[Spontaneous remission of congenital leukemia cutis].

BACKGROUND

Aleukaemic leukaemia--without blasts in the blood or the bone marrow--with isolated cutaneous manifestations has been very rarely reported since only seven patients have been described to date. The prognosis is variable, and the indications for an aggressive treatment such as polychemotherapy are currently unclear. We report a case of spontaneously remitting aleukaemic leukaemia in a newborn child and compare it with other cases in the literature.

CASE REPORT

A male newborn presented diffuse, violaceous skin nodules reminiscent of the so-called "blueberry muffin syndrome" present since birth. Blood and marrow examinations did not show any blasts and karyotype was normal. Biopsy of a nodule established the diagnosis of acute myeloid leukaemia type 5. The course was spontaneously favourable despite the absence of specific therapy and the boy was asymptomatic after one year of follow-up.

DISCUSSION

Of the eight reported infants (including ours), three died, including two through acute transformation of the leukaemia. The prognosis seems to be highly dependent on cytogenetic features with the 11q23 rearrangement being at higher risk of acute transformation, prompting recourse to aggressive chemotherapy. Our case further illustrates the favourable prognostic value of a normal karyotype, a situation in which therapeutic abstention seems possible, and is even recommended.