A 4-year-old girl was seen in our department for erythroderma, palmopl
antar hyperkeratosis, and scalp desquamation present since birth. The
dermatosis had run an intermittent course, with exacerbations after in
fections and spontaneous remissions. A specimen from a skin biopsy per
formed at 1 year of age showed the characteristic features of psoriasi
s, findings that were confirmed in our biopsy specimen. Treatment with
acitretin controlled the outbreaks. At 7 years of age she has develop
ed, for the first time, plaque type psoriasis. Congenital erythroderma
is an unusual form of psoriasis with a wide differential diagnosis.