Other names: congenital hemidysplasia with ichthyosiform erythroderma and limb defects|
Inheritance: X-linked dominant
Incidence: very rare
Key findings: The descriptive name of the disease identifies the key findings. Poor development of many organs is a constant feature and usually occurs on same side of the body as the ichthyosiform erythroderma.
skin: distinctively patterned, red patches usually stop at the midline and have greasy scale; half of the body can be involved or segmental patches can be limited primarily to one side
hair: alopecia can occur on same side as ichthyosis
nails: various nail abnormalities can occur