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February 1989

Picture of the Month

Am J Dis Child. 1989;143(2):255-256. doi:10.1001/archpedi.1989.02150140149040

The statements listed below are best associated with which of the above figure(s):

(a) Transient hematologic and cutaneous eosinophilia.

(b) Histologically the skin lesions show vascular dilatation and/or proliferation.

(c) The hallmark of the clinical diagnosis is the presence of Darier's sign.

Denouement and Discussion  (a) Incontinentia pigmenti, also referred to as Bloch-Sulzberger syndrome, is a genodermatosis with either an autosomal or X-linked dominant (often prenatally lethal in males) inheritance pattern. Characteristic cutaneous features usually, but not always, occur in three successive stages. Present at birth or appearing during early infancy is an erythematous vesiculobullous eruption located primarily on the limbs. This is followed by linear verrucous lesions that are also distributed on the limbs. Subsequently, whorled hyperpigmented lesions (usually asymmetric) involving the torso appear (Fig 3). These pigmented macules gradually spontaneously resolve and the skin usually appears normal by early adulthood. Infrequently, residual atrophic and depigmented lesions are noted

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