Comparison of the IP and DKC phenotypes affecting ectodermal tissues and the haemopoietic system
Incontinentia pigmenti (IP) | Dyskeratosis congenita (DKC) | |
---|---|---|
Skin | ||
Reticulate hyperpigmentation | + (late stage) | + (early stage) |
Hypopigmentation | + (reticulate or linear) | + (scattered macules) |
Alopecia | + (scarring) | + (non-scarring) |
Epidermal atrophy | + (late stage) | + |
Pigment incontinence | + | + |
Teeth | ||
Hypodontia | + (prominent) | + (occasional) |
Eyes | ||
Retinal involvement | + (retinal detachment, vascular proliferation) | − |
Epiphora | + (rare, one case) | + (frequent) |
Haemopoietic system | ||
Pancytopenia | − | + |
Bone marrow failure | − | + |