Table 1

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+