.Alopecia mucinosa occurs when mucinous material accumulates in the hair follicles and sebaceous glands. This triggers an inflammatory response, and affects the follicles ability to produce hair. This hair loss is reversible in the early stages, but once the disease advances, the hair follicles are destroyed, and Scarring alopecia occurs. See also
List of cutaneous conditions

References


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