Curly hair-acral keratoderma-caries syndrome

Also known as: CHAC syndrome, CHACS

Definition

Orphanet

Curly hair-acral keratoderma-caries syndrome is an extremely rare ectodermal dysplasia syndrome characterized by premature loss of curly, brittle, dry hair, premature loss of teeth due to caries, nail dystrophy with thickening of the finger- and toe-nails, acral keratoderma and hypohidrosis. Additionally, sparse eyebrows and eyelashes, receding frontal hairline and flattened malar region are associated. The severity of features appears to increase with age.

Go To Source: Orphanet

Symptoms

Medical Term Other Names Description
Curly hair
Nail dystrophy Poor nail formation, Dystrophic nails, Onychodystrophy [more] Onychodystrophy (nail dystrophy) refers to nail changes apart from changes of the color (nail dyschromia) and involves partial or complete disruption of the various keratinous layers of the nail plate.
Dry hair Hair that lacks the lustre (shine or gleam) of normal hair.
Sparse eyebrow Hypotrichosis of eyebrow Decreased density/number of eyebrow hairs.
Carious teeth Tooth decay, Rotting teeth, Dental decay, Caries, Dental cavities, Frequent caries, Dental caries, Early dental caries, Cariosity of teeth, Tooth cavities [more] Caries is a multifactorial bacterial infection affecting the structure of the tooth. This term has been used to describe the presence of more than expected dental caries.
Premature loss of teeth Premature exfoliation of teeth, Early tooth loss, Loss of teeth, Premature tooth loss [more] Premature loss of teeth not related to trauma or neglect.
Ectodermal dysplasia Ectodermal dysplasia is a group of conditions in which there is abnormal development of the skin, hair, nails, teeth, or sweat glands.
Hypohidrosis Decreased ability to sweat, Oligohidrosis, Inadequate sweating, Sweating, decreased [more] Abnormally diminished capacity to sweat.

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