| • | (anhidrotic) skin, hair, and teeth vs. (hydrotic)skin, hair, and nails |
Anhidrotic ectodermal dysplasia
| • | X-linked recessive (therefore almost all men) |
| • | triad: sparse hair, anodontia (partial or total; peg teeth), anhidrosis (or hypohidrosis) |
| • | diagnosis: biopsy of palmar skin confirms that eccrine glands are absent or rudimentary |
| • | if unsure, EM of hair shaft shows longitudinal groove |
| • | characteristic facies: (frontal bossing, thick everted lips, lower half of face is narrow, saddle back nose) |
| • | hair is scraggly, thin, sparse, and dry |
| • | increased frequency of atopic dermatitis |
| • | mnemonic: don’t let the anhidrotics go to the SPA (SPA = Sparse hair, Peg teeth, Anhidrosis) or they will overheat (but they can get a facial for their abnormal facies) |
Hidrotic ectodermal dysplasia
| • | normal facies, normal sweating |
| • | triad: nail dystrophy anonychia and micronychia, sparse hair, palmoplantar keratoderma |
|