Table 1. Specification of our patients and the two subgroups of Vohwinkel syndrome
|
|
Mode of inheritance |
Gene locus |
PPK |
Pseudoainhum |
Onset |
Extensor starfish keratosis |
Erythematous border |
Extra cutaneous feature |
Histopathology |
|
Vohwinkel syndrome (classic form) |
AD |
13q11, |
Diffuse |
+ |
Infancy |
+ |
+ |
Deafness |
Hyperkeratosis, Parakeratosis, Acanthosis and hypergranulosis |
|
Vohwinkel syndrome (variant form) |
AD |
1q21 |
Diffuse |
+ |
Infancy |
+ |
+ |
Icthyosis |
Hyperkeratosis, Parakeratosis, Acanthosis and hypergranulosis |
|
Our patients |
AR |
? |
Focal(striata) (transgredient) |
+ |
Infancy |
_ |
+ |
Hypotrichosis |
Hyperkeratosis, Parakeratosis, Acanthosis and hypergranulosis |